Surgical Atlas of Orbital Diseases 2008

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Surgical Atlas
of
Orbital Diseases
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Surgical Atlas
of
Orbital Diseases
Subrahmanyam Mallajosyula MS, DO

Head, Dept of Ophthalmology
Bhaskar Medical College
Former Superintendent and Chief
Dept of Oculoplastics and Orbital Services
Sarojini Devi Eye Hospital
Hyderabad, Andhra Pradesh, India
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Surgical Atlas of Orbital Diseases
2008, Jaypee Brothers Medical Publishers
All rights reserved. No part of this publication and DVD ROM should be reproduced, stored in a retrieval system, or transmitted in any form or
by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editor and the
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This book has been published in good faith that the material provided by contributors is original. Every effort is made to ensure accuracy of
material, but the publisher, printer and editor will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters
are to be settled under Delhi jurisdiction only.
First Edition: 2009
ISBN 978-81-8448-394-9
Typeset at JPBMP typesetting unit
Printed at Ajanta Offset & Packagins Ltd., New Delhi
This book is dedicated

to
my family members, my teachers, my team members
and
my patients
Contributors
B Ranganadha Reddy MS
Professor and Head
Dept of Otorhinolaryngiology
Gandhi Medical College
Hyderabad, India
Geeta K Vemuganti MD
Director
Ophthalmic Pathology Service
LV Prasad Eye Institute
Hyderabad, India
Cat N Burkat MD
Assistant Professor
Oculoplastic Surgery Service
Department of Ophthalmology and Visual Sciences
University of Wisconsin
600 Highland Avenue
Madison, WI 53792, USA
Golam Haider MS, FCPS

Associate Professor
National Institute of Ophthalmology
Dhaka, Bangladesh
Christopher M Knapp BSc (Hons), FRC Ophth

Clinical Lecturer
Dept of Ophthalmology
University of Leicester
Leicester Royal Infirmary
Leicester LEI 5 WW, UK
D Ravi Varma DM (Neuroradiology)
Consultant, Interventional and Neuroradiologist
Dept of Radiology
Krishna Institute of Medical Sciences
Hyderabad, India
Debraj Shome DO, DNB, FRCS (Glasgow), MNAMS, MS
Consultant, Department of Ophthalmic
and Facial Plastic and Ocular Oncology
Aditya Jyot Eye Hospital Pvt Ltd
Mumbai and Honorary Consultant
Dept of Ocular Oncology
Advanced Center for Treatment Research and
Education in Cancer
Tata Memorial Center
Mumbai, India
Dinesh Selva MBBS (Hons), FRACS, FRANZCO
Professor and Chairman
South Australian Institute of Ophthalmology
University of Adelaide, Australia
Jack Rootman MD, FRCSC

Professor
Dept of Ophthalmology and Pathology
University of British Columbia
Vancouver, Canada
Kahana Alon MD, PhD
Assistant Professor
Kellogg Eye Center, University of Michigan
1000 Wall Street, Ann Arbor, MI 48105, USA
Kasturi Bhattacharjee MS, DNB, FRCS (Ed)
Senior Consultant and Head
Dept of Orbit
Ophthalmoplastic and Reconstructive Surgery
Shankaradeva Netralaya
Beltola, Guwahati, Assam India
Kuldeep Raizada PhD
Head Dept of Ocular Prosthesis Service
Hyderabad
Leaurence Brown FRC (Path)
Consultant Histopathologist
Dept of Pathology
viii Surgical Atlas of Orbital Diseases

M Chandrasekhara Reddy MS, MCh
Professor, Dept of Neurosurgery
Gandhi Medical College
Hyderabad, India
Mark J Lucarelli MD
Associate Professor
University of Wisconsin, 600 Highland Avenue
Modini Pandarpurkar MS
Assistant Professor of Ophthalmology
Fellow Oculoplastics and Orbital Services
Hyderabad, India
Mohd Ather MS
Assistant Professor of Ophthalmology
Oculoplastics and Orbital Services
Hyderabad, India
Mohd Javed Ali MS, FRCS, FRCGP
Fellow, Dept of Oculoplastics and Orbital Services
Hyderabad, India
Nancy Kim MD, PhD
Fellow, Oculoplastics and Orbital Services
University of Wisconsin Medical School
Madison, Wisconsin, USA
Peter J Dolman MD, FRCSC
Associate Professor
University of British Columbia, Vancouver, Canada
Raghavan Sampath FRCS, FRC Ophth
Consultant Lid, Lacrimal and Orbit Surgeon
Dept of Ophthalmology
Ram Vaidhyanath FRCR
Consultant Radiologist
Dept of Radiology
Raman Mittal DNB
Consultant
Dept of Ophthalmic Plastic Surgery
Orbital Diseases and Ocular Oncology
MGM Eye Institute, Raipur, India
Ramesh Murthy MD, FRCS

Senior Consultant
Oculoplasty and Ocular Oncology Service
and Pediatric Ophthalmology
and Strabismus Service
Hyderabad, India
Ratnakar KS MD
Head
Dept of Pathology
Global Hospital
Hyderabad, India
Ravindra Mohan E MD, FRCS (Edin)
Director
Shankara Netralaya, Chennai, India
Richard K Dortzbach MD, FACS
Professor Emeritus
University of Wisconsin
600 Highland Avenue
Santosh G Honavar MD, FRCS
Director
Dept of Oculoplastics
Orbital Services and Ocular Oncology
Hyderabad, India
Subrahmanyam Mallajosyula MS, DO
Head, Dept of Ophthalmology
Bhaskar Medical College
Former Superintendent and Chief
Hyderabad, India
Venkatesh C Prabhakaran MD
Clinical Lecturer
Oculoplastic and Orbital Division
South Australian Institute of Ophthalmology
and Department of Pathology
University of Adelaide
Australia
Vijay Anand P Reddy MD
Chief, Dept of Radiology and Radiotherapy
Apollo Hospital
Hyderabad, India
Foreword
I became acquainted with Subrahmanyam Mallajosyula (Subbu) when he spent a period of time during 1998
training with me as a fellow in orbit and oculoplastics at the University of British Columbia in Vancouver,
Canada. At that time, he had been trained in India by some of the top surgeons and had furthered his
practical knowledge by visits and fellowships throughout the world. On a personal level, Subbu is an
energetic, kind and competent man driven by a strong desire to teach and to bring contemporary care to
those in great need. He has become a considerable force in aiding his colleagues in India with regard to
oculoplastics and orbit. He is supported by his wife, Kalyani and family, all of whom are wedded to a deep
caring for humanity.
In the last three decades, advances in imaging, pathology, genetics, immunology and endocrinology,
clinical evaluations have led to a consolidation of knowledge concerning diseases of the orbit. Coupled with
surgical innovations, these advances have led to a better understanding of the management of disease
affecting the orbit. There is, however, a need to bring together and simplify this knowledge in order to
provide practical and obtainable care in the developing order. Subbu has gathered a group of distinguished
and well-known orbital specialists as well as colleagues from India with vast, practical experience to
accomplish this goal. His hope is to target readers who are graduate students, residents of ophthalmology,
fellows in oculoplastic and orbital services, and general ophthalmologists who encounter an oculoplastic
problem. This is an important and unique endeavour grounded in Subrahmanyam's long-time practice in
public service in Hyderabad, where he has encountered the full range of orbital problems and challenges.
He has brought to bear his skills, nurtured first in India and then through a range of travel and fellowships
at some of the best orbital centers in the world. It is from these centers and from his wide collegial network
that he has been able to produce this practical compendium of orbital knowledge. I believe his contribution
will not only further the orbital and oculoplastic services in India, Asia and Africa, but also it will bring an
awareness of the vast experience in those areas to the rest of the world. The book is meant to provide
precise and succinct information on orbital disease, its evaluation and management. The emphasis is of
course on common disorders but also presents information on uncommon conditions backed up by case
illustrations.
As a mentor, colleague and author, I feel privileged to be a part of this enterprise.
Jack Rootman
MD, FRCSC
Professor
Department of Pathology and Laboratory Medicine
University of British Columbia
Vancouver, British Columbia, Canada
Preface
When I was a student, I often heard from my teachers saying "Proptosis is a Pandora's box". Surprisingly
I continue to hear it even today! Many a time I am asked to speak on the topic titled "Proptosis is a Pandora's
box", and I always change it to "Is Proptosis still a Pandora's box?" It was so, in the past, when the only
imaging available was orbital venography. The information of the orbital disease process obtained with it
was very meager. I salute my professor Dr. Vengala Rao, who used to perform orbitotomies in those days.
It is the challenges he used to encounter, that stimulated me to take up this branch. Fortunately, advances in
imaging techniques have made an immense contribution in the assessment of a case of proptosis, so that
today, we know what we are dealing with. With careful clinical assessment, and knowledge in reading of
CT/MRI, we can even arrive at the histopathological diagnosis of majority of cases. Hence, surprises are
very few and far in between. Similarly, advances in histopathology and immunohistochemistry, anesthesia,
chemo and radio therapy have made immense contributions in understanding and management of proptosis.
The best example is Rhabdomyosarcoma. Today, nearly half the cases of proptosis can be managed by nonsurgical methods or with very minor surgical procedures. I thankfully acknowledge the roles of Dr Vengala
Rao, my first teacher, Jack Rootman, Peter J Dolman, Brad Lemke and Mark J Lucarelli in furthering my
understanding of orbital diseases.
The specialty of orbital diseases is very well advanced in North America and Europe, but not so in most
other countries. Availability of ophthalmic literatures authored by orbital surgeons from the developing
countries are very few. The idea of bringing this color atlas is to share two decades of my experience in
orbital diseases. Though it is an atlas in principle, enough information is provided to understand the
conditions and plan treatment strategies. It covers most of the common and some of the rare causes of
proptosis. With color illustrations, and case presentations, I tried to make this book interesting to read and
also to provide practical knowledge in clinical situations.
I thank all my contributing authors, who are internationally reputed, for their co-operation. The chapters
they contributed were those in which they have a wealth of experience, viz. Jack Rootman on mesenchymal
tumors, Peter J Dolman on thyroid associated orbitopathy (We rarely see such severe TAO in India ), Mark
J Lucarelli on anatomy and fractures of orbit.
I hope my efforts help shatter the myth that "proptosis is a Pandora's box". If this book inspires at least
some ophthalmologists to pursue this specialty with more interest and vigor and blossom into efficient
orbital surgeons, the purpose of this book is served. I truly believe that "What I do, you can also do" and
who knows you may do even better.
Subrahmanyam Mallajosyula
15-05-2008
Hyderabad
Acknowledgements
At the outset, I wish to acknowledge the significant roles played initially by my parents and then by my
wife Rama, in my professional pursuits, which took a lot of time from my family and children Harsha and
Aahlad. I greatly appreciate their cooperation.
My teacher Dr. Kotagiri Vengala Rao was the first to introduce me to orbital surgery during my postgraduation. I still relish those memories. I thank Dr. Jeffrey Nerad for introducing me to Dr. Jack Rootman.
I acknowledge the role of Dr. Jack Rootman, Peter J Dolman in fine tuning my skillsboth clinical and
surgical. They are not only great teachers, but also wonderful human beings. My fellowship with them was
made possible due to the financial assistance I received from them. I also thank the Orbis Inc. for awarding
me the Ziegler's International Fellowship, which has part financed my fellowship at University of British
Columbia, Vancouver. Similarly I thank the Association of Asian Indians in Ophthalmology for awarding
the competitive fellowship, which financed my training with Mark J Lucarelli, Brad Lemke and Richard K
Dortzbach at the University of Wisconsin, Madison. It was a great learning experience.
I thank all my contributing authors (and their supporting staff), who are all very eminent and highly
reputed, for sparing their time to make this book wonderful. I thank my fellow Dr. Mohd Javed Ali, for all
his assistance in proofreading. He is ever ready to help.
I wish to acknowledge the support and encouragement I received from Shri Jitendar P Vij, Chairman
and Managing Director, Mr Tarun Duneja, Director (Publishing) and Mr PS Ghuman, Sr Production Manager
of M/s Jaypee Brothers Medical Publishers (P) Ltd. I also thank Mr Upinder, Mr Pankaj, Mr Ram Murti and
Mrs Seema Dogra of the same family (Jaypee) for their technical support.
Contents
Part One : Basic Concepts
1.
Applied Anatomy of Orbit ................................................................................................................. 03

Mark J Lucarelli, Nancy Kim
Orbital osteology 3; The periorbita 6; The orbital apex 6; The cavernous sinus 8; The globe 8; The
extraocular muscles 9; Lids 10; The lacrimal system 13; The nerves of the orbit 14; Vascular anatomy of the
orbit: Arterial supply 17; Vascular anatomy of the orbit: Venous outflow 19; Paranasal sinuses 20;
Conclusion 20
2.
Clinical Approach to Proptosis ......................................................................................................... 23

Pain 23; Progression 25; Proptosis 28; Axial proptosis 29; Measurement of proptosis 35; Pulsations 37;
Pupil 41; Perception of color vision 41; Prism bar-cover test (PBCT) 42; Periorbital changes 45; Lid changes
46; Conjunctival changes 48; Palpation 51; Auscultation 51; Evaluation of a case of proptosis 53
3.
Imaging a Case of Proptosis: CT and MRI .................................................................................... 56

Subrahmanyam Mallajosyula, Ravi Varma
Evaluation of a CT scan of orbit 58; Common mistakes 59; Bony orbit 60; Eyeball 65; Enlarged extraocular
muscle 69; Soft-tissue lesions 72; Lacrimal gland tumors 76; Cystic lesions of the orbit 78; Metastatic
lesions 81; Contrast enhancement 83; 3-D reconstruction of the orbit 84
4.
Role of Cytology in Orbital Lesions ................................................................................................ 85

Geeta K Vemuganti, Anirban Bhaduri
Fine needle aspiration/sampling techniques 85; Intraoperative-operative diagnosis by squash and imprint
cytology 85; Squash/imprint cytology 85; Case illustrations 86
5.
Pathology of the Orbital Diseases ................................................................................................... 97

KS Ratnakar
Classification 97; Diagnosis of orbital tumors 98; Developmental lesions 98; Inflammatory lesions 100;
Orbital infections 101; Cysticercosis 102; Neoplastic lesions 102; Benign tumors 103; Malignant tumors
104; Metastasis 105; Grave's disease 106; Mucocele 106
xvi Surgical Atlas of Orbital Diseases
Part Two : Disease Patterns of Proptosis

6.
Thyroid-Associated Orbitopathy .................................................................................................... 111

Peter J Dolman
Incidence and epidemiology 112; Risk factors and predictive variables 112; Pathogenesis 112; Course of
disease 114; Clinical classification 114; The VISA classification 114; Vision/optic neuropathy 114;
Inflammation/congestion 116; Strabismus/motility restriction 117; Appearance/exposure 117; General
management guidelines 118
7.
Orbital Infections ............................................................................................................................... 120

Shome debraj, Walinjkar Jaydeep, Mukherjee Angshuman
Risk factors 121; Etiological causes of orbital infections 121; Bacterial infections 121; Fungal infections
121; Parasitic infections 121; Protozoal infections 121; Diagnosis 121; Imaging studies 121; Emergency
department care 122; Further inpatient care 122; Case illustrations 123
8.
Orbital Inflammatory Disease ........................................................................................................ 128

E Ravindra Mohan, Moupia Goswami, Vinathi Mutyala
Orbital amyloidosis 129; Sarcoidosis 130; Nonspecific orbital inflammatory syndrome (NSOIS) 131;
Kimuras disease 132; Wegeners granulomatosis 132; Langerhans histiocytosis 133; Rosai-Dorfman
disease 133; Orbital xanthogranuloma 134; Case illustrations 134
9.
Orbital Lymphoma ............................................................................................................................. 146

Christopher Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath
REAL classification 146; WHO classification of NHL 146; Modified Ryes classification of Hodgkins
lymphoma 147; When to suspect lymphoma 147; When to suspect idiopathic orbital inflammatory disease
148; Case illustrations 148
10.
Vascular Lesions of Orbit ................................................................................................................ 151

Subrahmanyam Mallajosyula, Mohd Javed Ali
Malformations 151; Lymphangioma 151; Orbital varices 152; Cavernous hemangioma 152; Other
congenital malformations 152; Sturge-Weber syndrome 152; Wyburn-Mason syndrome 153; KlippelTrenaunay syndrome 153; Shunts 153; Carotid-Cavernous fistula 153; New growths 154; Capillary
hemangioma 154; Hemangiopericytoma 155; Angiosarcoma 155; Kaposis sarcoma 155;
Hemangioendothelioma 155; Hemangioblastoma 155; Case illustrations 157
11.
Orbital Tumors of Neurological Origin ........................................................................................ 162

Christopher M Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath
Optic nerve glioma 162; Optic nerve meningioma 163; Orbital schwannoma (neurilemmoma) and
neurofibroma 165; Case illustrations 166
12.
Mesenchymal Tumors ....................................................................................................................... 170

E Weis, J Rootman
Mesenchymal soft tissue tumors 170; Rhabdomyosarcoma 170; Rhabdomyoma 172; Leiomyoma 172;
Leiomyosarcomas 172; Adipose tumors 172; Liposarcoma 174; Fibrous tissue tumors 174; Histiocytic
tumors 175; Fibrous histiocytoma 175; Malignant tumors of uncertain type 175; Rhabdoid tumor 175
Contents xvii
13.
Bone Tumors of Orbit ....................................................................................................................... 180

Venkatesh C Prabhakaran, Dinesh Selva
Clinical presentation 180; Osteoma 180; Fibrous dysplasia 181; Ossifying fibroma 182; Osteoblastoma
183; Chondroma 183; Cholesterol granuloma 184; Aneurysmal bone cyst 184; Giant cell lesions 184;
Osteogenic sarcoma 184; Chondrosarcoma 186; Mesenchymal chondrosarcoma 186; Ewings sarcoma
186; Langerhans cell histiocytosis (LCH) 186; Intraosseous hemangioma 186
14.
Tumors of Lacrimal Gland ............................................................................................................... 190

Raman Mittal
Classification 190; Epithelial cyst (Dacryops) 190; Case illustrations 191; Pleomorphic adenoma 191;
Adenoid cystic carcinoma 194
15.
Cystic lesions of Orbit ...................................................................................................................... 199

Golam Haider, Subrahmanyam Mallajosyula, Mohd Javed Ali
Classification 199; Dermoid and epidermoid cysts 200; Teratomas 201; Cephalocele 201; Microphthalmos
with cyst 202; Mucocele 202; Cysts of the optic nerve sheath 203; Hematic cyst 203; Simple cyst 204;
Retention cyst 204; Lacrimal ductal cyst 204; Implantation cyst 205; Dacryocele 205
16.
Parasitic Cysts of Orbit ..................................................................................................................... 207

Subrahmanyam Mallajosyula, Mohd Ather, Modini Pandarpurkar
Cysticercosis 207; Case illustrations 208; Hydatid cyst of orbit 217
17.
Orbital Fractures ................................................................................................................................ 220

Alon Kahana, Mark J Lucarelli, Cat N Burkat, Richard K Dortzbach
Introduction 220; Anatomy 220; Imaging 226; Implant materials 227; General operative considerations
229; Pediatric patients 231; Timing of surgery 231; Decision: repair or not repair 232; Floor fractures 233;
Medial wall fractures 236; Lateral wall and zygomatico maxillary fractures 238; Late and secondary
fracture repair 238
18.
Secondary and Metastatic Orbital Tumors ................................................................................. 244

Kasturi Bhattacharjee, Harsha Bhattacharjee, Ganesh Kuri, Shyamanga Borooah
Orbital extension of intraocular tumors 244; Orbital extension of retinoblastoma 244; Orbital extension of
medulloepithelioma 246; Orbital extension of uveal melanoma 247; Orbital extension of lacrimal sac
tumors 250; Orbital extension of eyelid tumors 252; Basal cell carcinoma (BCC) 252; Sebaceous carcinoma
of the eyelid 253; Squamous cell carcinoma of the eyelid 255; Malignant melanoma of eyelid 256; Orbital
extension of intracranial tumors 257; Orbital extension of conjunctival tumors 258; Squamous cell
carcinoma of the conjunctiva 258; Malignant Melanoma of the conjunctiva 259; Orbital extension of
tumors of the nasal cavity and paranasal sinus 260; Orbital extension of nasopharyngeal tumor 262;
Metastatic orbital tumors 263
xviii Surgical Atlas of Orbital Diseases
Part Three : Management Strategies: Surgical

19.
Decision Making ................................................................................................................................ 271

Intraconal lesion 273; Reese-Berkes incision 273; Steps of Reese-Berke approach 274; Steps of superior
lidcrease incision 275; Apical conal lesions 279; Lesions of superior peripheral space 279; Thyroid
associated orbitopathy 285
20.
Orbitotomies ........................................................................................................................................ 288

Ramesh Murthy, Anirban Bhaduri, Vikas Menon, Santosh G Honavar
General principles 288; Approaches 289; Anterior orbitotomy 289; Swinging lower eyelid flap 289; Lateral
orbitotomy 290; Stallard-Wright lateral orbitotomy 290; Transfrontal orbitotomy 291; Complications 291;
Postoperative management 291; Case illustrations 291
21.
Multidisciplinary Approach to Proptosis .................................................................................... 299

Subrahmanyam Mallajosyula, B Ranganadha Reddy, M Chandrasekhar Reddy
Surgical anatomy 299; ENT approach to proptosis 300; Various etiological factors of proptosis in ENT
300; Sinus diseases causing proptosis 301; Purulent infections 301; Extensive nasal polyposis 301;
Mucormycosis 301; Allergic fungal sinusitis 301; Fronto- ethmoidal mucocele 302; Tumors of paranasal
sinuses causing proptosis 302; Fibrous dysplasia 302; Hemangiopericytoma 302; Juvenile nasopharyngeal
angiofibroma 303; Squamous cell carcinoma 303; Rhabdomyosarcoma 303; Non-Hodgkins lymphoma
303; Esthesio-neuroblastoma 304; Caldwell-Luc operation 304; Lateral rhinotomy/medial maxillectomy
305; Total maxillectomy 306; Pattersons operation 305; FESS 309; Neurosurgical approaches of proptosis
309; Transcranial approach 311; Extracranial approach 309; Case illustrations 313
22.
Orbital Exenteration .......................................................................................................................... 318

Ramesh Murthy, Anirban Bhaduri, Sima Das, Santosh G Honavar
Indications 318; Patient preparation 318; Surgical procedure 318; Types 319; Management of the
exenterated socket 320; Prosthesis 320; Complications of exenteration 320; Case illustrations 321
23.
Orbital Prosthesis............................................................................................................................... 327

Kuldeep Raizada
Types of prosthesis 327; Complete prosthesis 327; Factors that affect the fit of an orbital prosthesis 328;
Preparation of the patient 328; Impression of the orbital defect 328; Casting 329; Sculpting 329; Moulding
330; Using the desired material 331; Fabrications of ocular prosthesis 331; Assemble of prosthesis 331;
Care of your prosthesis 331; Storing the prosthesis 332; Preventing mishaps 333
Part Four : Management Strategies: Nonsurgical

24.
Medical Management of Proptosis ................................................................................................ 337

Nonspecific inflammations of the orbit (NSOIS) 337; Specific inflammations of the orbit 338; Orbital
cellulitis 338; Rhino-orbital mucormycosis 338; Chronic granulomatous infections 338; Parasitic
infestations 338; Tolosa- Hunt syndrome 339; Capillary hemangioma 339; Acute intraorbital hemorrhage
and emphysema 340; lymphoprolifarative and other neoplastic lesions 340; Case illustrations 340
Contents xix
25.
Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy .. 344

Vijay Anand P Reddy, Nitin More, Ramesh Murthy, Anirban Bhaduri, Santosh G Honavar
Introduction 344; Ionizing radiation 344; Radiation therapy delivery methods 344; External beam radiation
(teletherapy) 344; Internal radiation therapy (brachy therapy) 345; Plaque radiotherapy 346; Principles of
anti-neoplastic therapy 347; Capillary hemangioma 348; Basal cell and squamous cell carcinoma 348;
Tumors of lacrimal gland 348; Malignant conjunctival tumors 349; Intraocular lymphoma 349;
Retinoblastoma 349; Choroidal melanomas 349; Chemoreduction regimen 350; Oculor metastasis 352;
Rhabdomyosarcoma 352; Orbital lymphoma 352; Graves ophthalmopathy 353; Optic nerve glioma 353;
Optic nerve meningioma 353; Sequelae of radiation therapy 353
26.
Carotid-Cavernous Fistulae: Role of Interventional Radiologist .......................................... 356

D Ravi Varma, D Radhika Varma
Pathophysiology 357; Clinical features 357; When to suspect CCF 359; Radiological investigations 359;
Management of CCF 360; Direct CCF 360; Indirect CCF 363; Prognosis 363
27.
Ocular and Systemic Associations of Proptosis ......................................................................... 366

Capillary hemangiomas 366; Neurofibromatosis 366; Craniofacial dysostosis 367; Encephalocele 367;
Wegeners granulomatosis 367; Wyburn-Mason syndrome 367; Hurlers syndrome 368; Nonspecific
orbital inflammation syndrome 368; Sclerosing inflammation of the orbit 368; Osteoma 368; Orbital
hamartoma (tuberous sclerosis) 368; Hemangioblastom 368
Index ..................................................................................................................................................... 369
Applied Anatomy of Orbit
CHAPTER
Mark J Lucarelli, Nancy Kim
Fundamental to the understanding of orbital

pathology and its surgical management is a sound
working knowledge of the anatomy of the normal
orbit in three dimensions. The goal of this chapter is
to review the location of critical ocular adnexal,
orbital and related craniofacial structures and the
anatomic relationships between them.
OVERVIEW
The orbit is defined as the bony cavity containing
the globe, extraocular muscles, fat, nerves and blood
vessels. Although the orbit is often described as
pyramidal in shape, the space is actually pear-shaped,
with its largest horizontal and vertical diameters
lying 1 cm past the orbital rim and adjacent to the
equator of the globe. Average orbital volume is
approximately 25-30 cc, of which the globe occupies
approximately 7 cc of space. The lateral walls are
oriented about 90 to one another and run 40 to 45
mm in length to the apex. The medial walls of each
orbit run parallel to each other and measure 45 to 50
mm in length. The optical axes themselves are also
parallel to the course of the medial walls, rather than
the diverging central axes of the orbits. Therefore,
each globe is tonically held in adduction by the
extraocular muscles to maintain ocular alignment.
These relationships and other important dimensions
of the orbit are illustrated in Figure 1.1.
which make up the four orbital walls: the frontal,

sphenoid (greater and lesser wings), ethmoid,
lacrimal, maxillary, palatine, and zygomatic bones
(Figure 1.2).
The roof of the orbit is comprised of the frontal
bone anteriorly, and the lesser wing of the sphenoid
bone posteriorly (Figure 1.3). The overall thickness
of the roof is significantly greater than that of either
the medial wall or orbital floor and is therefore,
relatively resistant to fracture. Within the lesser wing
lies the optic foramen, through which the optic nerve
exits the orbit via the optic canal. In about 30% of
individuals, just above the frontosphenoid suture,
lies the meningeal foramen through which the
recurrent meningeal artery (a branch of the external
carotid system) passes to anastomose with the
lacrimal artery (a branch of the internal carotid
system). This communication provides an important
potential source of collateral blood flow to the orbit
Orbital Osteology
The orbital rim is roughly in the shape of a spiral,
with its starting and end points at the anterior and
posterior lacrimal crests.1 There are seven bones
Figure 1.1: An axial view of the orbits demonstrating the dimensions and relationships between associated structures
4 Surgical Atlas of Orbital Diseases
Figure 1.2: An anterior-posterior view into the left bony orbit
Figure 1.3: The left orbital roof
Applied Anatomy of Orbit 5

should its primary supply via the internal carotid
system become disrupted. When the meningeal
foramen is absent, the middle meningeal artery
courses directly via the superior orbital fissure.2
Other important bony landmarks include the lacrimal
gland fossa in the temporal roof and the trochlear
fossa anteromedially. Just superolateral to the
trochlear fossa and at the medial one-third junction
of the superior rim, lies the supraorbital notch which
gives passage to supraorbital artery, vein, and nerve.
In some individuals, this point of egress is completely
enclosed and appears as the supraorbital foramen.3
The medial wall includes the ethmoid, maxillary,
and lacrimal bones, as well as the lesser wing of the
sphenoid (Figure 1.4). Within the bony suture line
separating the frontal from the ethmoid bone, there
are two important apertures, the anterior and
posterior ethmoidal foramina. These foramina are
the exit points for the anterior and posterior
ethmoidal arteries and nerves, respectively. The
anterior ethmoidal foramen is typically located
approximately 24 mm posterior to the orbital rim
and the posterior ethmoidal foramen lies
approximately 36 mm posterior to the rim. The optic
foramen, in turn, is located approximately 6 mm
posterior to the posterior ethmoidal foramen. These
foramina help the surgeon delineate the frontoethmoidal suture which is an important surgical
landmark for the roof of the ethmoid sinus, or fovea
ethmoidalis. The orbital roof slopes downward as it
travels medially. Medial to the orbital space, just
beyond the frontoethmoidal suture line, the fovea
ethmoidalis continues in a downward plane and ends
Figure 1.4: Medial wall of the right orbit
sagittally just above the nasal cavity and below the

anterior cranial fossa at the cribriform plate. Bony
dissection of the medial wall above the suture line
exposes the dura of the frontal lobe.
The ethmoid portion of the medial wall, the
lamina papyracea, is extremely thin and is thus prone
to fracture with trauma and to easily transmit
infection from the ethmoid air cells into the orbit as
subperiosteal abscesses. The medial wall thickens
again in the area of the inferior suture between the
ethmoid and maxillary bones. This maxilloethmoid
strut4 provides support to the inferomedial orbital
wall and often survives trauma which fractures the
more superior aspects of the wall. At the anterior
aspect of the medial wall is the lacrimal sac fossa,
bounded by the anterior and posterior lacrimal
crests. The anterior and posterior limbs of the medial
canthal tendon insert on the anterior and posterior
lacrimal crests, respectively.
The floor of the orbit consists of the maxillary,
zygomatic and palatine bones. The maxillary bone
forms the bulk of the floor while the zygomatic bone
contributes anterolaterally and the palatine bone
contributes to the posterior floor. A major landmark
in this area is the infraorbital groove, which
originates approximately 25-30 mm posterior to the
orbital rim. The groove deepens and becomes an
enclosed canal as it travels anteriorly within the floor
to open again on the face of the maxillary bone at
the infraorbital foramen on the maxillary face, 4-6
mm from the rim in adults.5 This pathway contains
the infraorbital neurovascular bundle which is easily
injured by floor fractures or inadvertent surgical
dissection. Just medial to the infraorbital groove is
the thinnest portion of the maxillary bone. Not only
does this render the posteromedial part of the floor
particularly susceptible to blowout fractures, but it
also provides an area where bone can be removed
with relative ease for inferior orbital decompression.
The thicker, maxilloethmoid strut lies in the medial
floor and provides support for the orbital soft tissues
and the globe.4, 6 In the anteromedial floor, the bony
nasolacrimal duct travels from the base of the
lacrimal fossa in an inferior and usually slightly
posterolateral direction through the maxillary bone
in the lateral nasal wall to empty into the inferior
meatus of the nose. The vector of the nasolacrimal
duct shows considerable variability.

The lateral wall contains the zygomatic bone and
the greater wing of the sphenoid which separates
the posterolateral orbit from the middle cranial fossa.
The posterior borders of the lateral wall are defined
by the superior and inferior orbital fissures. The
boundary between the lateral wall and roof is formed
by the frontosphenoid suture, which transmits the
recurrent meningeal artery. The anterior part of the
lateral wall is comprised of the zygoma. Important
landmarks in this region include the lateral orbital
tubercle, or Whitnalls tubercle, which is the insertion
point of the posterior head of the lateral canthal
tendon, the lateral horn of the levator aponeurosis,
the check ligament of the lateral rectus muscle, and
Lockwoods ligament. The tubercle can be found just
inside the orbital rim and approximately 11 mm
below the frontozygomatic suture. 7 The superoanterior zygoma also contains the zygomaticotemporal and zygomaticofacial canals through which
branches of the lacrimal artery and the lacrimal and
zygomatic nerves pass (Figure 1.5).
superior orbital fissure and optic canal to become

continuous with the dura. The potential space outside
the periorbita is an important surgical plane. Access
to the orbital walls in decompression surgery, for
example, entails dissection between the bone and this
overlying periosteal sheet.
The Orbital Apex
The periorbita refers to the tough, fibromembranous

lining of the bony orbit which acts as a physical barrier
to infection and provides a scaffold to which other
intraorbital connective tissues can attach. The regions
of greatest adherence between this sheath and bone
are at the orbital rim, suture lines, bony fissures,
trochlear fossa and the lacrimal crests. At the orbital
margins, at the arcus marginalis, the periorbita
thickens and gives rise to the orbital septum. Deep
in the orbit, the periorbita continue through the
The orbital apex merits special attention, as the region

in which many critical orbital structures converge
and communicate with other important, periorbital
spaces (Figure 1.6). Cranial nerves II through VI,
major orbital vessels, and all of the extraocular
muscles excluding the inferior oblique sit in tight
proximity within the apex, and pathology in this
region can produce profound deficits in vision and
ocular motility.8, 9
The apex is defined by only three walls; the floor
is absent in the far posterior orbit. 1 Major bony
landmarks include the superior orbital fissure,
inferior orbital fissure, and the optic canal. The
superior orbital fissure divides the sphenoid into the
greater (lateral) and lesser (medial) wings and lies
inferiorly and laterally to the optic foramen. This
fissure measures approximately 20-22 mm in overall
length and is separated into superolateral and
inferomedial sections by the tendon of the lateral
rectus muscle. The superotemporal part of the fissure
lies above the annulus of Zinn, the fibrous ring
formed by the common origin of the rectus muscles.
The lacrimal, frontal and trochlear nerves, and the
superior ophthalmic vein pass through this region as
they traverse the apex. The inferomedial segment of
Figure 1.5: Lateral wall of the right orbit
Figure 1.6: The left orbital apex
The Periorbita
Figure 1.7: The superior and inferior orbital fissures and associated apex structures of the right eye
the superior orbital fissure, also called the oculomotor

foramen, is located inside the annulus and transmits
the superior and inferior divisions of the oculomotor
nerve, the abducens nerve, sympathetic fibers, and
the nasociliary nerve, a terminal sensory branch of
the ophthalmic division of the trigeminal nerve10-12
(Figure 1.7).
The inferior orbital fissure bounds the greater
wing of the sphenoid, separating it from the
maxillary bone inferomedially. This fissure
communicates primarily with the pterygopalatine
fossa (Figure 1.8). The maxillary branch of the
trigeminal nerve passes through the pterygopalatine
fossa and subdivides into the infraorbital nerve
which, in turn, travels anteriorly into the orbit via
the infraorbital groove. The zygomatic nerve, another
branch of the maxillary branch of cranial nerve V,
enters the orbit through the inferior orbital fissure
to provide sensory innervation to lateral orbit and
cheek after passing through the zygomaticofacial
foramen. Also within the pterygopalatine fossa is
located the maxillary artery which gives rise to the
infraorbital artery, part of the neurovascular bundle
traveling through the infraorbital groove. Parasympathetic fibers originating from the pterygo-
palatine ganglion and terminating in the lacrimal

gland are transmitted by the inferior orbital fissure
as well.2 The inferior ophthalmic veins also pass from
the orbit into the pterygoid plexus via the inferior
orbital fissure.
The optic canal penetrates the superomedial
orbital apex through the lesser wing of the sphenoid
bone as the optic foramen. The canal is approximately
6 mm in diameter and 8-10 mm in length and houses
the optic nerve and the ophthalmic artery. The canal
runs along the upper, lateral wall of the anterior
sphenoid sinus and in the floor of the anterior cranial
fossa.
Figure 1.8: Lateral cross-section of the orbit
The Cavernous Sinus

The cavernous sinus is a large venous sinus
posterior to the orbital apex contained within a dural
cleft which is situated lateral to the sphenoid sinus
(Figure 1.9). Its tributaries consist of the ophthalmic,
cerebral middle meningeal, and pterygoid veins. The
left and right cavernous sinuses communicate with
one another via small channels which run superior
to the roof of the sphenoid sinus. Several critical
structures pass through the cavernous sinus as they
travel into the orbit. The carotid siphon and the
sympathetics which ride along on its sheath traverse
centrally. In the lateral wall of the cavernous sinus
area embedded the oculomotor nerve, trochlear
nerve, the ophthalmic and maxillary divisions of the
trigeminal nerve, and the abducens nerve. The optic
nerves course superomedially to the cavernous sinus.
The optic chiasm is formed just above the anterior
aspect of the cavernous sinus.
As in the orbital apex, pathological processes
involving the cavernous sinus such as the formation
of carotid-cavernous fistulas, inflammation or
infection typically cause multiple cranial neuropathies
affecting the eye.13 Further, because the right and
left cavernous sinuses are interconnected, disease
processes extending posteriorly from the orbit on
one side can spread to the other via these spaces.
The Globe
The average-size globe measures approximately
23.5 mm in the horizontal meridian and 23 mm
Figure 1.9: A cross-section of the cavernous sinus
vertically, with an anterior-posterior dimension of

about 24 mm. Its overall volume is about 7cc. The
globe is surrounded by a loose fascial sheath, or
Tenons capsule, which is interconnected to the sclera
by fine fibrous bands. The potential space between
these layers is the episcleral space, and the areas of
greatest adherence between them are approximately
1.5 mm from the limbus anteriorly, and posteriorly,
at the optic nerve sheath. Tenons capsule is
suspended inside the orbit by interconnections with
fine connective tissue septae within the surrounding
orbital fat. This sheath must be traversed by the
nerves and blood vessels which supply the globe.
Likewise, the extraocular muscles must penetrate
Tenons layer to attach to the globe. As the muscles
pass from outside to inside the episcleral space to
fuse with the sclera, Tenons capsule makes
attachments with the intermuscular septum, a fibrous
network which encases and interconnects the
extraocular muscles (Figure 1.10).14-18 Thus, following
enucleation, orbital implants that are placed within
Tenons capsule may demonstrate a fair amount of
motility even if they are not sutured to the extraocular
muscles themselves.18 The intermuscular septum and
rectus muscles delineate the intraconal versus
extraconal space.
Interconnections between the extraocular muscle
sheaths and the periorbita comprise the check
ligaments. Superiorly, the check ligaments consist of
the fascial complex surrounding the upper lid
retractors, the superior rectus and levator palpebrae
muscles. 19 Similarly, the inferior check ligament
consists of the muscle sheaths surrounding the lower
lid retractors, the inferior rectus and inferior oblique.
Figure 1.10: The extraocular muscles and intermuscular fascia

Medially, the muscle sheath of the medial rectus
inserts just inside the posterior lacrimal crest, to the
medial orbital septum and caruncle to collectively
form the medial check ligament. The analogous check
ligament of the lateral rectus inserts onto the lateral
orbital tubercle of Whitnall.7
The Extraocular Muscles

The four rectus muscles originate at the annulus of
Zinn, within the orbital apex. Specifically, the superior
and medial recti originate adjacent to the lesser wing
of the sphenoid, next to the optic canal. The inferior
rectus originates from a portion of the annulus which
extends from the body of the sphenoid bone to its
great wing. The lateral rectus has a bifid origin from
a tendinous segment of the annulus which extends
across the superior orbital fissure from the greater
to the lesser sphenoid wing and a more inferior
portion which extends directly from the greater wing
itself.
The superior rectus lies just underneath the
levator palpebrae. Immediately beneath and medial
to the superior rectus run the nasociliary nerve and
ophthalmic artery. The superior edge of the medial
rectus travels just under these structures. From its
origin at the annulus, the inferior rectus closely
follows the floor of the orbit until reaching the
anterior orbit, where it becomes separated from the
floor by the inferior oblique muscle as the latter
crosses from the medial to the lateral wall, and by
fat. Medial and superior to the lateral rectus, is found
the ciliary ganglion which is usually adherent to the
intraorbital segment of the optic nerve.
The superior oblique muscle also begins along
the annulus of Zinn, superomedially and extends
forward superiorly and along the junction between
the orbital roof and the medial orbital wall. As it
courses toward the anterior orbit, the muscle belly
transitions to a tendinous segment as it reaches the
trochlea, a pulley-like cartilaginous structure which
lies approximately 6-10 mm posterior to the
superomedial orbital rim.20, 21 From this point, the
tendon passes posterolaterally, making a 54 angle,
to attach to the globe. It is this course which gives
rise to the main actions of the superior oblique,
namely intortion and depression of the globe with
contraction. During orbital surgery, caution to avoid
injuring the trochlea must be taken to avoid
subsequent scarring and restriction of superior

oblique muscle action, or Browns syndrome.22
The inferior oblique, unlike the other extraocular
muscles, does not originate at the annulus, but from
the periosteum of the anterior, inferomedial orbit
on the maxillary bone. It courses posterolaterally,
just beneath the inferior rectus to insert on the
inferolateral globe, which gives rise to its main
actions: extortion and elevation of the globe. The
capsulopalpebral fascia and Lockwoods ligament
interdigitate with the muscular fascia of the inferior
oblique.
The extraocular muscles approximate a spiral in
the distance between their individual insertions and
the corneal limbus, the so-called spiral of Tillaux
(Figure 1.11). Beginning with the medial rectus, each
successive muscle inserts farther from the limbus.
Although there is individual variation, the average
distances are: medial rectus, 5.5 mm; inferior rectus,
6.5 mm, lateral rectus, 6.9 mm, and superior rectus,
7.7 mm.23
Innervation to the extraocular muscles is largely
carried by the oculomotor nerve (third cranial nerve),
which supplies the medial, inferior, and superior
rectus muscles, the inferior oblique, as well as the
levator palpebrae superioris. The superior oblique
and lateral rectus receive innervation from the
Figure 1.11: Spiral of Tillaux

trochlear nerve (fourth cranial nerve) and the
abducens nerve (sixth cranial nerve), respectively.
The blood supply to the muscles is carried by
muscular branches of the ophthalmic artery.
Lids
Understanding the general anatomy and dimensions
of the major eyelid landmarks is important in the
evaluation of structural disease of the lids and in
planning surgical repair. The normal interpalpebral
fissure height is 10-12 mm and the average length is
28-30 mm. The distance between the upper lid crease
and lid margin measures approximately 8-11 mm at
the pupillary axis. The highest point of the upper lid
contour rests just nasal to the center of the pupil and
the upper lid margin is typically located 1-2 mm below
the superior limbus in adults. The lateral canthal
angle sits approximately 2 mm higher than the medial
canthus.
Both the upper and lower eyelids can be divided
into the skin, orbicularis, orbital septum, orbital fat,
lid retractors, tarsus, and conjunctiva. For descriptive
purposes, the layers can be grouped into the anterior
Figure 1.12: A cross-section of the upper and lower lids
and posterior lamellae. The anterior lamella contains

lid structures which lie outside the orbit per se, and is
comprised of the skin and the orbicularis oculi
(Figure 1.12).
The skin of the lids is the thinnest of the body
and unlike skin elsewhere, has no subcutaneous fat
layer. The portion which lies anterior to tarsus is
relatively firmly attached to deeper structures while
the preseptal portions are loosely connected. Anterior
to the margin, the skin contains the lash follicles with
their associated sebaceous glands of Zeis and apocrine
glands of Moll, as well diffusely distributed eccrine
sweat glands.
The orbicularis muscle is a C-shaped complex
of muscle fibers which functions to close the lids
(Figure 1.13). It is divided into pretarsal, preseptal
and orbital sections, all of which receive innervation
from the facial nerve (seventh cranial nerve). The
pretarsal and preseptal parts of orbicularis are
primarily involved in involuntary closure of the lids,
as elicited by the blink reflex. These fibers insert at
the medial canthal tendon as deep and superficial
heads. A subset of pretarsal orbicularis fibers, also
called Horners muscle, inserts deep at the posterior
lacrimal crest as the deep limb of the medial canthal
Figure 1.13: The orbicularis oculi

tendon, along the posterior aspect of the lacrimal
sac and surround the canaliculi. These fibers are
thought to provide a pumping action as they contract
which facilitates tear drainage.24, 25 Horners muscle
is also critical in maintaining close contact between
the posterior aspect of the lid and the globe. The
remaining pretarsal orbicularis inserts superficially
within the anterior limb of the medial canthal tendon.
Laterally, slips from the upper and lower lid pretarsal
orbicularis insert onto the lateral canthal tendon
which in turn, inserts on the lateral orbital tubercle.
Medially, the deep head of the preseptal orbicularis
inserts into the fascia surrounding the lacrimal sac
while the superficial head inserts onto the anterior
limb of the medial canthal tendon.
The orbital orbicularis is chiefly responsible for
forced lid closure, such as winking or in blepharospasm. Medially, its insertions lie along the
orbital rim and anterior medial canthal tendon. As
they course laterally, these fibers overlie the zygoma
and the elevators of the lateral mouth, the
zygomaticus major and minor.
Underlying the pretarsal orbicularis and resting
anterior to the tarsus is the muscle of Riolan which
consists of small, horizontally oriented slips of muscle.
These fibers appear grossly as the gray line of the
lid margin which is posterior to the lash line and
function to turn the lashes toward the eye during
blinking. The gray line is a useful landmark in
aligning the wound edges in marginal lid laceration
repair.
The septum, orbital fat and posterior lamella,
which consists of the retractors, tarsus and conjunctiva, are considered to be intraorbital structures.
The septum is comprised of tough fibrous connective
tissue arranged in sheets which originate from the
periosteum of the orbital rims at the arcus marginalis.
This structure acts as an relative barrier between the
orbit and lid in limiting the deep spread of superficial
hemorrhage and infection. In the upper lid, the
septum fuses with the aponeurosis of the levator
muscle, the primary upper lid retractor muscle,
approximately 2-5 mm above the superior tarsal
edge. 26 In the lower lid, the septum condenses
with the capsulopalpebral fascia as the two layers
converge toward the inferior edge of the lower tarsal
plate.27
The orbital septum lies anterior to the

preaponeurotic orbital fat pads, which prolapse
forward with any violation of the septum. As a
natural consequence of aging, as the septum thins
and stretches, these fat pads tend to gradually
herniate anteriorly. In the upper lid, there are two
distinct fat pads, the medial and central fat pads.
The medial pad can be distinguished by its relatively
white color. The medial palpebral artery typically
runs within this pocket and care should be taken to
avoid inadvertent laceration or cauterization during
surgery. Laterally, there is usually little fat in the
upper lid. Instead, this space is usually filled by orbital
lobe of the lacrimal gland.
The lower lids contain medial, central and lateral
fat pads. The medial pad lies just medial to the inferior
oblique and as in the upper lid, has a characteristically
white color and contains the lower palpebral artery.
The central fat pad lies between the inferior oblique
muscle and a fascial band that separates it from the
lateral fat pad. The latter extends to the inferior edge
of the lacrimal gland.28
The upper lid retractors consist of the levator
muscle which is innervated by cranial nerve III, and
the sympathetically innervated Mllers muscle. The
levator, which is the primary retractor, originates
from a point just above the annulus of Zinn, from
the lesser wing of the sphenoid bone. The levator
complex includes a muscular component which is
approximately 40 mm long extending from its origin
on the lesser sphenoid wing just outside the annulus
of Zinn, and the fibrous levator aponeurosis which
is 14-20 mm in length. As the muscular portion
courses forward in the orbit, it rides just above the
superior rectus muscle and the two are interconnected by interdigitated fibrous bands. As they
reach the equator of the globe, the levator broadens
and transitions into its aponeurotic component.
Medially and centrally, the aponeurosis inserts onto
the anterior tarsal surface and passes through the
orbicularis to insert onto pretarsal skin. These
insertions create the upper lid crease. Medially, the
aponeurosis separates into a single medial horn which
inserts into the posterior lacrimal crest and becomes
continuous with the medial canthal tendon complex.
Similarly, the aponeurosis courses into a lateral horn
which inserts into the lateral orbital tubercle, also

called Whitnalls tubercle, and becomes continuous
with the lateral canthal tendon complex (Figure 1.14).
The normal magnitude of upper lid elevation is
approximately 14-16 mm. Elevation of less than
10-12 mm is usually abnormal. Elevation of less than
5 mm is considered severe dysfunction and has
important implications in ptosis surgery. Because of
the close apposition and fibrous interconnections
between the levator and the superior rectus muscle,
when the globe is elevated, the upper lid follows.
This relationship is not passive, and the levator and
superior rectus actually co-contract. Likewise, when
the globe is depressed, both muscles relax together
and the upper lid moves downward.29
At the transitional zone between the anterior
muscular component of the levator and its
aponeurosis is a fascial sleeve called Whitnalls
ligament, or the superior transverse ligament. This
band runs both over and beneath the levator at this
point and behaves as a fulcrum point for the levator
where contraction of the muscular portion in the
horizontal plane becomes directed in the vertical
direction.30-32 However, Whitnalls ligament is not a
stationary fulcrum,29 rather, it acts more as a swinging
suspender of the levator.31 Whitnalls ligament also
provides mechanical support for the superior orbital
soft tissues. Medially, this structure inserts within
the fascial tissue surrounding the superior oblique
tendon and the trochlea. Laterally, the ligament
inserts within the inner surface of the lateral wall
into the periorbita of the lacrimal gland fossa,
approximately 10 mm above the lateral orbital
Figure 1.14: The levator superioris complex,

including Whitnall's ligament
tubercle, or Whitnalls tubercle. (Note that despite

the shared eponym, Whitnalls ligament does not
directly insert into Whitnalls tubercle). Prior to its
lateral insertion, the ligament courses across and
divides the lacrimal gland into a superior orbital lobe
and an inferior palpebral lobe.30 (Figure 1.15).
Mllers muscle is a secondary upper lid retractor,
providing approximately 1-2 mm of elevation.
It originates just deep to the levator aponeurosis at
the level of Whitnalls ligament and is about 12-14
mm in length. Mullers muscle inserts at the superior
edge of the tarsal plate. An important landmark for
Figure 1.15: The lacrimal gland and its relation to the levator superioris complex

this structure is the peripheral vascular arcade which
lies between the levator aponeurosis and Mllers
muscle just above the tarsus. Injury to Mllers or
loss of sympathetic innervation, as occurs in
Horners syndrome, causes a characteristic mild
(1-2 mm) ptosis.33
In the lower lid, the retractor complex is called
the capsulopalpebral fascia. This structure is a
condensation of fibrous attachments to terminal
muscle slips from the inferior rectus which course
anteriorly to surround the inferior oblique muscle
and fuse with its sheath. From this point, an important
component of this fascial complex forms Lockwoods
ligament, which extends across the width of the
inferior orbit somewhat like a hammock, inserting
laterally at the lateral orbital tubercle and medially
into the medial canthal tendon and providing some
suspensory support to the orbital soft tissues.34
Anterior to Lockwoods ligament, the capsulopalpebral fascia send fibers into the inferior
conjunctival fornix (thus forming the suspensory
ligament of the inferior fornix), while additional
fibers continue on to fuse with the septum and to
finally insert into the inferior border of the tarsal
plate. As in the upper lid, the lower lid retractors
work in tandem with the inferior rectus to lower the
lid with downgaze.
The analogous lower lid structure to Mllers
muscle in the upper lid is the inferior tarsal muscle.
Loss of sympathetic innervation may cause a small
amount of reverse ptosis of the lower lid, elevating
the inferior lid margin by approximately 1 mm above
its usual resting position.33
The tarsal plates are comprised of dense
connective tissue that act at the structural skeleton
of the lids. In both lids, the tarsi are 1 mm in
thickness. In the upper lid, the tarsus is approximately
10-12 mm in height at the pupillary axis, while the
vertical extent of the lower tarsus is 4 mm. The tarsi
contain the oil-producing meibomian glands which
open on the margin, just posterior to the lash line. In
the upper lid, approximately 2-3 mm from the tarsal
margin, lies the marginal arterial arcade. In the lower
lid this arcade typically lies within 1 mm of the lashes.
Distichiasis is the abnormal growth of lashes from
the meibomian gland orifices and may occur as a
congenital anomaly or as an acquired state. In the
latter case, distichiasis is often a result of severe

chronic inflammation of the lids35.
At their medial and lateral borders, the tarsi
taper. The upper and lower tarsi come together at
the canthus to form the deep lateral canthal tendon,
which inserts just anterior to the lateral orbital
tubercle. Recall that the more superficial components
of the lateral canthal tendon extend from the lateral
pretarsal and preseptal orbicularis oculi muscles.
Similarly, the medial aspects of the upper and lower
tarsi contribute to the medial canthal tendon, with
larger, more superficial components which arise from
the orbicularis oculi.
The conjunctiva comprises the most posterior
layer of the lids. Basal tear flow is provided by the
accessory lacrimal glands of Krause in the upper
conjunctival fornix, and the glands of Wolfring in
the lower fornix. Additional mucin-producing glands
are distributed within both the orbital and palpebral
conjunctivae.
The Lacrimal System

The main lacrimal gland lies in the anterolateral
orbital roof, within the lacrimal gland fossa of the
frontal bone, and measures roughly 20 12 5 mm.
The gland is separated into a palpebral and an orbital
lobe by the lateral levator aponeurosis. The primary
suspensory support for the main lacrimal gland comes
from the Whitnalls ligament. 1 Damage to the
ligament leads to forward and downward prolapse
of the gland in the orbit.36 Ducts from both lobes
pass through the palpebral lobe to empty into the
superolateral fornix. Therefore, ideally, lacrimal
gland biopsies should not be performed on the
palpebral lobe, since injury here may affect drainage
from both lobes37 (Figure 1.15).
Innervation and blood supply are provided by
the lacrimal nerve and lacrimal artery, which enter
the gland posteriorly. Venous drainage occurs via
the lacrimal vein, which empties into the superior
ophthalmic vein. Parasympathetic inputs originate
from the lacrimal nucleus of the pons. These
preganglionic fibers pass through the geniculate
ganglion and then travel with the greater petrosal
nerve to synapse eventually within the pterygopalatine ganglion. These fibers then directly synapse
in the lacrimal gland.38, 39 Additional postganglionic

fibers traveling along branches of the maxillary
division of the trigeminal nerve that converge with
the lacrimal nerve to enter the orbit also innervate
the lacrimal gland.40
Tears drain medially via the upper and lower lid
puncta, into the canaliculi, and into the lacrimal sac
(Figure 1.16). The puncta are approximately
0.3 mm in diameter. The initial segment of each
canaliculus extends 2 mm perpendicular to the lid
margin then turns roughly 90 medially toward the
canthus. These horizontal canalicular segments are
approximately 8 mm in length. The lower canaliculus
is typically slightly longer than its upper lid
counterpart. In 90% of individuals, the upper and
lower canaliculi then fuse to form a 2 mm long
common canaliculus which lies between the anterior
and posterior limbs of the medial canthal tendon and
enters the lacrimal sac.41 The valve of Rosenmller is
located at this junction and prevents the reflux of
tears from the sac retrograde into the canaliculi. The
lengths of each component of the lacrimal drainage
system become important when performing probing
and irrigation to evaluate the patency of the outflow
system.
The lacrimal sac sits within the lacrimal sac fossa.
It is 12 mm long and its fundus lies 3-4 mm superior
to the valve of Rosenmller.42 The sac lies just anterior
to the middle turbinate of the nose. The inferior sac
is contiguous with the nasolacrimal duct which
courses in the wall of the lateral nose and empties
via the valve of Hasner just below the inferior
turbinate. The valve of Hasner may be imperforate
in young infants, and is the most common site of
nasolacrimal duct obstruction in this age group.
inadvertent perforation of the optic nerve sheath

during retrobulbar anesthesia.43
There are four major segments to the optic nerve,
including the intracranial, intracanalicular, intraorbital and the intraocular segments. The
intracanalicular segment of the optic nerve is tightly
surrounded by its dural sheath and tethered within
the bone. Because of this, the intracanalicular segment
of the optic nerve is particularly susceptible to blunt
trauma.44, 45 Once it passes through the optic foramen,
the length of the intraorbital portion of the nerve is
roughly 24-30 mm as it traverses the 20 mm or so
distance to the globe. Thus, the nerve has a slightly
serpentine course inside the orbit that allows for
movement of the globe and some degree of proptosis.
However, severe proptosis puts the nerve on stretch,
described radiographically as globe tenting. 46
The intraocular length of the nerve is approximately
1 mm.
Sensory innervation of the orbit: Sensory innervation
of the periorbital region is carried by the ophthalmic
and maxillary divisions of the trigeminal nerve (fifth
cranial nerve). Both branch from the trigeminal
ganglion which is located within the lateral wall of
the cavernous sinus.
The ophthalmic branch further subdivides into
three segments: the frontal, lacrimal and nasociliary
nerves. The frontal and lacrimal branches enter the
The Nerves of the Orbit

The optic nerve: The optic nerve (the second cranial
nerve) is actually part of the central nervous system,
extending directly from the brain into the orbit. Like
the rest of the central nervous system, the optic nerve
is invested within a dural sheath and leptomeninges,
surrounded by cerebrospinal fluid, and in part, is
covered with myelin. The fact that cerebrospinal fluid
surrounding the optic nerve communicates with the
fluid surrounding the cerebrum and brainstem is the
basis for the seizures and life-threatening
cardiopulmonary depression which can occur with
Figure 1.16: The lacrimal drainage system

orbit in the superolateral part of the superior orbital
fissure, outside the annulus of Zinn. The frontal nerve
courses through the extraconal fat and separates in
the anterior orbit into several smaller branches
including the supraorbital branch which supplies the
scalp, forehead, upper lid, and conjunctiva. The
supraorbital nerve exits via the supraorbital notch
or foramen and should be carefully avoided during
dissection of the superior orbital rim. Injury to the
deep, lateral branches of the supraorbital nerve which
run beneath the frontalis muscle, as can occur during
forehead lift surgery leads to scalp numbness to the
vertex.47 The other major division of the frontal
nerve, the supratrochlear nerve, exits just above the
trochlea to innervate parts of the lower forehead and
medial canthal region. The lacrimal nerve travels with
the lacrimal artery superolaterally in the extraconal
space, along the superior border of the lateral rectus
(Figure 1.17). As it travels forward, it is joined by
parasympathetic motor fibers within the orbit which
began within the nervus intermedius and which
supply the lacrimal gland, superolateral lid and

conjunctiva.37
The nasociliary nerve enters the orbit via the
superior orbital fissure within the annulus of Zinn,
traversing just under the superior rectus muscle and
over the optic nerve medially as it courses forward
in the orbit in association with the ophthalmic artery.
In the posterior orbit, it subdivides into long
posterior ciliary nerves which run medially and
laterally toward the globe, giving off sensory fibers
which travel through the ciliary ganglion without
synapsing. The long ciliary nerves enter the sclera
and continue forward, innervating the iris, cornea
and ciliary muscles. Additional fibers from the
nasociliary nerve travel superomedially and are
responsible for sensation from the nasal mucosa and
the skin on the medial tip of the nose via the anterior
ethmoidal nerve. It is this branch which is responsible
for Hutchinsons sign in cases of herpes zoster
ophthalmicus. The final anterior branch of the
nasociliary nerve is the infratrochlear nerve, which
Figure 1.17: Lateral view of the orbit and major orbital sensory nerves

traverses the orbital septum inferior to the trochlea
to supply the medial eyelid skin, lacrimal sac and
the caruncle.
The maxillary division of the trigeminal nerve
exits the middle cranial fossa via the foramen
rotundum to enter the pterygopalatine fossa. From
here, the zygomatic branch enters the inferior orbit
via the inferior orbital fissure. It further subdivides
into the infraorbital, zygomaticotemporal, and
zygomaticofacial nerves. The infraorbital nerve exits
the orbit via the infraorbital notch or groove to supply
the skin of the lower lid, cheek and medial upper lip
(Figure 1.2). Injury to this nerve by fractures
involving the orbital floor result in hypesthesia over
these areas. The zygomaticotemporal and zygomaticofacial nerves provide sensory innervation to
the lateral brow and lateral cheek, respectively.
Motor innervation of the orbit: Motor innervation
to the orbit involves the oculomotor, trochlear and
abducens nerves, or the third, fourth and sixth cranial
nerves, respectively. The oculomotor nerve exits the
brainstem medially, leaving its dural sheath to enter
the superolateral aspect of the cavernous sinus. Here,
it divides into superior and inferior divisions which
both pass into the orbit through the superior orbital
fissure, within the annulus of Zinn. The superior
division sends branches to the levator muscle and
superior rectus while the inferior division branches
into three parts to supply the medial rectus, inferior
rectus and inferior oblique. The branch which
innervates the inferior oblique also carries parasympathetic fibers which synapse in the ciliary
ganglion. Thus, injury due to surgery or trauma to
these inferior orbital structures can lead to an efferent
pupillary defect and dilation.48
The trochlear nerve, the smallest and longest of
the cranial nerves, arises from the dorsal midbrain,
crosses the midline to emerge adjacent to the superior
cerebellar peduncle. It enters the cavernous sinus
along its lateral wall, reaching the orbit via the
superior orbital fissure, above the annulus (along with
the frontal and lacrimal nerves). It travels
anteromedially above the levator just inferior to the
periorbita, and enters the superior oblique at the
muscle bellys posterior third. The trochlear nerve is
unique among the cranial nerves. It is the only cranial
nerve innervating an extraocular muscle which does
not penetrate the intraconal surface of the muscle it
serves. It is also the smallest cranial nerve, has the

longest intracranial component, and is the only
cranial nerve to exit dorsally from the brainstem.
For these reasons, it is also the most prone to injury
with closed head trauma.49
The abducens nerve originates from the pons and
enters the cavernous sinus, initially following a
course within the sinus near the internal carotid
artery before coursing laterally along the wall. It
passes into the orbit via the intra-annular portion of
the superior orbital fissure, running along the inner
surface of the lateral rectus and piercing the muscle
belly at its posterior one-third. The intracranial
course of the abducens nerve turns sharply as it
crosses the petrosphenoidal ligament, making it
particularly prone to injury50, 51 with acute increases50
or decreases52 in intracranial pressure.
Sympathetic innervation of the orbit: Sympathetics
to the orbit which supply the iris dilator, eyelid
muscle, eccrine sweat glands, and blood vessels
originate from the superior cervical ganglion. These
fibers travel along the internal carotid artery, through
the cavernous sinus and into the orbit along the
ophthalmic artery, via the superior orbital fissure.
The sympathetics pass through the ciliary ganglion
(located lateral to the optic nerve at the apex) without
synapsing.12
Parasympathetic innervation of the orbit: Parasympathetics innervate the iris sphincter muscle,
ciliary muscle, lacrimal gland and orbital blood
vessels to produce miosis, lacrimation and relaxation
of vascular tone. These inputs originate in the
Edinger-Westphal nucleus (third cranial nerve), the
salivatory nucleus via the nervus intermedius 10
(the parasympathetic nerve fibers originating from
the facial nerve), and the parasympathetic ganglia
supporting the orbit. Preganglionic parasympathetics
course with the oculomotor nerve, along its inferior
division, and enter the orbit via the inferior orbital
fissure. These fibers run superficially in the
oculomotor nerve as it exits the brainstem adjacent
to the posterior communicating artery. Thus,
aneurysms of the posterior communicating artery
may produce a third nerve palsy with an associated
dilated pupil. These nerves synapse in the ciliary
ganglion and enter the globe as the short posterior
ciliary nerves.

The ciliary ganglion lies adjacent to the lateral
aspect optic nerve at the orbital apex. The ganglion
also contains sympathetics that travel into the orbit
via the ophthalmic artery to reach the iris dilator
and ocular blood vessels, as well as sensory fibers
from the nasociliary nerve which supply intraocular
structures. Neither the sympathetics nor the sensory
fibers synapse within the ganglion.
Preganglionic fibers from the facial nerve nucleus
pass through the geniculate ganglion and then travel
with the greater petrosal nerve to eventually synapse
within the pterygopalatine ganglion. These fibers
course via the infraorbital fissure to the orbit and
directly to the lacrimal gland. 37, 38 Additional
postganglionic fibers travel along branches of the
maxillary division of the trigeminal nerve that
converge with the lacrimal nerve to enter the orbit.
Vascular Anatomy of the Orbit: Arterial Supply

The ophthalmic artery, the first intracranial branch
from the internal carotid artery, provides most of
the blood supply to the orbit and globe. The
ophthalmic artery arises just as its parent vessel exits
the cavernous sinus, just inferior to the optic nerve
and posterior to the anterior clinoid process. It
immediately joins the optic nerve along its
inferolateral surface, traveling within a common
dural sheath, and entering the apex via the optic
foramen.53, 54 Once inside the orbit, the artery crosses
medially and gives off its major apical branches
(Figure 1.18).
The major intraconal vessels include the central
retinal artery, branches to the extraocular muscles,
and the long and short posterior ciliary arteries. The
first branch of the ophthalmic artery is the central
Figure 1.18: Lateral view of the orbit and major branches of the ophthalmic artery

retinal artery. This vessel typically pierces the nerve
inferomedially, at a point approximately 10 mm from
the globe, and as it reaches the globe, gives off the
end arteries to the retina. Branches to the extraocular
muscles show greater individual variation in
distribution. Generally, these vessels run within the
muscle belly or along their medial surfaces. As they
continue to travel anteriorly with the muscles, the
terminal branches of these arteries enter the globe
at the tendinous muscle insertions, becoming the
anterior ciliary arteries. These branches provide
anastomoses with the long posterior ciliary arteries
to supply the iris, ciliary muscle and other anterior
intraocular structures. Because of this contribution
of the muscular arteries to the anterior segment,
disinserting more than two extraocular muscles from
the globe during surgery at one time is generally
avoided.
Typically, there are two or three posterior ciliary
arteries which branch from the ophthalmic artery near
the apex and run medially and laterally within the
orbit. Some of these vessels divide into 15-20 short
posterior ciliary arteries which enter the posterior
aspect of the sclera to supply the choroid and the
optic nerve head. Others, the two long posterior
ciliary arteries, continue to travel anteriorly within
the sclera, entering the globe medially and laterally
to supply the anterior segment and anastomosing
with terminal branches of the muscular arteries.
The major extraconal, apical branches of the
ophthalmic artery include the lacrimal and posterior
ethmoidal arteries. The lacrimal artery, along with
the lacrimal nerve, runs above the superior border
of the lateral rectus to reach the lacrimal gland and
lateral upper lid. It anastomoses with the middle
meningeal artery via the recurrent meningeal artery,
and the temporal arteries. The posterior ethmoidal
courses medially, along the frontoethmoidal suture
to exit via the posterior ethmoid foramen where it
gives off branches supplying the sinus and nasal
mucosa and the frontal dura.54, 55
As the ophthalmic artery continues forward in
the orbit, it then gives rise to the anterior ethmoidal
artery and finally, its terminal branches. The anterior
ethmoidal vessel exits via the anterior ethmoidal
foramen to supply frontal dura and ethmoid and
frontal sinus mucosa. Anastomoses from this
circulation and branches of the external carotid
provide blood flow to the nose and septum. The

frontoethmoidal suture, along which the anterior and
posterior ethmoidal arteries and associated branches
of the nasociliary nerve run, is an important landmark
for the roof of the ethmoid, or fovea ethmoidalis
which lies just beyond this line. Penetration of the
medial wall above this suture would allow
communication between the anterior cranial fossa and
the orbit. Additionally, the posterior ethmoidal
foramen characteristically lies 6 mm anterior to the
optic canal and 12 mm posterior to the anterior
ethmoidal foramen.2
The terminal branches of the ophthalmic artery
are the supraorbital, supratrochlear, dorsal nasal and
the medial palpebral arteries. The supraorbital and
supratrochlear arteries provide the blood supply to
the forehead and medial lids, while the dorsal nasal
and medial palpebral arteries supply the medial lids
and nose. The supraorbital artery travels above the
levator via the supraorbital notch or foramen and
should be carefully avoided during surgical
dissection of the orbital roof. All of these vessels
anastomose extensively with external carotid
branches to the face.
It is clear that there is great degree of collateral
flow to the orbit and lids between the internal and
external carotid circulation. Therefore, a review of
relevant branches of the external carotid artery,
namely branches of the maxillary artery, is also
important. Superiorly, the superficial temporal artery
provides blood supply to the forehead, anastomosing
with the circulation of the supraorbital and
suprotrochlear arteries. The angular artery provides
anastomoses with the dorsal nasal and palpebral
arteries medially. Within the orbit itself, the
sphenopalatine artery, like the ethmoidal circulation,
supplies the nasosinus mucosa and nasal septum. The
superficial branches of the infraorbital artery
anastomose with the inferomedial palpebral arteries,
while the deeper branches anastomose with the
muscular arteries. The anastomosis between the
lacrimal artery and the middle meningeal artery has
already been discussed. This occurs via the recurrent
meningeal artery, which enters the orbit through the
sphenoid, through a foramen superolateral to the
superior orbital fissure or directly via the fissure
itself.2
Vascular Anatomy of the Orbit: Venous

Outflow
The venous drainage pathways of the orbit run
independently of the arteries and are a completely
valveless system. There are three major outflow
systems, involving the cavernous sinus, pterygoid
plexus, and an anterior venous system which drains
via the facial vein (Figure 1.19). The superior
ophthalmic vein provides outflow from the
superifical, superior periorbital and orbital regions,
via the supraorbital, nasofrontal and angular veins.
It can be divided into three segments as it runs
anterior-posteriorly. The first segment courses
adjacent to the trochlea and along the medial edge
of the superior rectus. The second passes inferior to
the muscle and into the cone. This segment receives
the ciliary and superior vortex veins from the globe.
The third portion of the superior orbital vein travels
along the lateral edge of the superior rectus and exits

the orbit via the extra-annular superior orbital fissure
to drain into the cavernous sinus.
The inferior ophthalmic vein drains the inferior
orbit, including tributaries from the inferior rectus
and oblique muscles and from the inferior vortex
veins. The inferior ophthalmic vein anastomoses with
a branch of the superior ophthalmic vein. A portion
of the outflow is directed into the pterygoid plexus
and the rest directly into the cavernous sinus.
Anteromedially, venous drainage occurs mainly via
the angular and facial veins. Because of the high
degree of anastamoses and absence of valves,
some degree of venous obstruction can be
redirected within the system. However, acute
thromboses, particularly of the cavernous sinus, cause
marked orbital congestion and subsequently,
exophthalmos.
Figure 1.19: Venous drainage system of the orbit
Paranasal Sinuses
There are four pairs of paranasal sinuses: the frontal,
ethmoid, sphenoid and maxillary sinuses which
directly neighbor the orbital roof, medial wall, and
floor. Knowledge of their anatomy is useful since
these spaces can share disease processes with the
orbit such as infection and tumors, and can also
provide surgical access to the orbit and lacrimal
system.
The frontal sinus overlies the anterior portion of
the orbital roof and drains into the frontonasal duct
which travels though the anterior portion of the
ethmoid sinus (ethmoid infundibulum) to empty into
the middle meatus within the nose. This sinus
develops in childhood and is usually difficult to
appreciate radiographically until age 7 or so.
Pneumatization is typically completed by early
adulthood.56 The frontal sinus is a common site for
mucocele formation.57
The ethmoid sinuses lie between the medial
orbital walls and immediately posterior to the nose.
The lateral wall of this sinus is comprised of the very
thin lamina papyracea, which is easily fractured in
surgery or trauma, or compromised by local infection.
A frequent source of orbital cellulitis is ethmoid
sinusitis which spreads secondarily. The ethmoid
roof, or fovea ethmoidalis, is located just beneath
the anterior cranial fossa and just medial to the
frontoethmoidal suture line within the orbit. The
most medial portion of the ethmoid roof is the
cribriform plate, which overlies the nasal cavity
(Figure 1.20). The sinuses themselves are comprised
of many individual, thin-walled air cells and can be
divided into three groups. The anterior and middle

air cells drain into the middle meatus while the
posterior air cells empty into the superior meatus of
the nose. In performing dacryocystorhinostomy to
create a passage between the lacrimal sac and nose,
the ethmoid air cells are frequently encountered
extending anterior to the posterior lacrimal crest.58,59
The sphenoid sinuses are located midsagittally
and posterior to the ethmoid air cells. Like the frontal
sinuses, they pneumatize relatively late in life and
do not reach full size until adolescence. Drainage
occurs via the sphenoethmoid recess located in the
anterior sinus wall. Because the contents of the orbital
apex and nearby cavernous sinus exit the orbit
through the sphenoid bone, the walls of the sphenoid
lie in close proximity to a number of critical
structures. Anteriorly and superolaterally, the optic
nerve and intracavernous portion of the internal
carotid artery run along the lateral sinus walls. Severe
sphenoid sinusitis can therefore cause optic nerve
injury. 60 Likewise, congenital dysplasia of the
sphenoid, as can occur in neurofibromatosis type 1,
can produce pulsatile proptosis.61, 62 The sphenoid
sinus also provides a useful surgical approach to the
pituitary fossa which is located posteriorly to the
sinus.63
The maxillary sinus underlies the orbital floor
and is the largest of the paranasal sinuses. Drainage
from this sinus occurs via the maxillary ostium into
the middle meatus. The ostium is located high within
the medial sinus wall, close to the level of the orbital
floor. Thus, trauma to the orbital floor (i.e. orbital
fracture or inferomedial decompression) can obstruct
drainage from the sinuses. Inside the medial walls
of the maxillary sinus, lie the bony nasolacrimal
canals. The posterior most aspect of the sinus extends
from the area of the infraorbital fissure, and the
infraorbital nerve and artery run along the maxillary
roof within the infraorbital canal. Behind the
maxillary sinus is located the pterygopalatine fossa
and the maxillary artery runs in its posterior wall.
Conclusion
Figure 1.20: Computed axial tomography illustrating the relationship

between the anterior cranial fossa, orbit and ethmoid sinus
The orbit and its surrounds represent a complex

anatomical space, incorporating critical ocular, neural,
and vascular structures. The purpose of this chapter
has been to provide an overview of orbital anatomy,
as well as basic anatomy of the eyelids, lacrimal

system, and paranasal sinuses. A detailed
understanding of this anatomy is fundamental to
oculoplastic surgery and the management of orbital
disease.
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CHAPTER
Clinical Approach
to Proptosis
Before we discuss about proptosis let me remind you

that we have to distinguish it from certain conditions
which resemble proptosis (pseudo-proptosis) like
unilateral high myopia, buphthalmos, unilateral lid
retraction, unilateral minimal ptosis of contralateral
eye.
Though advances in imaging techniques have
revolutionized the diagnosis of orbital diseases,
proper clinical evaluation of proptosis is still very
important because it gives us the insight into the
disease process and helps in evaluating the CT/MRI
and arriving at a correct diagnosis. I follow the
Figure 2.1: Orbital abscess
Figure 2.3: Carotid-cavernous fistula
9 Ps, an extension of the 6 Ps of Krohels. The

9 Ps are: Pain, Progression (from history), Proptosis,
Pulsations, Pupil, PBCT, Perception of color vision,
Periorbital changes (inspection) and Palpation.
Pain: When severe pain is the presenting
symptom in proptosis, we have to consider the
following conditions: infection and inflammatory
lesions like orbital cellulitis, orbital abscess
(Figure 2.1), myocysticercosis, vascular conditions
like lymphangioma (Figure 2.2), high flow
carotid- cavernous fistula (Figure 2.3). Metastatic
lesions (Figure 2.4) are also very painful.
Figure 2.2: Lymphangioma
Figure 2.4: Metastasis from thyroid carcinoma

Retinoblastoma (Figure 2.5), and rhabdomyosarcoma
(Figure 2.6) can be very painful and mimic orbital
cellulitis. Thyroid orbitopathy which is usually
chronic, can rarely present acutely and can be
very painful. (Figures 2.7 and 2.7A) Moderate pain
is a feature of idiopathic orbital inflammatory
syndrome, myocysticercosis, (Figures 2.8 and 2.8A)
ruptured dermoid cyst, while dull boring pain is
associated with bone-erosion usually due to
neoplastic tumors (Figures 2.9 and 2.9A). Pain can
be a feature of proptosis following trauma (Figures
2.10 and 2.10A)
Proptosis following trauma can be immediate

(due to retrobulbar hemorrhage or surgical
emphysema) or delayed due to carotid cavernous
fistula. I came across a single case of pulsatile
proptosis following trauma, due to herniation of
brain through fractured roof of orbit.
Figure 2.5: Retinoblastoma
Figure 2.6: Rhabdomyosarcoma
Figure 2.7: Acute thyroid orbitopathy with chemosis and exposure

keratopathy. Notice the lid retraction of left eye
Figure 2.7A: CT showing enlarged recti with sparing of tendons
Figure 2.8: Myocysticercosis presenting as ptosis and proptosis

with pain. Note the periocular inflammatory response
Figure 2.8A: CT scan of the orbit showing cystic lesion involving

SR-LPS complex with hyper dense spot in the cyst (Scolex)
Clinical Approach to Proptosis 25
Figure 2.9: Eccentric proptosis with globe pushed down.

Note fullness of lacrimal gland region
Figure 2.9A: CT scan orbit showing a mass in the fossa of

lacrimal gland. Note the bony erosion and heterogenisity of the
mass (Adenoidcystic carcinoma of lacrimal gland)
Figure 2.10: Acute proptosis following Trauma. Note

severe chemosis, exposure keratitis with hypopyon
Figure 2.10A: After anterior orbitotomy shows

complete recovery. Vision improved to 20/30
Progression: The onset of proptosis can be acute

(hours to week), subacute (1 to 4 weeks) or chronic
(more than 1 month). Acute proptosis can be due to
infections, inflammations, parasitic infestations,
trauma, metastatic lesions or lymphangioma.
Subacute presentation is common in inflammations,
parasitic infestations or metastatic neoplasia.
Figure 2.11: Metastatic orbital lesion presenting subacutely.

Note the inflammatory changes
(Figure 2.11) Chronic presentation is commonly due

to thyroid associated orbitopathy (Figure 2.12),
orbital varices or benign neoplasia like cavernous
hemangioma, (Figures 2.13 to 2.13B), Neurofibroma
(Figures 2.14 to 2.14E), Schwannoma (Figures 2.15
to 2.15B), Glioma of Optic Nerve, (Figures 2.16 to
2.16C). Chronic presentation is characteristic of most
of the primary neoplasia of the orbit, both benign
and malignant. However, if the presentation is less
than 6 months, consider the possibility of a malignant
lesion.
Figure 2.12: Thyroid associated orbitopathy with chronic presentation. Note the lid retraction and lateral flare of right upper lid. Note the
absence of congestion and edema
Figure 2.13: Axial proptosis of left eye 3 yrs. Notice

how quiet was the globe
Figure 2.13A: CT scan showing well encapsulated tumor

(Cavernous hemangioma)
Figure 2.14: M 52, RE proptosis since 3 yrs,

Def. Vision 1yr. RAPD +, VA : 20/200
Figure 2.13B: Excised tumor
Figure 2.14A: CT shows a large well defined

mass with bony expansion
Figure 2.14B: Extension of tumor into superior

peripheral space pushing the globe down
Figure 2.15: F 30, presented with proptosis of 3 yrs.

Defective vision(20/800) RAPD +ve
Figure 2.14C: Well encapsulated tumor on gross exam
Figure 2.15A: CT scan showing a well encapsulated

intraconal mass. Note the excavation of lateral wall
Figure 2.14D: Spindle-cells of neurofibroma
Figure 2.14E: Postoperative status recovery from proptosis. VA

improved to 20/40
Figure 2.15B: Excised tumor, proved to be schwannoma
Intermittent proptosis is usually due to idiopathic

orbital inflammatory syndrome, lymphangioma
(Figure 2.17), orbital varices and myocysticercosis

(in endemic areas). (Figure 2.18)
Figure 2.16: M 14 yrs, proptosis of right eye since 8 years.

RAPD + Vision absent PL
Figure 2.16A: CT scan orbit showing optic nerve glioma

with cystic degeneration
Figure 2.16B: Excised tumor

Figure 2.16C: Postoperative status. No proptosis
Figure 2.17: Girl of 12 years presenting with recurrent episodes of

proptosis (3 in 5 yrs). Note the subconjunctival hemorrhage in the
proptosed LE (Lymphangioma)
Proptosis: Proptosis or protrusion of the eye ball

depends on the location of the orbital lesion. A lesion
in the intraconal space pushes the globe forwards to
cause axial proptosis (Figures 2.19 to 2.19B), where
as a lesion in the peripheral surgical space pushes
the globe to the opposite side and causes eccentric
Figure 2.18: CT scan showing an enlarged superior rectus muscle

with a cyst showing hyper-dense spot within (Myocysticercosis)
proptosis. However, since these surgical compartments are not strictly water-tight, a large intraconal
lesion can enter peripheral surgical space and cause
eccentricity to an otherwise axial proptosis (Figure
2.14 series).

Axial proptosis: Lesion is in the intraconal space.
In my experience the most common lesion of
intraconal space causing axial proptosis is
cavernous hemangioma followed by Schwannoma,
and neuro-fibroma. Optic nerve glioma and
meningioma of optic nerve sheath are important but
not very common lesions. Other lesions include orbital
varix, lymphangioma. The most common intraconal
cystic lesion is hydatid cyst (Figures 2.20 and
2.20A).
One has to remember that most of the intraconal
lesions can compress optic nerve and lead to visual
loss. Hence loss of vision in axial proptosis does not
necessarily mean that the patient had either optic
nerve glioma or meningioma of optic nerve sheath.
It can be due to any other lesion of intraconal space
like hemangioma, schwannoma, neurofibroma,
hydatid cyst or even idiopathic orbital inflammatory
syndrome. Optic nerve can also be compressed by
enlarged extraocular muscles as in thyroid associated
orbitopathy.
Down and out proptosis is due to lesions

of superomedial space (pushing the globe down
and out). Frontoethmoidal lesions are the most
common cause of such eccentric proptosis.
Mucocele (Figures 2.21A to C), fungal granuloma,
neoplastic lesions and fibrous dysplasia (Figures
2.22A and B) are the common lesions. Lesions of
supero-medial space like dermoids, hemangiomas can
also present with eccentric proptosis with globe
displaced down and out (Figures 2.23A and B).
Osteoma of ethmoid (Figures 2.24A to E) is another
rare cause.
Figure 2.19: Axial proptosis of left eye due to intraconal lesion
B
Figures 2.19A and B: CT scans of orbit showing intraconal tumor arising from optic nerve (Optic nerve glioma)
Figure 2.20: Intraconal hydatid cyst of orbit
Figure 2.20A: Cyst excised with cryo after aspirating fluid
C
Figures 2.21A to C: Eccentric proptosis with the eyeball pushed down and out. Notice the gross outward displacement
with fullness in the superomedial aspect. CT scan shows a huge fronto-ethmoid mucocele
Figures 2.22A and B: Eccentric proptosis with globe pushed down

and out due to fibrous dysplasia of frontal bone as demonstrated by
the CT scan (B)
Figures 2.23A and B: Coronal and axial sections of CT scan orbit showing a very large cystic lesion in the superomedial peripheral space
(blue arrow), displacing the Globe (green pentagon) down and out. Compare the size of the cyst with that of opposite eyeball. Note the bony
excavation of the roof and medial wall (red arrow). This is a case of Hydatid cyst of orbit
Figures 2.24A to C: This young male of 22 yrs presented with recurrent, eccentric proptosis of right eye since 1year, and defective vision
since 3 months. He underwent surgery elsewhere for similar lesion 2 years back. Note the periocular fullness, and lateral displacement of the
globe. He had RAPD and the vision was 20/40. CT scan of orbits revealed a huge osteoma involving the ethmoid bone (arrow)
E
D
Figures 2.24D and E: Excised ivory osteoma. The postoperative

recovery was uneventful. The patient's vision improved to 20/20

Down and in displacement of the globe is mostly
due to enlargement of lacrimal gland, due to infection
(Dacryoadenitis), inflammation (idiopathic orbital
inflammation, Mukulitz syndrome (Figures 2.25A
to C), or neoplasia. Pleomorphic adenoma (Figures
2.26A to D) is the most common benign tumor while
Adenoidcystic carcinoma of the lacrimal gland
(Figures 2.27A to D) is the most common and
dreaded malignant tumor of the lacrimal gland.

Other rare causes include lymphoma (Figures 2.28A
to D) pleomorphic adenocarcinoma, adenocarcinoma, squammous cell carcinoma and reactive
lymphoid hyperplasia. Other lesions of the fossa
of lacrimal gland like dermoids (Figures 2.29A to
D) can also cause eccentric proptosis with globe
displaced down and in.
Figures 2.25A to C: Female 55 years, presented with proptosis left eye of 18 months duration. Notice the globe was pushed down and in, with
fullness of outer half of left upper lid. The tear film was normal. CT scan of orbit showed bilateral enlarged lacrimal glands, molding to the globe
suggestive of lymphoma (B). The excised specimen (C) (note the concavity of medial surface of the excised specimen, which corresponds to
the globe) The histopatholgical diagnosis was Sjogren's syndrome
Figures 2.26A and B: This female 32 yrs. presented with proptosis of right eye since 2 years. Note the globe pushed down and in, and the
fullness of lacrimal gland region and the mass effect. CT scan of orbits showed a well encapsulated mass lesion of lacrimal gland. The
surrounding bone was normal. Clinical diagnosis was pleomorphic adenoma of lacrimal gland
D
Figures 2.26C and D: The mass was excised through superior lid
crease incision. Note the well encapsulated tumor (C) and well hidden incision in the lid crease (D) Histopathology confirmed it to be
pleomorphic adenoma of lacrimal gland
Figures 2.27A to D: This female 28yrs. presented with eccentric proptosis of 5 months duration. Note the fullness at lacrimal gland region. The
globe was pushed down and in (A) CT scan revealed a lacrimal gland tumor with irregular surface and bony erosion (B). It was a case of
adenoid cystic carcinoma of lacrimal gland for which exenteration was done (C). Postoperatively the patient was doing well and there was no
recurrence after 8 years (D)
Figures 2.28A and B: Note the fullness of left upper lid with globe
pushed down and in (A). CT scan of orbit shows enlarged lacrimal
gland, molding to the globe (B)
Figures 2.28C and D: The tumor was excised through Sub-brow

incision (C). Histopathology revealed it to be a case of lymphoma. 3
days postoperative photo showing marked improvement from proptosis (D)
B
A
Figures 2.29A to D: Female 25 years, presented with eccentric proptosis of right eye since, 2 years, She had 2 episodes of pain and
worsening of proptosis in the past 6 months. Note the fullness of the upper lid, and the globe which was pushed down and in (A) Axial and
coronal sections of the CT scan show a cystic lesion with variable consistency, situated at the fossa of lacrimal gland (B and C). Excised
dermoid cyst (D)
Upward displacement of globe is due to either

an extension of lesion of maxillary sinus into orbit
(neoplasia, fungal granuloma or dumbbell dermoid)
or lesions of inferior space like cavernous
hemangioma, neurofibroma, schwannoma, cystic
lesions like myocysticercosis involving inferior rectus
(Figures 2.30A to C). Many a time lesions of maxillary
sinus extend into frontoethmoidal sinuses, or nose.
Anterior extension leads to fullness of cheek (Figures
2.31A to C). It is my experience that orbital extension
of lesions from paranasal sinuses is a common cause
of eccentric proptosis.
Measurement of proptosis is a very important
part of evaluation of the patient. The displacement
of the globe should be quantified in all the 3
directions, anteroposterior (axial), horizontal and
vertical axes. Naphzeigers test is a useful bedside
clinical test to detect mild proptosis (Figures 2.32A

and B). Ask the patient to look at a distant object,
located at the same level as that of the patients eye.
Stand behind the patient, and gently tilt the head
backwards and look down over the patients forehead.
The proptosed eye appears ahead of the other.
Another simple bedside test to detect mild
proptosis is with the help of a scale. Ask the patient
to gently close the eyes, and keep a scale across the
eye in contact with his forehead and cheek. Normally
there will be space between the scale and the closed
lid. In the presence of proptosis this space is
obliterated (Figures 2.33A and B).
Axial proptosis is best measured with Hertel's
exophthalmometer (Figure 2.34A). Leudde's
exophthalmometry is less accurate and inter-observer
variation is more significant.
Figures 2.30A to C: This female, 42 years in age, presented with

proptosis left eye, which was displaced upwards (A). Note the mass
lesion in lower lid, which was transilluminant (B). This demoid cyst
was removed through swinging lower lid approach. Note the minimal
swelling of lower lid on 2nd postoperative day (C)
Figures 2.31A to C: This child presented with proptosis of right eye since 2 years. Note the fullness of cheek, and gross upward displacement
of the globe due to lesion of the Maxillary sinus (A). CT scan of the orbit (B and C) revealed it to be ossifying fibroma. Note that the mass is
involving the nasal cavity (red arrow) and the oral cavity (blue arrow)

compare results over a period of time. When once the
BR is recorded, subsequent readings should be taken
with the same BR so that the results are comparable.
Figures 2.32A and B: Naphzeigers test: Note the prominence of

left eye (arrow). The right eye is not visible
Another very important factor you have to

remember is that while performing exophthalmometry, you should occlude the unexamined eye
with your thumb, (Figure 2.34C) so that the eye being
examined comes to the primary position. Then only
the readings are accurate.
Figure 2.33A: Note the space between the closed

right eye and the scale in a normal eye
Figure 2.33B: Note the obliteration of space. The scale is in

contact with the eyelid of proptosed left eye
Hertels exophthalmometry consists of 2 foot

plates - one is fixed and the other sliding. Each foot
plate has a viewing mechanism (Figure 2.34B) where
the eye being examined is seen in profile below a
scale in millimeters (Figure 2.34D). There are 2 red
lines for reference to avoid parallax error. Rest the
fixed footplate on the anterior part of lateral wall of
right orbit and slide the other footplate on the scale
till it rests on the anterior part of lateral wall of left
orbit. Note the distance between the 2 foot plates
from the scale. This is called the Base Reading (BR).
It is very important to record the base reading since
the exophthalmometry values change with base
reading. In other words, if you take 2 readings on
the same patient one after the other with 2 different
base readings, the values you get will be different.
In fact the one with wider BR will give larger value!
If you forget to note the BR, you can not accurately
To measure the horizontal displacement of the

eye, put a mark on the center of root of the nose.
Measure the distance from that point to the nasal
limbus of the proptosed eye, with the other eye being
covered. Repeat the same for the normal eye,
covering the proptosed eye. The difference between
these two readings gives you the horizontal
displacement (Figures 2.35A and B).
Vertical displacement is measured with the help
of 2 rulers. Hold the first scale in line with the lateral
canthi. Measure the distance from this scale to the
6 O'clock limbus of each eye.
A larger reading of proptosed eye means that
the globe is pushed inferiorly, and a lesser reading
is obtained when the globe is displaced up (Figures
2.36A and B).
Pulsations: Pulsations of globe in proptosis can
be either vascular pulsations or transmitted
Figure 2.34A: Hertels exophthalmometer: It consists of 2 footplates, one fixed (blue arrow) and another sliding (red arrow)
which slides on a scale (green arrow). The distance between the 2 plates is the base reading (98 mm in this picture)
Figure 2.34B: Viewing system of each footplate. It has a red reference line in front (green arrow).Another redline (blue arrow) is seen through
the prism. You have to align both the redlines into a single line to eliminate parallax error. The eye ball is seen in profile and its position is read
from the scale (red arrow).
Figures 2.34C and D: Hertel's exophthalmometry. Note the unexamined right eye was occluded with the thumb, so that the eye being examined
(left eye) is in the primary position. The scale reading of the anterior most part of the cornea seen in profile (green arrow) is the axial position
of the globe
Figures 2.35A and B: Measurement of horizontal displacement of the eyeball. Ask the patient to fix at a distant object in the straight gaze,
covering the unexamined eye. Measure the distance from the central point of reference on the root of nose (red arrow) to the nasal limbus;
Compare the same with that of other eye. Lesser reading of the proptosed eye means that the globe was pushed medially (A: Central point of
reference drawn on the nose, B: Nasal limbus, C; Cover)
Figures 2.36A and B: First Scale is held joining the lateral canthi and is the point of reference. The second scale measures the vertical
displacement. Note that the proptosed left eye is displaced down and gives larger reading
pulsations. Vascular pulsations are due to increased

blood flow and is typically seen in CarotidCavernous Fistula (CCF). It is almost always seen in
high-flow CCF (Figures 2.37A to C). The other
important causes include meningioma (Figures 2.38A
to C), orbital varix, aneurysms, etc. Transmitted
pulsations are due to cerebral pulsations which are
transmitted due to bony deficiency as in
neurofibroma (Figures 2.39A and B). I have seen a

child who presented with pulsatile proptosis due to
herniation of frontal lobe of brain into orbit following
trauma (Figures 2.40A and B).
To detect pulsations observe from a side. Subtle
pulsations can be recognized while recording IOP
with an applanation tonometer.
Figures 2.37A to C: Note the caput medusae of conjunctival congestion with limitation of abduction of left eye (A). She has a pulsatile
proptosis, 3 months after head injury. The IOP was 28 mm of Hg. Bruit
was heard with the stethoscope (B). CT scan of orbit revealed a very
grossly enlarged superior ophthalmic vein (green arrow), typical of
carotid cavernous fistula (C)
Figures 2.38A to C: This lady of 58 years, presented with pulsatile

proptosis of right eye since 1 year (A). Note the temporal fullness,
(blue arrow) which was showing pulsations (B), CT scan revealed a
mass from temporal lobe and involving the sphenoid wing with extension in to orbit and the temple (C). Note the hyperostosis of Sphenoid
bone (red arrow) This is a case of meningioma
A
Figures 2.39A and B: This male 42 years of age, presented with
pulsatile proptosis of left eye since his childhood. Note the tumor
involving both the lids of left eye and temporal fullness (A). He had
lisch nodules on Iris and caif-au-lait spots typical of neurofibroma. CT
scan (B) shows defect in the roof of orbit (red arrow), which explains
the transmitted pulsations
B
Figures 2.40A and B: This child presented with pulsatile proptosis following head injury.
Note the herniated frontal lobe through the defect in the roof of orbit (arrow)
Pupil: Examination of pupil and its reaction is

very important. The presence of Relative Afferent
Pupillary Defect (RAPD) indicates that optic nerve is
being damaged. Optic nerve can be damaged either
due to tumors of the optic nerve or its sheaths, or by
compression due to any space occupying lesion of
central space, by enlarged extraocular muscles as in
thyroid associated orbitopathy, or by nonspecific
inflammations of the orbit. Due to narrowing of the
orbital space at the apex, a smaller lesion at the orbital
apex can lead to optic nerve compression. Optic nerve
damage is also seen in severe stretching of optic
nerve, as seen by "tenting" of the posterior pole on
CT scan.
Perception of Color Vision: This is an important,

simple and very sensitive way to know the status of
the optic nerve. This statement is relevant for the
following reasons. In very early optic nerve
compression the vision can still be 20/20. The patient
may not notice defective vision in the presence of
diplopia, watering and discomfort/ pain. In bilateral
cases, RAPD may not be elicited. During fundus
examination, very early Optic disc compression can
be missed. Visual field analysis may not detect any
abnormality in very early cases. Hence the
importance of color vision testing can not be over
emphasized. When a patient of proptosis is under
observation, as in a case of TAO, I instruct the patient

to look at a bright red colored object with each eye
separately on every sunday, and to report to me
immediately if he notices any change in brightness
of the color in one eye. If color vision is defective in
the proptosed eye, evaluate very carefully the pupil
for RAPD, do a very detailed fundus evaluation for
the presence of optic disc edema, pallor, presence
of opticociliary shunts, retinal/choroidal striae,
retinal detachment. Visual field assessment is
mandatory.
PBCT: Limitation of ocular motility in proptosis

is mostly due to restrictive pathology as in thyroid
associated orbitopathy (Figures 2.41A and B),
Idiopathic orbital inflammation (myositis component)
(Figures 2.42A to D), myocysticercosis (Figures 2.43A
and B), and fungal granuloma (Figures 2.44A to C).
Another important cause is CCF, wherein the
restriction is paralytic in nature (Figures 2.45A to
C). Large mass lesions can cause mechanical
restriction. Limitation of ocular motility following
A
Figures 2.41A and B: TAO: restricted elevation due to enlarged
inferior rectus (arrow)
Figures 2.42A to D: Female 42 yrs., presented with subacute proptosis of left eye with pain and diplopia. Note the congestion of left eye and
limitation of abduction (A). CT scan showed enlarged medial rectus involving the tendon. A diagnosis of idiopathic orbital inflammatory syndrome
was made. (Blue arrow) (B) She responded very well to systemic steroids. Note that the conjunctival congestion disappeared (C) and
abduction restored to normal within 1 week (D)
A
Figures 2.43A and B: This young girl presented with proptosis of
left eye since 4 weeks. She also complained of pain and diplopia.
Note the fullness of upper lid, mild congestion of conjunctiva on the
medial side and convergent squint. (A) CT scan of orbit revealed
myocysticercosis involving the superior oblique muscle. Note 2 cysts,
one involving the muscle belly and the other the reflected tendon of
superior oblique (B)
trauma can be due to soft tissue edema, entrapment

of muscle or its sheath in orbital fracture and rarely
due to injury to the muscle itself. I came across a
rare cause wherein after trauma; the displaced bone
itself caused limitation of motility (Figures 2.46A to
D). I routinely quantify the ocular motility in degrees,
just like Hirschberg's, so that it is easier to compare
the course of the disease over a long period.
Figures 2.44A to C: This elderly male, (A) an agricultural worker by

occupation, presented with gross proptosis with frozen globe (B).
His vision was PL. Retropulsion was positive. FDT was positive in all
directions. CT scan of the orbit revealed a heterogenous mass lesion
occupying the entire orbit. Note the molding of the lesion to the globe
(red arrow), the tenting of posterior pole (blue arrow) and involvement of anterior ethmoidal sinus. Histopathology showed it to be a
fungal granuloma (C)
When ocular motility is limited, I routinely do

FDT (Forced Duction Test) and differential
tonometry to know whether it is due to restrictive
pathology or paralytic. I prefer FDT to FGT
(force generation test).In restrictive pathology FDT
is positive and you feel resistance when you try to
move the globe in the direction of limitation.
Elevation of intraocular pressure by more than 5 mm
Figures 2.45A to C: This girl presented with diplopia and restricted

motility following trauma. Note subconjunctival hemorrhage in right
eye. (A) FDT was positive. Clinical diagnosis was blowout fracture
of floor of orbit with muscle entrapment. But CT scan of orbit revealed
fracture roof of orbit with the bony spicule mechanically restricting
the ocular motility. (B) Elevation restored to normal after removing the
bony spicule (C)
Figures 2.46A and B: Male 27 yrs. presented with proptosis right eye, diplopia on dextroversion and pain of 2 weeks duration. He had a
closed head injury 3 months prior to the onset of proptosis. Notice mild congestion of proptosed right eye (A), better seen in the close-up of the
eye (B)
Figures 2.46C and D: Notice the congested blood vessels and the subtle under action of the lateral rectus of right eye (as evidenced by
the over action of medial rectus of left eye). (C) The diagnosis of A-V Fistula is confirmed by the engorged Superior ophthalmic vein (D)

from the base pressure when the patient attempts to
move the globe in the direction of limitation of
movement is significant. This occurs in restrictive
pathology as the globe is compressed between the
constricting muscle and its opponent which can not
relax. Quantify the motility restriction, with PBCT
(Prism Bar Cover Test) in different directions.
This helps to know what is happening to the
extraocular muscles, especially when you have to
follow a patient for a long time like thyroid
associated orbitopathy, and to decide if the patient
benefits with prescription of prisms.
Periorbital Changes: There are numerous
periorbital changes which help us in understanding
the pathology of the lesion and aid in clinical
diagnosis. Some of these make the diagnosis very
obvious (like the lid retraction of thyroid associated

orbitopathy, Salmon patch of lymphoma, temporal
fullness of sphenoid ridge meningioma) while
others throw light into the disease process and its
activity.
Temporal Fullness: This is very characteristic of
sphenoid wing meningioma, especially of lateral part
(Figure 2.36) Sphenoid wing meningioma of lateral
half typically presents as proptosis with fullness of
the temple, which often exhibits pulsations. Sphenoid
wing meningioma of medial half presents as proptosis
with restricted ocular motility (Figures 2.47A and B).
Another very rare cause of temporal fullness is a
dumbbell dermoid (Figures 2.48A and B), in which
eyeball can move forwards on mastication due to
contraction of temporalis muscle.
Figures 2.47A and B: Note proptosed left eye with restricted adduction. All movements other than abduction were restricted (A). CT scan of
orbit revealed a heterogenous mass lesion with calcification, involving the temporal lobe, medial half of sphenoid wing. Note the hyperostosis
of sphenoid bone (arrow), suggestive of meningioma (B)
Figures 2.48A and B: Dumble dermoid with temporal fullness. Note the fullness of temple and the medial displacement of the globe (A).
CT scan shows dumble dermoid, extending into the temporal region with a big defect (blue arrow) in the lateral wall of the orbit (B)

Lid changes: The important lid changes include
lid retraction, lid lag, sinuous lid margin, tumors of
the lid (primary malignant tumors of the lid with
secondary orbital extension, primary vascular
neoplasia with components of orbit and lid, or
multiple tumors involving the lids and orbit like
neurofibromatosis (Von Recklinghausen)).
Lid Retraction is the most common lid change
seen in thyroid associated orbitopathy. It can involve
both upper and lower eyelids. It is measured by
recording MRD values and subtracting the normal
values (4 mm and 5 mm) from it. The lid retraction
of TAO is due to over action of sympathetic system
(Mller's muscle) and can also be due to hypotropia
and limitation of elevation, so that with the attempt
to move the globe, the upper lid goes up and lid
retraction worsens. The lid retraction can be
unilateral or bilateral. As per the NOSPECS
classification, the lid retraction is classified as follows:
mild if the lid margin is at limbus, moderate if up to
4 mm of sclera is seen and severe if > 4 mm of sclera

is seen (Figures 2.49A to C). Usually in TAO, the
contor of lid is altered in that the highest point of
the upper lid is at the lateral part (lateral flare).
Lid lag: Lid lag is the second most common lid
change in thyroid orbitopathy. It can be unilateral
or bilateral. Lag-ophthalmos is a very important
finding one has to look for. It can lead to exposure
keratopathy which in the early stages can present
with pain and photophobia (Figures 2.50A and B).
There may be associated defective vision. If the
lagophthalmos is severe and prolonged, it can lead
to frank corneal ulcer and even perforation. Hence
it is very important to detect lag ophthalmos as early
as possible and take remedial measures. To detect
early lagophthalmos, look from below (Figures 2.51A
and B). Severe lagophthalmos is usually seen in very
gross proptosis which can be due to a very large
benign tumor, but more often due to faster growing
lesions like metastatic lesions (Figures 2.52A to C).
C
Figures 2.49A to C: Lid retraction three grades in thyroid associated orbitopathy. Mild-upper lid is at the limbus (A)
Moderate 2 mm of scleral show (B) More than 4 mm of scleral show (C)
B
Figures 2.50A and B: Lag-lag: Unilateral (left eye) and bilateral in thyroid associated orbitopathy.
Note a small corneal lesion at 6 O clock position due to exposure
B
Figures 2.51A and B: Mild lagophthalmos may not be detected (A) unless examined from below (B)
Figures 2.52A to C: Mild lagophthalmos in a case of TAO (A), severe lagophthalmos in metastatic orbital lesion from carcinoma of thyroid
(B). The elderly female (a case of hydatid cyst of orbit) had anterior staphyloma in her left eye due to perforated corneal ulcer (C)

Mass: Look at the details of any visible mass
(Figures 2.53A and B), like its surface, look for any
vasculature, transillumination (Figure 2.30B), the
margins, and posterior extent of the borders.
Measure the size of the mass lesion. Some times there
can be more than 1 mass lesion as in Von
Recklinghausen (Figures 2.55A to C). The mass can
have an orbital and a lid component (Figures 2.54A

and B).
Conjunctival changes: The common conjunctival changes in proptosis are Salmon patch,
chemosis, caput medusae, and subconjunctival
hemorrhage. Occasionally tumor components may
be visible.
B
Figures 2.53A and B: Oncocytoma of lacrimal gland presenting as proptosis with a bleeding tumor involving the left upper lid (A).
Proptosis in a patient of xeroderma pigmentosum due to orbital extension of squamous cell carcinoma (B)
B
Figures 2.54A and B: Capillary hemangioma involving the eyelid (A) and with its orbital component seen through the conjunctiva (B)
Figures 2.55A to C: Neurofibromatosis can present either as Von Recklinghausen disease with multiple tumors on the eyelid (A),
or as plexiform neurofibroma (B) involving the lid and orbit as seen in the CT scan (C)
Figures 2.56A to C: Malignant lid tumors, when neglected, can extend into orbit as seen with the Meibomian Carcinoma lower eyelid of
right eye (A). Squamous cell carcinoma of right upper lid with orbital extension demonstrated in the CT scan (B and C)
Salmon patch is a pinkish, smooth mass, typically

seen in the subconjunctival plane, either at the limbus,
or fornix (Figures 2.57A to C). It is very typical of
lymphoma. The lesion at the fornix, which is more
commonly seen, is due to the extension of the tumor,
as it moulds to the adjacent globe and extends
anteriorly along the sub-tenon's plane and can extend
upto the limbus. The isolated limbal mass is due to
proliferation of the lymphocytes.
Caput medusae: Engorged blood vessels, typically

around the limbus are seen in AV malformations and
fistula (Figures 2.58A and B). The engorgement is
usually due to increased venous pressure due to AV communication. It can be very subtle and can be
easily over looked by the novice. In high flow fistula
the caput medusae can be very significant and point
towards the diagnosis. Some times it can be
associated with chemosis of conjunctiva.

Chemosis: Chemosis is divided into 3 grades,
grade 1 is when the chemosed conjunctiva covers up
to half the lid margin, grade 2 when it covers the
entire lid margin and grade 3 when it overhangs the
lid margin (Figures 2.59A to C). Conjunctival
chemosis can be because of active infections and

inflammations like orbital cellulitis, orbital abscess,
thyroid orbitopathy, or due to high flow arteriovenous communications, lymphangioma (Figures
2.60A to C)
Figures 2.57A to C: Salmon patches of varying degrees presenting at the fornix and some extending up to limbus.
Notice the typical color and also varying degrees of vasculature above them
B
Figures 2.58A and B: Caput medusae due to CCF (A). Note the grossly engorged superior ophthalmic vein
(blue arrow) in the CT scan. Compare its size with the normal (red arrow)
Figures 2.59A to C: Chemosis grade1 (covers upto half of lid margin) (A), grade 2 (covers upto entire length of lid margin) (B)
and grade3 (overhangs the lid margin) (C)
Figures 2.60A to C: Grade 3 chemosis in orbital cellulitis (A), carotid-cavernous fistula (B) and a case of lymphangioma (C)
Subconjunctival hemorrhage is an important

feature of lymphangioma, leukemia, trauma and
bleeding diathesis. In a case of trauma, apart
from associated ocular and systemic injuries,
look for RAPD, retrobulbar hemorrhage and
orbital fractures. If retrobulbar hemorrhage is
present, do canthotomy and cantholysis (Figures
2.61A and B).
Val salva: Increase in proptosis after Val salva
maneuver is typically due to Orbital Varix (Figures
2.62A to C)
Examination of nasal cavity and oral cavity is
mandatory, especially in the presence of paranasal
sinus involvement (Figures 2.63A and B). It is much
easier to get a biopsy done from these lesions to
know the nature of the lesion.
Other important periocular changes involving
cornea, ocular motility, pupil, visual acuity, fundus
examination, color vision were already mentioned
earlier.
Palpation: Palpate the orbital margin, look for

any palpable mass lesion and assess the orbital
pressure by retropulsion. While palpating the orbit,
ask the patient to look in the direction in which you
are palpating, so that the orbital septum is relaxed.
Use the pulp of your finger to palpate the orbit for
any mass. If a mass is palpable, note its consistency,
tenderness, extent, surface, reducibility, posterior
extent. Assess the orbital tone by gently applying
pressure over the closed eyelids and pushing the
globe into the orbit. Compare the resistance offered
by the proptosed eye with that of the normal. From
this you can know if the orbital lesion is compressible
or unyielding mass.
Auscultation: In pulsatile proptosis, auscultate
with the bell of a stethoscope for any bruit. It is
typically heard in high flow fistula.
Common causes of bilateral proptosis in children
are congenital skeletal deformities, followed by
lymphoma, leukemia and other lymphoproliferative
Figures 2.61A and B: Acute proptosis with subconjunctival hemorrhage, and surgical emphysema following trauma (A). Note that the
conjunctiva became flat after 2 snips were made into it to permit the escape of air. Lateral canthotomy and cantholysis was performed to drain
the retrobulbar hemorrhage (B)
Figures 2.62A to C: Female 21 years presented with proptosis of right eye since her childhood (A) She gives the history that the proptosis
gets worse when ever she bends. Notice the increase in the amount of proptosis, fullness of upper lid, (B) and increase
in the volume of the vascular component in a case of orbital varix (C)
Figures 2.63A and B: Nasal examination and oral examination can show the extension of lesions, especially if they arise from the Maxillary
sinus. Note the visible mass in the left nostril (A) and the swelling of hard palate (B) which is the floor of maxillary sinus
disorders. Other important secondary orbital lesions

that cause bilateral involvement, though not
necessarily at the same time, include squamous cell
carcinoma of the conjunctiva associated with
xeroderma pigmentosum, and neglected retinoblastoma. The most common cause of unilateral
proptosis in children is dermoid cyst, followed by
hemangiomas. Rhabdomyosarcoma is the most
common malignant lesion of pediatric age group. In
adults, the most common cause of proptosis, either
unilateral or bilateral is thyroid associated
orbitopathy. Nonspecific orbital inflammation,
granuloma (especially fungal granuloma involving
frontoethmoid sinuses) and lymphomas are the other
common bilateral lesions of orbit. However, the
frequency of these conditions can change from place
to place, depending upon the disease patterns of those
areas. For example in India we see a lot of cases of
orbital myocysticercosis, which many of you in the

western world might not have come across. Similarly,
we rarely come across Wagener's granulomatosis,
which is fairly common in the west. The disease
presentations also can vary in different parts of the
world. When compared to the west, we in South India
very rarely come across severe thyroid orbitopathy.
The incidence of infection due to tuberculosis is on
the rise because of HIV. So is the case with lymphoma.
Thus the spectrum of orbital diseases is varied in
different parts of the world (Table 2.1). With a good
knowledge of the disease patterns of the region and
a good clinical work-up, a reasonable clinical
diagnosis can be made most of the times. Advances
in investigative modalities like imaging (CT, MRI)
,histopathology (FNAC, IHC, Squash) come to our
aid to make a very accurate diagnosis, which goes a
long way in managing the case.

Table 2.1: Etiology of proptosis in comparision with western literature (Incidence in percentage)
Lesion
Subrahmanyam
Rootman
Hendserson
Richard Dallow
1578
3919
1376
1825
Systemic
Inflammatory
Trauma
Congenital
Primary neoplasia
Secondary neoplasia
Metastatic lesions
Vascular lesions
Others
12.2%
37.1%
4.9%
2.8%
16.6%
1.9%
1.3%
3.0%
20.2%
51.7%
8.6%
4.9%
4.9%
14.3%
2.3%
1.5%
4.6%
7.2%
3.8%
4.4%
2.8%
48.1%
19.5%
8.1%
4.7%
8.6%
32 %
13%
2%
2%
6%
2%
3%
6%
34%
This table shows considerable difference in the etiology of proptosis, when I compared my series with 3 large series reported
from "clinical practice". I am not comparing with the reported series from pathology records, which have a strong bias towards
neoplastic lesions. Hence knowledge of disease pattern for the area, from which the patient has come, is very important. In
India we come across compressive optic neuropathy due to thyroid associated orbitopathy very rarely, whereas at vancouver
I noticed that its prevalence among the locals of Indian origin is similar to the caucasians. It may be due to the gross difference
in the climatic conditions or the life style and needs to be investigated.
Evaluation of a Case of Proptosis

Name:
Age:
Occupation:
Address:
Sex:
Regn. Number
Presenting complaints:
Treatment history
Past History:(Circle the ones relevant)
Thyroid disease HIV
Any Medications /
anticoagulants
Tuberculosis
Diabetes
Any Surgeries
Syphilis
Hypertension Any known drug
reactions : List them
Trauma history:
Family history:
Personal history:
General systemic examination:(circle the ones
relevant)
Pulse:
Pallor
Freckles
BP:
Icterus
Neck Swellings
RR:
Lymphadenopathy Tremors
Temperature: Caif au lait spots
Other history: (circle the one relevant): Thyroid

disease (Wt loss/gain, tremors, neck swellings,
palpitations, others), sinusitis, aggravation with
.
respiratory infections, others
CVS:
Respiratory System:
Abdominal Examination:
ENT Examination:
History of present illness:

Proptosis : Onset and Progression
Defective vision:
Double vision:
Pain scoring:

Visual acuity
Refraction
Color Vision:
Visual fields (confrontation)
Proptosis Proper:
Inspection:
Compensatory head posture:
Facial symmetry:
Ocular symmetry:
Any apparent mass description:
Eyelids
Position (MRD1 and MRD2):

Fullness
Contour
Movements/Lid Lag
Lagophthalmos
Mass lesion (describe if present)
Palpebral fissure height
Proptosis
Pulsations
MEASUREMENTS:
Hertel's (B.Rmm)
Horizontal
Vertical
Valsalva maneuver:
Periocular changes:
Conjunctiva
Cornea
Anterior chamber
Pupil
Lens
Fundus
Ocular motility
Cover test
PBCT
IOP
Palpation:
Bony regularity:
Temperature :
Tenderness:
Crepitus:
Description of Mass:
Thrill/ Reducibility:
Retropulsion:
RE
LE

RE
LE
Auscultation:
Fundus Examination:
Impression: (D/D):
Imaging:
FNAC:
Special Investigations:
Apropriate Plan Of Management:
Communication With The Patient:
Procedure Performed:
HPE Diagnosis:
Postop Follow:
BIBLIOGRAPHY
1. Ben Simon GJ, Yoon MK, Atul J, Nakra T, McCann JD,
Goldberg RA. Clinical manifestations of orbital mass lesions
at the Jules Stein Eye Institute, 1999-2003. Ophthalmic Surg
Lasers Imaging. 2006;37(1):25-32.
2. Dunsky IL. Normative data for Hertel exophthalmometry
in normal adult black population. Optom.Vis Sci
1992;69:562-4.
3. Fledelius HC, Stubgaard M. Changes in eye position during
growth and adult life as based on exophthalmometry,
interpupillary distance, and orbital distance
measure-ments.Acta Ophthalmologica. 1986;64:481-6.
4. Gladstone JP. An approach to the patient with painful
ophthalmoplegia, with a focus on Tolosa-Hunt syndrome.
Curr Pain Headache Rep. 2007;11(4):317-25.
5. Grove AS Jr. Modern examination methods of orbital
disease. Orbital radionuclide examinations. Trans Am Acad
Ophthalmol Otolaryngol. 1974;78(4):OP587-98.
6. Knudtzon K On exophthalmometry; the result of 724
measurements with Hertels exophthalmometer on normal
adult individuals. Acta Psychiatr Neurol. 1949;24(3-4):523-37.
7. Kolasa P, Kaurzel Z. Post-traumatic pulsating exophthalmus
coexisting with congenital carotid-cavernous fistula. Neurol
Neurochir Pol. 2001;35 Suppl 5:58-63.
8. Krayenbhl HA. Unilateral exophthalmos. Clin Neurosurg.

1966;14:45-71.
9. La Mantia L, Erbetta A, Bussone G. Painful
ophthalmoplegia: an unresolved clinical problem. Neurol
Sci. 2005;26 Suppl 2:s79-82.
10. Malhotra R, Wormald PJ, Selva D. Bilateral dynamic
proptosis due to frontoethmoidal sinus mucocele. Ophthal
Plast Reconstr Surg. 2003;19(2):156-7.
11. Meyer DR. Compressive optic
Ophthal-mology. 2007;114(1):199.
neuropathy.
12. Miller NR. Neuro-Ophthalmology of orbital tumors. Clin

Neurosurg. 1985;32:459-73.
13. Rootman J. An approach to diagnosis of orbital disease.
Can J Ophthalmol 1983;18:102-7.
14. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients
with orbital tumors and simulating lesions: The 2002
Montgomery Lecture, part 1. Ophthalmology.
2004;111(5):997-1008.
15. Sugawara Y, Harii K, Hirabayashi S, Sakurai A, Sasaki T. A
spheno-orbital encephalocele with unilateral exophthalmos.
Ann Plast Surg. 1996;36(4):410-2.
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1980;89(2):263-7.
CHAPTER
Imaging a Case of
Proptosis: CT and MRI
Subrahmanyam Mallajosyula, Ravi Varma
Proptosis is a Pandora's box was the most often

quoted sentence in the past, and it was rightly so,
since "surprises on the operation table" were quite
frequent. The surprises could be in the form of
pathology, localization and extent of the lesion. These
problems were too frequent for the occasional orbital
surgeon. Hence, many a time in the past it was the
team efforta team of neurosurgeon and
ophthalmologistthat used to perform surgery on
every case of proptosis. Advances in imaging
techniques have changed the scenario completely.
With the advent of CT scan and later MRI we know
the exact location of the lesion, the nature of the lesion
(tumor or a cyst, encapsulated/ infiltrating, highly
vascular /less vascular, benign/malignant/
metastatic). We can also very accurately predict the
histological nature of the lesion like glioma of the
optic nerve/meningioma of the optic nerve sheath/
sphenoid ridge meningioma/ thyroid orbitopathy/
cavernous hemangioma/ lymphangioma/ myocysticercosis/ myositis/lymphoma, etc. Hence the
surprises in proptosis were very rare now. After a
thorough clinical evaluation and imaging with CT,
most often we know the nature and location of the
lesion, which enables us to manage proptosis in a
systematic way. We strongly believe that the
advances in imaging technologies have not only made
the diagnosis and management of proptosis more
scientific, they have revived interest in the
subspecialty of orbital services.
The globe, conjunctiva and optic disc are the
orbital structures amenable for direct examination.
Pathology involving the rest of the orbital structures
needs imaging studies for optimal evaluation. Several

imaging techniques such as plain radiography,
ultrasound, color Doppler, computed tomography
(CT) and magnetic resonance imaging (MRI) are
available for imaging orbital pathology. Each
technique has its own advantages and disadvantages, and the information obtained from these
studies is often complementary.
Plain radiography has been the only investigation
available for orbital imaging for most of the last
century. Now, CT and MRI have largely replaced
radiography, and skull X-rays are now performed
only in selected cases of facial fractures and to screen
for intraocular metallic foreign bodies before an MR
examination. Sonography is used largely in the
evaluation of intraocular structures. Large mass
lesions in the orbit can also be visualized and
characterized on sonography. Ultrasound combined
with Duplex Doppler plays an important role in the
noninvasive evaluation of vascular pathology
involving the orbit. It can differentiate low flow
lesions from high flow arteriovenous malformations.
Though sonography is convenient and non-invasive,
it suffers from limitations in the form of suboptimal
visualization of the posteriorly placed structures,
nonvisualization of intracranial pathology and need
for expertise on part of the examiner.
The most widely used radiological investigation
for evaluation of the orbit is CT scan. It has the
advantages of being widely available, fast,
inexpensive and relatively easy to interpret. The
technique is based on differential attenuation of
X-rays by tissues, where denser tissues attenuate
Imaging a Case of Proptosis: CT and MRI 57

more X-rays. Tissues can be characterized by their
attenuation values (also called Hounsfield units (HU)
- named after the inventor of CT). The retro-orbital
fat imparts a natural contrast that aids in delineation
of normal anatomy as well as pathology. On the
Hounsfield unit scale, air is defined as 1000 HU,
pure water as 0 HU, and dense cortical bone as +1000
HU. Retro-orbital fat usually measures 120 to 50
HU, cerebrospinal fluid measures 0 to +10 HU, extraocular muscles measure +40 to +50 HU and brain
measures +35 to +45 HU. CT scans of the orbit are
best obtained before and after intravenous
administration of iodinated contrast medium. Thin
sections (2-3 mm) are required to delineate the
pathology and differentiate it from the normal orbital
structures. Imaging in both axial and coronal planes
may be required to optimally evaluate orbital
pathology. The images can be viewed in different
window settings to evaluate structures such as the
soft tissues on bony structures.
Modern day spiral CT and multidetector CT
technology permit acquisition of the imaging data as
a volume, within a few seconds so as to minimize
movement artifacts. It is now possible to acquire
images in several phases during and after intravenous
administration of contrast medium, that may be
instrumental in diagnosing vascular and other similar
abnormalities of the orbit. In addition, post
processing of spiral CT data yields extremely high
resolution multiplanar reconstructions and 3dimensional images, without the need for additional
exposure to radiation. CT scan images are taken with
soft tissue windows (to study the details of the globe
and the soft tissue lesion) and bone windows (give
the details of the bone).
Though CT scan is considered as the work-horse
of orbital imaging, it suffers from several
disadvantages such as relatively low tissue contrast
as compared to MRI, and severe degradation of
image quality by dental fillings. Dose of radiation to
the lens, which is most sensitive organ in the body
to radiation exposure, is a concern.
Unlike radiography and CT scan, MR imaging
does not use ionizing radiation. Image production
in MRI is based on measurement of relaxation
properties of protons after excitation with
radiofrequency energy. By altering the parameters
of data acquisition, we can obtain several image
contrasts such as T1 weighted images, T2 weighted

images, proton density images, inversion recovery
images, fat saturated images and gradient recalled
images.
MR imaging has several advantages over CT scan
such as superior soft tissue contrast, direct
multiplanar capability and excellent visualization of
the optic nerve and intracranial pathology.
Availability of higher field strength magnets and
surface coils have significantly improved the image
quality in orbital imaging. However, the long
acquisition times that degrade the image quality due
to movement artifacts and poor visualization of bony
details and fractures still remain a significant
impediment in the use of MRI for orbital evaluation.
Furthermore, MRI is contraindicated in patients with
cardiac pacemakers and defibrillators, neurostimulators, metallic foreign bodies in the eye, and other
ferromagnetic implants within the body.
Though, the signal pattern is highly dependant
upon the specific imaging parameters used during
acquisition, in general the signal patterns of various
orbital and other related structures can be described
as follows:
T1 weighted
Bone-cortex
Bone-marrow
Fat
Muscle
Aqueous/
Vitreous
Lens
Sclera
CSF
Blood vessels
Air
T2 weighted Fat suppressed T1
No signal
Bright
Very bright
Gray
Dark
No signal
Bright
Bright
Gray
Bright
No signal
Dark
Very dark
Gray
Dark
Gray
Gray
Dark
No signal
No signal
Gray
Gray
Bright
No signal
No signal
Gray
Gray
Dark
No signal
No signal
Currently, the information obtained on imaging

studies is complementary. The choice of imaging
study should be based on the clinical presentation
and the specific pathology being suspected. Further,
the imaging techniques can be modified to specifically
address the pathology in question. Discussion with
the radiologist performing the imaging study goes a
long way in ensuring optimal imaging of the orbit.
While ordering for the imaging, the decision depends
upon the nature of lesion. However I (MS), very
rarely, if at all ask for a plain X-ray of orbit. The

information obtained from a plain X-ray is very
limited and grossly inadequate to understand the
nature of the lesion and plan management
(Figure 3.1).
My most preferred imaging modality is CT scan
of Orbit, 2 mm slices of axial, coronal sections with
sagittal reconstruction, with or without contrast.
Contrast studies are ordered only when vascular or
tumor pathology is suspected clinically. Otherwise
plain study alone is ordered. For example, in thyroid
orbitopathy, plain study is asked for, contrast study
is not indicated. My preference for CT is because of
its easy availability, cost to the patient, easy
interpretation. I ask for an MRI only when I am
dealing with a lesion of optic nerve or its sheath
which account for 2 to 3% of all cases of proptosis.
Evaluation of a CT scan of orbit: Though the
soft tissue lesion attracts our attention, it is prudent
to read the CT scan image in a systematic method,
so that we don't miss any subtle changes, which
provide very useful information.
The level of the scan: The orbital scans are
normally taken in a sequential order, from below
upwards for axial scans and from anterior to
posteriorly in coronal scans. To know the level of
the scan in the axial sections, remember that the

medial walls of the orbit are parallel in the midorbit
(Figure 3.2). If they were not parallel, the level of
the picture is either lower or upper. Look at anterior
and posterior parts of the picture. Anteriorly a flat
nasal root and posteriorly the presence of brain tissue
indicate that the scan was of upper part of the orbit
(Figure 3.3). A prominent nose anteriorly, and the
presence of oropharynx posteriorly indicate that the
picture was of lower part of orbit (Figure 3.4). The
important structures of orbit at various levels are
shown in Figures 3.5, 3.6 and 3.7.
Figure 3.2: Mid level: Note the medial
Figure 3.1: X-ray picture of orbit of a patient of proptosis. The

information obtained is very little. It is impossible to predict the nature
of the lesion, and its exact location
Figure 3.3: Note the flat nasal root anteriorly
Figure 3.5: Axial CT scan at lower level of orbit

showing important structures
Figure 3.4: Note the prominent nose anteriorly (blue arrow) and
the or opharynx posteriorly (yellow arrow). Normal anatomy of orbit
on CT
Figure 3.7: Axial CT scan at upper level of orbit showing

important structures
Figure 3.6: Axial CT scan at mid level of orbit showing

important structures
Common mistakes: We wish to discuss some of

the common mistakes committed by the residents
while interpreting the CT scan of the orbits. They
are described with Figures 3.8 to 3.10.
How to read?
Anatomical location /level
Bony orbit
Eye ball
Extra ocular muscles
Optic nerve
Soft tissue Lesions
Borders / consistency
Hounsfield units
Contrast enhancement
Bony Orbit
Excavation or molding (Figure 3.11) erosion, (Figures
3.12 to 3.14) absence (Figure 3.15), hyperostosis
(Figures 3.16A and B, 3.17) are the common changes
in bony orbit.
Figure 3.8: Discontinuity of optic nerve: Due to the sinuous course of

the optic nerve, axial sections often show an apparent discontinuity
of the optic nerve (Yellow arrow), which was misinterpreted in cases
of trauma as avulsion by the residents
Figure 3.11: Look at the bony Excavation with very smooth margins
(yellow arrow) of the medial wall. This is due to increased orbital
pressure exerted by the lesion which is in contact with the bone. This
denotes a "chronic course of a "benign lesion"
Figure 3.9: Cystic lesion abutting optic nerve: You can see a cystic
lesion (yellow arrow) temporal to optic nerve (green arrow). Remember that the optic nerve does not emerge from the most posterior
part of the globe. The apparent "cyst" is in fact the eyeball itself! This
patient had glioma of the right optic nerve (orange arrow)
Figure 3.10: Enlarged Optic canal is how most of the residents interpreted A which is in fact enlarged inferior orbital fissure. Compare its
size with normal inferior orbital fissure (B) Remember that optic canal
is seen at the level of anterior clinoid processes and you will be able
to see superior orbital fissure lateral to optic canal (Figure 3.6).
Figure 3.12: Bony erosion and hypertrophy in a case of fungal

granuloma of orbit. Look at the irregular borders of the bone in
comparison to the smooth borders of the excavation (Figure 3.11)
Bony erosion is due to infiltration of the bone. It is commonly seen in
malignant lesions or fungal infections
Figure 3.13: Sino-orbital mucormycosis. Note the involvement of sinus (yellow arrow),
Orbit (Blue arrow) and brain (red arrow) with erosion of roof and medial walls of orbit
Figure 3.14: Fungal granuloma of the maxillary sinus with bony erosion (yellow arrows) Leading to extension into the ethmoid sinus,
orbit and cheek
Figure 3.15: Bony dehiscence: Dumbbell Dermoid involving maxillary

sinus and orbit. Note the absence of floor (yellow arrow). Note also
the smooth excavation of the maxillary sinus and its expansion
B
Figures 3.16A and B: Note the hypertrophy (hyperostosis) of the Sphenoid bone (yellow arrow) In a case of sphenoid
ridge meningioma with extension to temporal region (green arrow). Note the intracranial component (red arrow)
Bony Lesions
Bony lesions include Osteoma (Figures 3.18A to C),
fibrous dysplasia (Figure 3.19), Ossifying fibroma
(Figures 3.20A and B). Subperiosteal hemorrhage
(Figure 3.21) and subperiosteal abscess (Figures 3.22A
to C) are other common lesion frontoethmoidal
mucoceles (Figures 3.23A and B), Angiofibroma from
sinuses (Figures 3.24A and B) are also seen fairly
common. Fractures (Figures 3.25A and B, 3.26A and
B) are quite frequent.
Figure 3.17: Hyperostosis and erosion of the sphenoid (yellow arrow)

in a case of sphenoid ridge meningioma with intracranial component
(red arrow), temporal fossa (green arrow) and orbital involvement
(Blue arrow)
Figures 3.18A to C: Osteoma of the ethmoid bone involving the entire ethmoid, and leading to optic nerve compression. See
the uniformly dense tumor with Hounsfield values similar to bone in both soft tissue windows (A) and bone window (B). The
tumor was excised through a modified Lynch incision (C)
Figure 3.19: Fibrous dysplasia usually involves flat bones of face and hence orbital involvement is not uncommon. Imaging shows
expansion of the bone with thinning of the overlying cortex. A ground-glass appearance is common on CT
B
Figures 3.20A and B: Ossifying fibroma Imaging shows a well circumscribed lesion eroding the bone with a sclerotic
margin (yellow arrow) and foci of internal calcification (green arrow).
Figure 3.21: Sub-periosteal hemorrhage: Subperiosteal hemorrhage is less common than subperiosteal abscess. The periorbita can be seen
clearly as a thickened membrane (yellow arrow). Note that the sinuses are clear in this film, unlike in subperiosteal abscess (red arrow) where
the sinuses are involved (Figure 3.22)
Figures 3.22A to C: Sub-periosteal abscess, infection extending from the Fronto-ethmoidal sinuses (red arrow). Note the congestion and
edema of the lids and peri-orbital region, chemosis and congestion of the conjunctiva apart from the eccentric proptosis. Periorbita (yellow
arrow) could be well made out
Figures 3.23A and B: Fronto-ethmoidal mucocele is a very common cause of eccentric proptosis in which the globe is pushed down and out.
You can see a very gross eccentric proptosis of left eye with fullness in the superomedial quadrant. The CT Scan shows a grossly enlarged
frontal sinus with mucosal thickness (yellow arrow)
Figures 3.24A and B: Angio fibroma arising from sinuses is not very rare. Note the huge tumor mass. Occupying the entire maxillary sinus,
and distorting the orbital cavity. Its extension into the nose and the fullness of left cheek could be made-out. Also note the severe eccentric
proptosis and corneal leucoma due to exposure
Figures 3.25A and B: Limitation of elevation of right eye with diplopia in upgaze following trauma while at play. Note the subconjunctival
hemorrhage. The FDT was positive. Clinical diagnosis of fracture floor of orbit with entrapment of inferior Rectus was made. But the CT scan
(B) of orbit showed fracture roof of the orbit with the displaced fragment impinging on the globe and mechanically restricting its movement
(yellow arrow)
Figures 3.26A and B: Fracture floor of orbit can either involve a large area (Red arrow- A) and may show hemorrhage in the sinus or may be
very tiny with trap-door mechanism and the typical "tear-drop" sign (yellow arrow- B). The rectus muscle may or may not be entrapped.
Entrapment of the rectus with positive FDT and diplopia is one of the important indications for early surgery
Trauma: Trauma leading to orbital fractures

(Figures 3.25A and B, 3.26A and B) is fairly common
and its incidence is on the raise in view of increase
in road traffic accidents and violence in the society.
Apart from traumatic optic neuropathy, diplopia is
another very important symptom. Persistent
diplopia due to entrapment of a rectus muscle and
positive FDT is one of the indications for surgery.
Eyeball: After examining the bony orbit, look

at the globe and its relation to the lesion. The lesion
may be soft and molding along the eyeball like a
lymphoma (Figure 3.27) The globe is pushed ahead
by the lesion and also can alter its shape depending
on its consistency (Figures 3.28A and B). In Severe
proptosis, tenting of posterior pole (Figure 3.29) can
be noticed.
Figure 3.27: Molding: Look at the lesion which is molding along the globe, without altering its curvature (yellow arrow). This is very typical
of lesions which are soft like lymphoma. Molding can also be seen in fungal granuloma, adenoid cystic carcinoma of lacrimal gland
Figures 3.28A and B: Firmer lesions indent the globe and can induce refractive errors. Compression of the globe by cystic lesion (Hydatid Cyst
figure A yellow Arrow) and compression of the globe by tumor (schwannoma figure B, yellow Arrow). The intraconal lesions cause hyperopic
shift in the refraction, by virtue of flattening the globe, while the lesions in the peripheral space cause astigmatism
Figure 3.29: In severe proptosis, tenting of the globe with stretching of the optic nerve can occur. This also can contribute to
defective vision. Look at the tenting of the posterior pole of the globe with loss of the normal curvature (yellow arrow)

After examining the contour of the eyeball,
(Figures 3.30 to 3.32) look at the intraocular contents.
Retinoblastoma and uveal melanoma (Figures 3.33A
to D) are the most common intraocular tumors which
can spread into the orbit and can cause secondary
proptosis. It is a routine practice to get a CT Scan of

orbit in retinoblastoma. The tumor can be seen as an
intraocular mass lesion with calcification. It can be
confined to the globe or can extend into the orbit,
optic nerve or brain.
B
Figures 3.30A and B: Retinoblastoma; The typical amaurotic cat's eye reflex in the left eye( A) The intraocular mass with
calcification (yellow arrowB) is very typical of retinoblastoma. The sclera looks normal
Figures 3.31A and B: This child had a painful proptosis of right eye. Note the gross proptosis with mild congestion, increase in the corneal
diameter, and amaurotic cat's eye reflex (A). The CT scan reveals intraocular masses with calcification (yellow arrow-B). in both the globes.
Note the loss of integrity of the sclera with orbital extension (red arrow)
Figures 3.32A to C: This child presented with recurrent mass after enucleation elsewhere for retinoblastoma. CT scan of the orbit reveals that
the entire socket is filled with the mass (B) leading to expansion of the orbital walls (yellow arrow) and a very significant intracranial extension
(red arrow-C)
Figures 3.33A to D: Uveal Melanoma is the most common intraocular malignancy in adults and it can extend into the orbits. Note the tumor
which could be seen very clearly in external examination(A) and its brown color and the retinal vessels over it in the slit lamp examination
(B). The tumor arising from the choroid is very well visualized in the CT scan in the coronal view (C) and its antero-posterior extent in the sagittal
reconstruction (D). Note the absence of calcification. The sclera appears intact

Enlarged extraocular muscle: Enlarged
extraocular muscle (EOM) is the most common
finding we come across on imaging in proptosis,
followed by mass (tumor). This is because of high
prevalence of myocysticercosis as cysticercosis is
endemic in our area. The important causes of
enlarged extraocular muscle include thyroid
orbitopathy, (Figures 3.34A and 3.35A and B)

myocysticercosis, (Figures 3.36A and B) idiopathic
orbital inflammation (Figure 3.34B), rhabdomyosarcoma, lymphoma (Figures 3.39A to D), carotid
cavernous fistula (Figures 3.37A and B to 3.38A and
B), and metastasis. (Figures 3.40A to D).
Figures 3.34A and B: Thyroid associated orbitopathy (TAO) is the most common cause of enlarged extraocular muscle in most studies (A).
Myositis due to idiopathic orbital inflammation (IOI) is also very common (B). Hence it is very important to differentiate these two conditions on
imaging. The above pictures are very classical. In TAO, the tendon is spared and in myositis it is also involved. Contrast enhancement and only
lateral rectus muscle involvement can occur in IOI but not in TAO. Lacrimal gland and even fat can be involved in IOI, but not in TAO. Most often
in TAO, the CT scan of the orbit reveals bilateral enlargement of extraocular muscles
Figures 3.35A and B: In TAO, enlarged extraocular muscle can compress the optic nerve and cause loss of vision. Note the grossly enlarged
recti muscles surrounding the optic nerve (red arrow) in the mid coronal sections of left orbit where as the optic nerve on the right side is free
from compression. The posterior coronal sections reveal a severe compression of optic nerve on the left orbit. This patient had presented with
a vision of 20/200 in left eye. Note that the floor and medial wall if orbit are reaching the posterior most part of the orbit, but not the zygoma.
Hence in orbital decompression aimed to relieve optic nerve compression, I invariably include the medial wall or floor or both. I also excise orbital
fat. Lateral wall decompression is more for cosmetic purpose
Figures 3.36A and B: Myocysticercosis is a very frequent cause of enlargement of extraocular muscle in endemic areas. It can involve any
of the extraocular muscles. The image is very characteristic in that a cystic lesion with a hyper-dense spot within (represents the scolex) is
seen in an enlarged EOM. Commonly a single cyst is common. Rarely more than one cyst is encountered. I am yet to see a case where more
than one muscle is affected. Intracranial cysticercosis can be rarely associated with orbital cysticercosis. In figure A notice the presence of
two cysts in the superior oblique (SO) muscle. Compare the size of this grossly enlarged SO with the normal SO of left eye. Figure B shows
the typical cyst with scolex involving the inferior rectus muscle
Another common cause of enlarged EOMs is

Carotid- Cavernous Fistula (CCF). The incidence of
it is on the raise due to increase in the incidence of
trauma. CCF may be low flow or high flow in nature.
In view of the arteriovenous communication,

enlarged EOMs and superior ophthalmic vein are
seen in the CT scan
Figures 3.37A and B: CCF low flow fistula. Note the dilated Superior ophthalmic vein of the right eye (yellow arrow) and the EOMs. Note
how big the lateral rectus (green arrow) is. Notice also the subtle enlargement of superior and inferior recti
Figures 3.38A and B: CCF High-flow fistula: Compare the grossly enlarged superior ophthalmic vein (yellow arrow) and the huge enlargement
of the inferior rectus muscle( green arrow) with the previous picture. The high flow CCF usually follows trauma, and clinically characterized
by pulsatile proptosis, caput medusae, chemosis, restricted ocular motility, gross retinal venous engorgement and secondary glaucoma
Figures 3.39A to D: Lymphoma: Lymphoma is characterized by a soft tissue lesion which typically molds around the globe (yellow arrow A,B
and D). Salmon's patch is the typical presentation (Cgreen arrow). Some times enlarged EOMS can be seen in some sections of the CT scan
(Dred arrow)
Figures 3.40A to D: Metastatic lesion from breast: This patient presented with painful proptosis of left eye (A) of 1 month duration. She
underwent mastectomy for carcinoma breast (B) 1 year back. CT scan of orbit shows enlarged lateral rectus muscle (C) associated with
erosion of the lateral wall of the orbit (red arrow). Enlarged lateral rectus associated with bony erosion is a very common finding of CT scan
of orbit in metastatic lesions, especially from breast and GIT. FNAC has confirmed the clinical diagnosis (D)
Soft-tissue Lesions
The clinical spectrum of primary soft tissue lesions
of the orbit is very varied. This is because of the
various types of tissues that exist in the orbit. Though
lesions of the optic nerve are not too common, their
importance can not be over emphasized since they
can lead to loss of vision
The most frequent lesions of the optic nerve are
glioma (Figures 3.41A and B, 3.42) and meningioma
of the optic nerve sheath (Figures 3.44A and B). Optic
nerve can also be involved in idiopathic orbital

inflammation (Figures 3.43A and B). It is very
important to know whether the intraconal lesion is
arising from the optic nerve (meningioma or glioma
of optic nerve) or separate from it [cavernous
hemangioma (Figures 3.45A to D), schwannoma
(Figures 3.46A and B), etc]. Schwannoma (Figures
3.46A and B) is another tumor from nerve tissues,
which mimics closely cavernous hemangioma
(Figures 3.45A to D).
Figures 3.41A and B Optic Nerve Glioma: The axial section of CT scan shows spindle shaped tumor of the optic nerve with very distinct
margins. The coronal section shows a large intraconal tumor. Note that the optic nerve is not seen separately( red arrow). The margins are well
made-out. There is no evidence of calcification and there is no contrast enhancement. These are the typical feature on imaging of a optic nerve
glioma, which help in distinguishing it from meningioma of optic nerve sheath
Figure 3.42: The axial CT scan of orbit shows a very large intraconal mass with distinct borders, and is similar to the Figure 3.39. It however
shows distinctly cystic space (yellow arrow). It is a case of optic nerve glioma with cystic degeneration. Note the bony expansion, flattening
of the posterior pole of the globe
B
Figures 3.43A and B: Optic nerve swelling (yellow arrow) as a part of idiopathic orbital inflammation.
Note the enlarged medial rectus (green arrow). Note that the margins are indistinct
Figures 3.44A and B Meningioma of Optic Nerve Sheath: Meningioma of the optic nerve sheath is more common than the optic nerve
glioma. On imaging it is characterized by indistinct margins (blue arrow), calcification, "tram- track" appearance and contrast enhancement
(yellow arrow). These features help in distinguishing this lesion from glioma of optic nerve. In the picture B, note the fluffy and indistinct margins
which differ from the smooth and distinct margins of glioma. The hypodense spot in the center of the lesion is the optic nerve. The hyperdense
spots along the optic nerve are due to calcification. Compare these figures with those of glioma to understand the differences between these
two lesions on imaging
Figures 3.45A to D: Cavernous Hemangioma is characterized by hyperdense lesion with well defined margins and very minimal contrast
enhancement (green arrow). It is more commonly located in the intraconal space and lateral to optic nerve (yellow arrow). In coronal section
the mass can be seen separate from the optic nerve. Since most often the duration is very long (average 30 months), bony excavation of the
orbit is very common. The excised tumor with very distinct borders is seen in C. Occasionally cavernous hemangioma may show calcified
spots (D yellow arrow) which represents the phleboliths. Note the subtle bony excavation of the lateral wall
Figures 3.46A and B: Schwannoma is another common benign tumor and is characterized by very distinct margins. It is often of long duration
and hence shows bony expansion. Note the gross axial proptosis of the left eye (A) and the large tumor with very clear cut margins in the
intra-conal space, and excavation of the lateral wall (yellow arrow). Schwannomas rarely enhance on contrast
Neurofibroma involving the orbit can be plexiform or as a part of Von Recklinghausen disease (Figure
3.47A to D).
Figures 3.47A to D Neurofibroma: Plexiform neurofibroma involving the orbit (AandB). Note the typical shape of the lesion and the imaging
showing the large tumor with indistinct margins involving the temple and the lid. Also note heterogenisity of the lesion. In Von Recklinghausen
disease (C and D), the clinical picture with numerous nodular lesions is very distinctive. On imaging apart from proptosis and multiple tumors,
dehiscence of the roof of orbit is common (Cyellow arrow), which results in pulsatile proptosis

Other vascular tumors like hemangioblastoma,
hemangioendothelioma, hemangiopericytoma,
angiosarcoma (Figures 3.48A to C), etc show on
imaging a well defined mass lesion with contrast
enhancement. Usually the clinical picture is
characterized by shorter duration, mild pain/
discomfort. The final diagnosis is by histopathology.
Lymphangioma usually shows intralesional
hemorrhage (chocalate cyst).
Lacrimal gland tumors: The lacrimal gland

lesions are common.Most common benign lesion is
pleomorphic adenoma (Figures 3.49A to C) and the
most common malignancy is adenoid cystic carcinoma
(Figures 3.50A and B) which has a very poor
prognosis. Clinically pleomorphic adenoma has a long
duration, and painless. Shorter duration, often less
than 6 months, and mild discomfort are frequent in
adenoid cystic carcinoma. Usually pleomorphic
adenoma is firm in consistency, and is non-tender.
Figures 3.48A to C: Angiosarcoma. This patient presented with painful proptosis of 3 months duration. Note the contrast enhancing mass
lesion with subtle indistinctness of posterior margins in the axial sections of the CT scan (C). The diagosis of angiosarcoma was confirmed on
histopathology and immuni-histochemistry
Figures 3.49A to C: Pleomorphic Adenoma: This patient presented with painless, gradually progressing proptosis of 18 months duration (A).
Note that on imaging, the tumor is not molding, and is actually pushing the globe and indenting it. The downward and medial displacement of the
globe is very well made-out in the CT scan (C)
Figures 3.50A and B: Adenoid Cystic Carcinoma: The duration of proptosis in this patient is 5 months. She presented with eccentric proptosis
and pain. Imaging shows a hyperdense mass lesion of lacrimal gland, molding to the globe. This is because of the softer consistency of the
tumor, and can mimic lymphoma of lacrimal gland
Figures 3.51A and B: Lymphoma of Lacrimal Gland: The clinical presentation is characterized by the presence of Salmon's patch in the
superior fornix (A). The CT scan shows a well defined enlargement of the lacrimal gland which is molding to the globe. Also note subtle bony
erosion of lateral wall of the orbit
Lymphoma of lacrimal gland: (Figures 3.51 A

and B) is a soft lesion which moulds along the
globe.
Rhabdomyosarcoma: (Figures 3.52A to D) is the

most common mesenchymal and malignant tumor
of children. Superior Rectus muscle is most commonly
involved.
Figures 3.52A to D: Rhabdomyosarcoma: This is a very common primary mesenchymal tumor of pediatric age group. The course can be rapid
and some times very acute and mimic orbital cellulitis. The child (A) shows very gross proptosis with anterior staphyloma, while the child (C)
shows less dramatic clinical picture. Note the fullness of the superior sulcus and eccentric proptosis with the eyeball pushed down in both the
children. This is because the tumor involved superior rectus muscle (the most common EOM to be involved). Note the enlarged superior rectus
with distinct margins (B and D). Note also the subtle bony erosion in B, while bone shows expansion, not erosion. Contrast enhancement is
observed in these tumors. Biopsy confirms the diagnosis. The tumor responds very well to radiotherapy/chemotherapy
Cystic lesions of the orbit: A variety of cystic

lesions occur in the orbit. They could be congenital
cysts like dermoid (Figures 3.53A and B), congenital
cystic eyeball, arachnoid cyst (Figures 3.56A and B),
parasitic cysts [cysticercosis (Figures 3.55A to C),

hydatid cyst] (Figures 3.54A and B), cystic
degenerations (glioma), mucocele (Figure 3.57),
lymphangioma.
Figures 3.53A and B: Dermoid Cyst is the most common cystic lesion of the orbit in most of the series, other than in areas endemic for
cysticercosis. Dermoids are usually congenital, preseptal and involve the temporal region in children, while they are acquired, postseptal, and
nasal in location (A) in adults. On imaging they show a cystic lesion with well defined margins. Some times, a part of the lesion can be
hyperdense, depending on its contents. Dumbbell -dermoids (B) are rare. Note the cystic lesion with an orbital component, and a temporal
component (yellow arrows), and the gap in the lateral wall of the orbit. Note also the smooth excavation of the lateral wall (blue arrow)
Figures 3.54A and B: Hydatid Cyst: Hydatid cyst is not very common. It usually occurs in younger individuals, and is often intraconal in
location. Hence visual symptoms, axial proptosis of few months duration and optic disc edema in a patient having pets, especially dogs should
arouse clinical suspicion. CT scan of the orbit shows a thin walled, isodense cystic lesion (red arrow). It can grow to a very large size and
dwarf the globe (yellow arrow: A and B). Note the large, thin walled cyst, pushing the eyeball inferolaterally. Compare the size of the cyst with
the eyeball. Also note the expansion of orbital walls. Because of such expansion of the orbital walls, and increased orbital volume, excision of
the cyst leads to enophthalmos
Figures 3.55A to C: Cysticercosis: Orbital Myocysticercosis is common in endemic areas. Usually solitary cyst is seen, involving a single extra
ocular muscle. Restricted motility, diplopia associated with mild discomfort or pain are the important clinical features. Note the restricted
elevation of right eye (A). CT scan of orbit shows enlarged inferior rectus muscle (green arrow) with a cyst. Note the hyperdense spot in it
(yellow arrow) which represents the scolex
Figures 3.56A and B: Arachnoid Cyst: Arachnoid cysts are very rare. Infants and young children with these congenital cysts present with
painless proptosis. CT scan of the orbit reveals a cyst associated with hydrocephalus. The Hounsfield units of the cyst are usually 5 to 10 units,
similar to CCF. Note the severe flattening of the posterior pole of the eyeball (yellow arrow) by the cyst (green arrow) Also note the bony
expansion of lateral and medial walls. Note the gross hydrocephalus (B white arrow)
Figure 3.57: Mucocele from frontoethmoidal sinus is a very common cause of eccentric proptosis.
On imaging, the cystic enlargement of the sinuses (yellow arrow). Note that the anterior ethmoidal sinus is also involved
Secondary involvement of the orbit due to

extension of lesions from the eye, sinuses (Figures
3.58A to C), lids (Figures 3.62A to C), lacrimal the

(Figure 3.59) `brain is not uncommon.
C
Figures 3.58A to C: Gross eccentric proptosis of left eye with perforated cornea (A).The CT scan of the orbit (B and C) shows the
mucormycosis fungal granuloma involving the maxillary, ethmoid and frontal sinuses, bony erosion of floor, medial wall and roof with orbital and
intracranial extension. Note how well the bony erosion is seen in the bone-window images (C)
Figure 3.59: Mucocele of the lacrimal sac is seen as a well demarcated lesion with anterior
part of nasolacrimal duct CT scan is ordered only when a tumor is clinically suspected
Metastatic lesions: Metastatic lesions of the orbit

usually present with pain and proptosis of a few days
to few weeks duration. Most often they give history
of previous surgery for a malignancy, but rarely the
proptosis may be the presenting sign of a undetected
primary elsewhere. CT imaging is very helpful in
that a mass lesion associated with bony erosion

(Figures 3.60A and B) is very common. Enlarged
lateral rectus muscle is common in metastatic lesions
from breast or GIT. (Figures 3.61A to D). Metastasis
can occur from other organs like prostate thyroid,
parotids, etc (Figure 3.63).
B
Figures 3.60A and B: Metastatic neuroblastoma in a male 15 years presented with painful proptosis of 1 month of right
eye associated with restricted ocular motility. Note the soft tissue lesion of orbit with bony erosion of roof and lateral walls
Figures 3.61A to D: Metastatic Carcinoma from right breast in a female of 45 years who presented with mild proptosis and periocular swelling
of left eye since 1 month (A). She underwent radical mastectomy 2 years back (B). CT scan of orbits reveals enlarged lateral rectus (green
arrow) with bony destruction of lateral wall and the roof (C and D orange arrow). FNAC was positive for ductal carcinoma of breast. She was
referred to oncologist for further management
Secondary orbital involvement can occur due to extension of tumors of eyelids (Figures 3.62A to C),
intraocular tumors or intracranial tumors.
Figures 3.62A to C: Secondary Involvemet From Lid Tumor in this elderly male who presented with a very large Basal cell carcinoma of the
upper lid of 6 years duration. Note the huge upper lid tumor (A), and its orbital extension (B and C) evident in both coronal and sagittal sections
Figure 3.63: Adenoid cystic carcinoma of parotid gland with intracranial

(red arrow) and orbital extension (green arrow). Also note the bony erosion
Contrast enhancement: Whenever I suspect a

vascular lesion or a tumor, I ask for contrast studies.
Contrast enhancement shows that the tumor is very
vascular. It differentiates a cavernous hemangioma
from a hemangioendothelioma. When the tumors are

very strongly enhance on contrast as in the Figures
3.64A to D, I will keep a unit of blood reserved at
the time of surgery.
D
Figures 3.64A to D: Contrast enhancement of hemangioendothelioma (B when compared to A) and Lymphoma (D and C)

3-D reconstruction of orbit: Orbital 3-D
reconstruction is ordered in severe orbital
fractures and also in congenital bone defects

(Figures 3.65A and B).
Figures 3.65A and B: 3-D reconstruction of the orbit and periorbital regions is very useful in cases
of trauma. Note how well the bony defect in the frontal bone and the cleft palate are seen
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Role of Cytology in Orbital Lesions 85
CHAPTER
Role of Cytology
in Orbital Lesions
Geeta K Vemuganti, Anirban Bhaduri
Cytologic diagnosis of lesions is one of the mainstay

of diagnosis for lesions affecting various organs of
the body. This speciality has made tremendous
impact even in ophthalmic pathology, an upcoming
subspeciality, which has been referred by Fredrick
Jakobiec as queen of subspecialities of
Ophthlmology. The techniques of obtaining
specimens of ocular cytology have undergone much
refinement and with increasing reports of larger
series of cases, the learning curve has been crossed
by many experts. In general, the techniques used for
orbital lesions include: Fine needle aspiration of
palpebral lesions, squash and imprint cytology of
fresh tissue for rapid intraoperative diagnosis.1-6 The
cytology specimens could also be subjected to
recently emerging molecular tools of diagnosis,7-8 thus
aiding in patient management.
Fine Needle Aspiration/Sampling Technique

The procedure for FNAC at any site in the body is
the same. The procedure can be done by the
pathologist or the surgeon, directly under vision or
under guidance of CT. Usually there is no need of
any local anesthesia injection by this technique, except
in children where a general anesthesia may be
preferred. The technique of obtaining the material
could be a sampling technique wherein a 23 /24
guage needle is introduced into the lesion and
pushed in various directions within the lesion and
gently withdrawn.9 By the capillary action, the cells
are drawn into the needle. This technique has also
been applied to ocular cytology.3,6 The advantages
of this technique are: It is easy, simple, less
hemorrhagic and causes less apprehension to the
patient. The cytologic material obtained by this

method is usually adequate with minimum amount
of hemorrhage. A few disadvantages of fine needle
aspiration in general include inadequate material,
hemorrhagic aspirate, bleeding at the site, especially
for highly vascular lesions. At our center, FNAC has
yielded diagnostic yield in more than 90% of cases.
Tijl et al.10 reported the diagnostic yield of orbital
FNAB combined with clinical and radiological
features as 80%. Very rare potential complications
include globe penetration, retrobulbar hemorrhage,
diplopia and ptosis.11
Intraoperative-operative Diagnosis by Squash

and Imprint Cytology
There is often a need for a reliable intraoperative
diagnosis, specifically in situations where a definitive
preoperative tissue diagnosis is lacking and where
the tissue diagnosis is likely to influence the
immediate surgical management.12 The established
methods of intraoperative diagnosis include frozen
section diagnosis and intraoperative cytologic
diagnosis, each of which has its own merits and
demerits.
Squash or Imprint Cytology

The utility of imprint cytology in eye lesions was
first described by Fuchs for uveal melanoma in 1988.13
Imprint cytology of fresh unfixed tissue specimens
and squash cytology of central nervous system lesions
have been extensively used in the last few decades,
but rarely applied to ophthalmic pathology practice.
The main indications for rapid intraoperative
diagnosis are: a) Infiltrative lesions, suspected

malignant lesions or deeply located lesions where a
preoperative tissue diagnosis is not available; b)
Where there is a discrepancy between a preoperative
clinical diagnosis and the intraoperative findings and:
c) Unusual clinical presentations with diagnostic
dilemma.
Fresh unfixed tissue obtained at the time of
diagnostic or excision biopsy can be used for making
squash preparation and impressions on glass slides.
The procedure for making squash or imprint smears
is usually based on the size, shape, consistency and
crushable properties of the tissue submitted. For soft,
easy to spread tissues, tiny bit of the fresh tissue is
placed between two clean glass slides and gently
drawn apart. For large firm specimens, the imprint
smears are prepared by touching the freshly cut
surface of the lesion with clean slides, avoiding
smearing to retain cell morphology. If the surface is
covered with blood or exudates, more number of
smears is made, after gently wiping the surface clean.
It is preferable to make a minimum of three slides
for each case. It is advisable to preserve extra
unstained smears for further tests like immunocytochemsitry or for any molecular studies in future.
These smears are either alcohol fixed for rapid
hematoxylin and eosin staining, or fixed by air-drying
for Diff-quick staining. A provisional cytologic
diagnosis can be made by the pathologist based on
the cellular and architectural features seen on smears
prepared from either or both techniques.
CASE ILLUSTRATIONS
Case 1
A 23-year-old female presented with swelling of left
upper lid for 2 months with occasional diplopia.
Examination showed non-axial proptosis with
downward displacement of globe and fullness of the
upper lid sulcus (Figure 4.1A). There was a firm,
slightly tender nodular mass in the superolateral
orbit. There was limited abduction of the left eye.
CT scan shows a soft tissue mass in superolateral
orbit, which could not be seen separate from the
lateral rectus muscle (Figure 4.1B). Incision biopsy
was done. Squash and imprint preparation shows a
polymorphous population of inflammatory cells
consisting of lymphocytes, neutrophils and also a few
eosinophils (Figure 4.1C), suggestive of a nonspecific
C
Figures 4.1A to C: (A) Photograph shows the downward displacement of the left eye and fullness of the upper lid sulcus due to the
presence of a lacrimal gland mass (B) CT scan (coronal cut) shows
a well-circumscribed, homogenous, extraconal, soft tissue mass in
the superolateral quadrant of mid-orbit, which is not seen separate
from the lateral rectus muscle. There is no orbital fat streaking.
(C) The squash smear shows a polymorphic population of cells consisting of lymphocytes, neutrophils, eosinophils. (Giemsa, x 500)
orbital inflammatory disease. Permanent sections

confirmed the diagnosis.
Case 2
Case 3
A 12-year-old female presented with swelling of left

upper lid and cheek for 1 month. There was no history
of cough, hemoptysis, fever or weight loss. There
was a soft, non-tender swelling in the superior orbit
(Figure 4.2A). Preauriclar and submandibular lymph
nodes of the left side were enlarged, firm, nontender and mobile. CT scan showed a soft tissue mass
in superior orbit with bone destruction. Clinically, a
provisional diagnosis of adenoid cystic carcinoma of
the lacrimal gland was made. FNAC from the orbital
lesion and preauricular lymph node showed
lymphocytic infiltrates, epithelioid granulomas and
few giant cells (Figure 4.2B). Extensive necrosis was
seen in some areas. Acid-fast bacilli staining of the
smears showed a few bacilli.
A 70-year-old female presented with history of

painful vision loss with drooping of the upper eyelid
of the right eye for 1 month. Examination showed
complete external ophthalmoplegia with ptosis,
dilated and fixed pupil and optic atrophy (Figure
4.3A). She was a diabetic on irregular treatment. CT
scan showed a large, elongated cystic mass in
posterior, superomedial orbit straddling intraconal
and extraconal spaces and lying close to the optic
nerve (Figure 4.3B). Differential diagnoses were: a
parasitic cyst or cystic degeneration in a solid tumor.
Preoperatively, an abscess was found, the wall was
excised after draining the contents. Squash and
imprint preparation of the walls showed chronic
inflammatory cells and fibrosis with fungal filaments
(Figure 4.3C). Cultures of the specimen confirmed
aspergillosis.
Case 4
An 18-month-old male child presented with right
sided non-axial proptosis and temporal fossa fullness
B
Figures 4.2A and B: (A) Photograph shows narrowed palpebral
aperture and fullness of left superior sulcus due to a soft mass in the
superior orbit. (B) The squash smear show cluster of epitheloid cells
with slipper shaped nuclei (hematoxylon and eosin, 200)
A
C
Figures 4.3A to C: (A) Photograph shows complete ptosis on right
side. The patient had total external ophthalmoplegia with optic atrophy
in the right eye. (B) Axial CT scan shows an elongated mass with
cystic changes in the superomedial quadrant of the right orbit close to
the optic nerve and soft tissue mass or mucosal thickening in adjoining
ethmoidal sinuses. (C) the smears shows prominent branching and
septate fungal filaments with inflammatory cells in the background
(Giemsa, 1000)
of 15 days duration (Figure 4.4A). Peripheral blood

smear was normal. CT scan showed an extraconal,
well circumscribed soft tissue mass temporally with
destruction of the greater wing of sphenoid and
extending into temporal fossa and middle cranial
fossa (Figure 4.4B). Incision biopsy was done from
the temporal fossa. The squash and imprint smears
showed cellular infiltrates consisting of neutrophils,
eosinophils, plasma cells and giant cells. In addition
there were large cells with moderate amount of
cytoplasm and large vesicular nucleus with
prominent grooves and folds (Figure 4.4C). Frequent
multinucleated giant cells are seen. Tingible body
macrophages and histiocytes with phagocytic activity
were noted. Based on the above features on squash
and imprint smears, a provisional diagnosis of
eosinophilic granuloma was given which was
confirmed on histology sections. Intralesional
triamcinolone was injected and the lesion resolved
completely. Systemic workup included USG
abdomen, skeletal survey, bone marrow biopsy and
liver function tests were normal. Bone remodeling
was complete at 6 months.
Case 5
A 45-year-old male presented with progressive
protrusion of the left eye for 3 months (Figure 4.5A)
C
Figures 4.4A to C: (A) Photograph shows swelling and erythema of
the right upper lid and fullness of the temporal fossa. (B) CT scan
(axial cut) shows a homogenous, well-circumscribed, low intensity
soft tissue mass with destruction of the greater wing of sphenoid.
The mass involves extraconal lateral orbit, temporal fossa and middle
cranial fossa. (C) The squash smears are cellular with polymorphic
population of cells with multinucleated giant cells, neutrophils,
eosinophils and histiocytes with prominent nuclear grooves and
cleaves (hematoxylin and eosin, 1000).

and non-tender, smooth, firm subcutaneous nodules
in left breast, upper abdomen and thigh for 2 months.
CT scan showed a well defined soft tissue intraconal
and extraconal mass in the left orbit (Figure 4.5B).
He had a previous episode of proptosis on the same
side ten years ago, which was diagnosed
histopathologically as reactive lymphoid hyperplasia
previously, which responded to local radiotherapy
after initial poor response to systemic steroids.
Incision biopsy performed on the orbital mass two
months ago showed sinus histiocytosis.
FNAC was performed from the breast and
abdominal nodule at this presentation and smears
showed high cellularity with a polymorphous
population of cells consisting of lymphocytes, plasma
cells, histiocytes and neutrophils. A large number of
histiocytic giant cells with abundant cytoplasm
extended by the presence of intracytoplasmic plasma
cells, lymphocytes and occasional neutrophils were
present(Figure 4.5C). This feature, described as
emperipolesis, confirmed that the subcutaneous
C
Figures 4.5A to C: (A) Photograph shows severe chemosis and
non-axial proptosis of the left eye. (B). CT scan (axial cut) shows a
well defined irregular hypodense soft tissue mass in intraconal and
extraconal spaces in the left orbit. (C) The smears show large
multinucleated giant cells with multiple lymphocytes, plasma cells within
the cytoplasm of the histiocytes-called as emperipolesis, a
pathognomonic feature of Rosai-Dorfman disease ( Giemsa, 500)
nodules were a part of multifocal Rosai-Dorfman

Disease.
Case 6
A 48-year-old male presented with a swelling on the

surface of the right eye with prominence of that eye
for 6 months. Examination showed a pink fleshy
conjunctival mass with intrinsic vessels (Figure 4.6A),
axial proptosis and limitation of eye movements. CT
scan showed a soft tissue mass moulding around the
globe and extending into the conjunctiva (Figure
4.6B). Incision biopsy was done from the conjunctiva.
Squash and imprint smears were cellular with a
monomorphic population of lymphoid cells. These
cells showed scant rim of cytoplasm and a round
nucleus with moderately coarse chromatin and small
nucleoli (Figure 4.6C). The biopsy confirmed a diffuse
large cell lymphoma, immuno-phenotyping
suggested a B cell lymphoma. Systemic workup did
not show any other focus of lymphoma. He was
treated with local external beam radiotherapy and
was doing well on last follow-up.
Case 7
A 65-year-old male presented with non-axial proptosis

of right eye with inferomedial displacement for 15
days. He also complained of low back ache for 3
months. Firm, non-tender, fixed nodular mass in

superolateral orbit with erosion of superior orbital
rim and temporal fullness (Figure 4.7A). CT scan
showed a soft tissue mass with bone destruction
(Figure 4.7B). X-ray skull shows multiple punched
out lesions (Figure 4.7C), skeletal survey shows
multiple osteolytic lesions in long bones and spine.
Cytology of the mass showed cellular smears with a
large number of plasma cells in varying stages of
differentiation (Figure 4.7D). Many bi-tri and
multinucleated forms were noted. The Giemsa
A
C
Figures 4.6A to C: (A) A closeup photograph of the right eye shows
the pink fleshy conjunctival mass in the lateral bulbar conjunctiva with
intrinsic vessels which seem to appear from and disappear into the
tumor. This mass represents the conjunctival component of the
lymphoma. There is also axial proptosis and limitation of eye
movements. (B) CT scan shows a soft tissue mass classically moulding
around the globe and extending into the conjunctiva. (C) The smears
are cellular with monomorphic cells, with scant rim of cytoplasm and
a moderately coarse chromatin patter. Note the absence of cohesion,
cytoplasm and any differentiation. (Giemsa, x 500)
A
D
Figures 4.7A to D: (A) Photograph shows fullness of upper lid
sulcus and downward displacement of the right eye by an orbital
mass.(B). CT scan (axial cut) shows a well-circumscribed,
homogenous, lobulated soft tissue mass in the orbit with bone
destruction and extension into the temporal fossa and middle cranial
fossa. (C). Radiograph of skull (lateral view) shows multiple, punched
out osteolytic lesions in the skull bones. (D) The cytology smear
shows multiple plasma cells with bi and multinucleated forms. The
amphophilic cytoplasm and the perinuclear halo is classical of plasma
cell lineage ( Giemsa, 500).
stained smears are helpful in identifying the

amphophilic cytoplasm and a perinuclear halo. The
Bone marrow shows 15-25% plasma cells in various
stages of differentiation.
Case 8
A 17-year-old male presented with gradual inferior
displacement of right eye for three months and
blurred vision following a trivial sports injury. Firm,
fixed, slightly tender superomedial orbital mass
observed. CT scan shows a soft tissue mass with
patchy enhancement in the superior orbit (Figure
4.8A.) Peripheral blood smear was normal. Incision
biopsy revealed a greenish yellow (chloroma) solid
tumor in the peripheral surgical space. Squash imprint
preparations of tissues showed a monomorphic
round cell tumor. However the characteristic feature
is the pale staining nucleus, irregular nuclear
membrane, and pinkish cytoplasm. The Giemsa
stained smears are of great importance in confirming
the blast like morphology with cytoplasmic granules
and sometime Auer rods can also be identified
(Figures 4.8B and C), which confirms the diagnosis
of leukemic deposits. Bone marrow biopsy was
normal in this patient, at the time of orbital
presentation suggesting the extramedullary leukemic
deposits.
C
Figures 4.8A to C: (A) CT scan orbit (coronal cut) shows a wellcircumscribed, homogenous, hypodense soft tissue mass, with no
surrounding bony changes, displacing the orbital contents inferiorly.
(B) The smears show large cells with scant to moderate amount of
blue cytoplasm with lack of cohesion. (C) The higher magnification
shows the cytoplasmic Auer rod, confirming the diagnosis of
granulocytic sarcoma (Giemsa, 1000)
Case 9
A 6-year-old female presented with an increasing
swelling below the left eye for 1 month, On
examination, there was a firm mass in inferonasal
orbit with non axial proptosis and superolateral
displacement of the eye. CT scan showed a, wellcircumscribed, smooth soft tissue extraconal mass
displacing adjacent medial rectus muscle and globe.
The tumor was completely excised. Squash and
imprint smears were highly cellular with irregular
cells with marked pleomorphism of nuclei and
cytoplasm. The cells with pink tongue like
projections, spindle cells are helpful in suggesting
Rhabdomyoblastic differentiation (Figures 4.9A to
D). The cells contained moderate to abundant amount
of cytoplasm staining deep blue and containing
occasional small glycogen vacuoles. Few cells show
ill-defined relatively dense cytoplasmic inclusion.

Tumor cells are found singly, but loose clusters also
seen. Based on degree of myogenic differentiation
Akhtar et al. 14 divided Rhabdomyoblasts into 3
categoriesEarly Rhabdomyoblasts are round
undifferentiated cells with high nuclear: Cytoplasmic
ratio. Intermediate Rhabdomyoblasts have relatively
abundant pale staining cytoplasm and one or more
irregular nuclei with occasional nucleoli. Late
Rhabdomyoblasts contain abundant cytoplasm
staining grayish blue and opaque. These cells vary
from round to markedly elongated. Some cells show
localized inclusion like grayish blue area within
cytoplasm. The differential diagnosis include:
Retinoblastoma, Burkitt's lymphoma, metastatic
Neuroblastoma, PNET/Ewing's sarcoma and
myeloid leukemia can all present in orbit.
Figures 4.9A to D: (A) Photograph shows a swelling in the inferomedial orbit visible as a swelling in the lower lid and upward displacement
of the globe. (B) CT scan, axial view shows a hyperdense mass in the medial orbit, indenting the globe. The mass is homogenous. (C) The
cytology smears show clumps of cells with pink cytoplasm. Note the presence of cells with tongue like projections a one pole of the cells.
(Hematoxylin and eosin 500) (D) The sections from the tumor show necrotic areas and large pink cells, characteristic of rhabdomyoblasts
Case 10
A 22-year-old female presented with pain, swelling
and decreased vision in right eye for 4 months.
Examination showed proptosis of right eye with
neurotrophic keratopathy and total ophthalmoplegia.
(Figure 4.10A) There was a firm, nodular, slightly
tender, immobile mass in superolateral orbit whose
posterior extent could not be palpated. MRI scans
showed a soft tissue mass in superolateral orbit
extending towards the orbital apex and another soft
tissue mass in cavernous sinus region on the same
side (Figure 4.10B). Differential diagnosis included
nonspecific orbital inflammation with Tolosa-Hunt
syndrome and Adenoid cystic carcinoma with
intracranial extension. Incision biopsy was done from
the lacrimal gland. Squash and imprint preparation
showed the characteristic features of basaloid cells
in sheets, finger like processes, lacy pattern and the

classical 3-dimensional cell balls (Figures 4.10C and
D) with minimal nuclear pleomorphism. Permanent
sections showed cribriform pattern typical of adenoid
cystic carcinoma. A final diagnosis of adenoid cystic
carcinoma of the lacrimal gland was made with
perineural spread and intracranial extension into the
cavernous sinus giving rise to orbital apex syndrome.
Case 11
A 67-year-old female noticed a swelling in the upper
lid of the left eye 7 months ago, which enlarged to
involve the lower lid 3 months later and appearance
of multiple neck masses. On presentation she had
complete ptosis, there was a firm, immobile, nontender nodular mass in the anterior orbit palpable
through both lids. The mass was not fixed to
Figures 4.10A to D: (A) Photograph shows ptosis and proptosis on right side with corneal haze due to neurotrophic keratitis. The patient also
has total ophthalmoplegia and loss of sensation in distribution of Ist division of trigeminal nerve (B) T2 weighted MRI scan shows a hypointense
mass in the right lacrimal fossa extending posteriorly. Note the normal lacrimal gland in the left orbit. (C) The smears show classical magenta
pink rounded acellular matrix with nuclei wrapped around it. (3 D cell balls) (Giemsa, 500). (D) The sections from the tumor shows
characteristic cribriform pattern of basaloid cells ( 100 Hematoxylin and eosin)

overlying skin. The lids were immobile and the
conjunctival surface could not be inspected. The mass
extended beyond the lower orbital rim, onto the
cheek. There was little blood on the lid margin and
eyelashes were intact (Figure 4.11A). She also had
an enlarged preauricular lymph node on the same
side and bilateral neck glands in both anterior and
posterior triangles. There were no other systemic
abnormalities. CT scan showed a soft tissue mass
involving the lids and anterior orbit (Figure 4.11B).
FNAC was done from the orbital mass and lymph
nodes which showed large pleomorphic epithelial
cells, seen in tissue fragments, small clumps as well
as singly. The cells show abundant vacuolated
cytoplasm and vesicular nucleus (Figure 4.11C). In
one of the smears, an Oil Red O staining was done
which showed prominent bright orange red globules
within the cytoplasm of tumor cells (Figure 4.11D),
thus confirming the diagnosis of sebaceous gland

carcinoma, with orbital invasion and regional lymph
node metastasis.
Case 12
A 45-year old male presented with a left upper lid
droop and diplopia for 1 month. There was a firm,
non-tender mass in lacrimal gland region,
inferomedial displacement of the globe and limitation
of elevation and abduction (Figure 4.12A). Fundus
examination showed choroidal folds and globe
indentation. CT scan showed a soft tissue mass in
lacrimal gland region with erosion of orbital roof
and intracranial extension. Orbital part of the tumor
was removed through a lateral orbitotomy. The
Squash and imprint smears showed clumps of large
polygonal cells with abundant cytoplasm, cytoplasmic
pink inclusions, a large vesicular nucleus with
Figures 4.11A to D: (A) Photograph shows irregular swelling of both eyelids of left eye causing mechanical ptosis and extending beyond the
inferior orbital rim. The lids could not be opened or everted for further examination. (B) CT scan shows a soft tissue mass involving the anterior
orbit. (C) The smears show large epithelial cells with classical vacuolated cytoplasm. Note the presence of lymphocytes in the background
(hematoxylin and eosin, x 500) (D) The Oil Red O staining fo the cytology smear shows the presence of bright orange fat deposits (Oil Red O
staining, x 500)

prominent inclusion like nucleolus.(Figure 4.12B).
Histology confirmed a metastatic carcinoma with a
trabecular pattern, intracytoplasmic ropy secretions,
osseous metaplasia and occasional cell with bile plugs
(Figure 4.12C). Subsequently, the abdominal
ultrasound scan showed a well-defined mass in the
right lobe of liver. Metastatic deposits from breast,
lung, thyroid, hepatocellular carcinoma may be seen
in the orbit and show similar appearance as seen in
the primary location. This is one of the important
indication of fine needle aspiration cytology of orbital
lesions which influences the surgical management of
the case.
In summary, it is important for the
ophthalmologists to apply cytology to ocular lesions
which can result in early diagnosis with less invasive

techniques, sometimes obviating the need for a
surgery.
Figures 4.12A to C: (A) Photograph shows non-axial proptosis with downward displacement of the left globe and fullness of the upper lid
sulcus. (B) The cytology smears show large plemorphic epithelial looking cells with vesicular nucleus and prominent nucleoli. Note the
cytoplasmic eosinophilic inclusions, which was reported as metastatic carcinoma. (C) The permanent sections revealed the trabecular pattern
osseous metaplasia and bile plugs in few cells (hematoxylin and eosin, 500)
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Pathology of the Orbital Diseases 97
CHAPTER
Pathology of the
Orbital Diseases
KS Ratnakar
INTRODUCTION
It is a common practice to include tumor-like
conditions such as inflammatory lesions, cysts and
lymphoid hyperplasias which mimic true neoplasms
in their clinical manifestations under the general term
orbital tumors. Approximately, 70% of all orbital
tumors originate from the orbital tissues while 30%
invade the orbit from adjacent structures or
metastasize from distant primary foci. The relative
incidence of the pathological lesions causing
proptosis reflects the bias of the reporting discipline,
namely, surgical, ophthalmological or radiological.
CLASSIFICATION
The classification essentially follows the tradition of
considering the lesions as congenital/developmental,
inflammatory and neoplastic. An outline of the
classification is shown in Table 1.
Table 1: Classification of orbital lesions
1. Developmental
a.
Sphenoid wing dysplasia
b.
c.
d.
Neurofibromatosis
i. Optic glioma
ii. Optic meningioma
iii. Optic schwannoma
Elephantiasis neuromatosa
Plexiform neurofibroma
e.
f.
g.
h.
Fibrous dysplasia
Dermoid/epidermoid
Hamartoma
Meningioencephalocoele.
2. Inflammatory
a. Orbital cellulites
b. Idiopathic Orbital inflammation
c. Abscess
d. Parasitic-cysticercus', hydatid
e. Granuloma: sarcoid, wegener's, tuberculosis, fungal.
3. Traumatic
a. Penetrating injury
b. Foreign body
c. Hematoma
4. Neoplastic
Lesions may be of benign or malignant type.
Benign lesions
a. Conal and intraconal
i. Hemangioma Cavernous (adult)
Capillary (child)
ii. Lymphangioma
iii. Hemangiopericytoma
iv. Neurofibroma
v. Meningioma
Malignant lesions
a. Conal and intraconal
i. Lymphoma
ii. Metastasis
iii. Rhabdomyosarcoma
iv. Neuroblastoma
v. Ewing's sarcoma
b. Optic nerve/sheath
i. Optic glioma
ii. Hemangiopericytoma
iii. Leukemia
5. Vascular
a. AV malformation
b. Carotidcavernous fistula
c. Venous varix
d. Superior ophthalmic vein thrombosis
6. EndocrineGrave's ophthalmopathy
7. MiscellaneousMucoceles
DIAGNOSIS OF ORBITAL TUMORS

Space occupying lesions, involving the orbit, produce
symptoms and signs by compression, infiltration
and/or infarction of orbital structures. In the final
analysis, the clinical presentation will result from
displacement and /or dysfunction of the globe, optic
nerve, oculomotor nerve and blood vessels.
Proptosis: It is a common feature of all orbital
tumors, though its degree may vary. Those within
the muscular cone, (e.g. optic glioma, hemangioma
and meningioma) usually produce axial proptosis, but
those outside the muscle cone, (e.g.dermoid, lacrimal
gland tumor, neuroma) tend to push the eye opposite
to that of the lesion, to cause eccentric proptosis.
Optic neuropathy: Optic nerve involvement may
result in: (a) Progressive visual loss associated with
edema of disc. In many patients, however, visual loss
may be minimal and of delayed onset. In such cases,
testing for color vision may reveal subtle defects even
when acuity is nearly normal, (b) Unilateral transient
visual loss which may occur in certain positions of
gaze and clears when the direction is changed, and
(c) A specific triad may develop in chronic
compression of the optic nerve, namely loss of vision,
swelling of disc which resolves into optic atrophy
and appearance of optociliary shunt veins, (e.g. in
spheno-orbital meningiomas).
Oculomotor paresis: The tumors located in
orbital apex may involve oculomotor nerves in early
stage even before causing proptosis. Some tumors
may involve one or two muscles, till late in the
disease. Diplopia produced by orbital masses may
be neurogenic or myogenic and rarely it may be a
combination of both. The mechanical restoration of
ocular mobility can be confirmed by performing
certain tests. Forced duction or traction test and
intraocular pressure increase on looking in the
direction of gaze limitation are the tests of choice
for this purpose.
Pain: Most of the lesions are painless. Generally,
pain is more frequent with malignant tumors. Lesions
that involve cavernous sinuses and paranasal sinuses
are usually painful.
Pupillary abnormalities: These abnormalities can
occur depending upon the involvement of the
parasympathetic or sympathetic nerves, but this is
often marked by involvement of oculomotor nerve

palsy.
DEVELOPMENTAL LESIONS
Sphenoid Wing Dysplasia
Pulsating exophthalmos results due to defective
development of sphenoid wing and roof of the orbit.
About one-half of the cases are associated with
neurofibromatosis. The lesion is evident in the early
years of life, starting with ptosis and thickening of
the upper eyelid when it is associated with
neurofibromatosis, followed by protrusion of the eye
ball. The vision is spared till late. There is usually a
bulge in the temporal fossa. X-rays of the skull reveal
the underlying bony deficits such as elevation of
sphenoid ridge, enlargment of the orbit, absence of
temporal line of the sphenoid wing, giving the
appearance of an " empty orbit".
Neurofibromatosis
Neurofibromatosis involves the orbit either alone or
in association with sphenoid dysphasia and produces
a variety of orbital lesions in bony and soft tissue
segments.
The peripheral nerve sheath tumors are
histologically characterized by the presence of
pallisading spindle cells with micro or macrocystic
changes. The collagen content may vary. In plexiform
type, neurites in bundles may be seen within the
stroma.
Case report: A 15-year-old boy presented with
proptosis and diminished vision in the left eye. Intraorbital soft tissue lesion was excised. He had in
addition bilateral acoustic neurinomas and intradural
neurofibroma in the lumbar region. The histological
study revealed wavy spindle cells embedded in lose,
fibrillary matrix. At places whirling bundles of
neuritis are noticed (Figure 5.1).
Meningioencephalocoeles
Sinonasal encephaloceles involve the medial orbital
wall and encroach into the orbit causing
hypertelorism. The tissues include either meninges
alone or brain parenchyma.
Case report: A 12-year-female has reported with
proptosis, predominantly affecting the upper medial

They are invariably benign and can be excised with
ease.
Case report: A 6-year-male child admitted with
bulging at the root of nose along with fullness in the
upper medial compartment of right eye. Radiological
investigations showed a mass involving the anterior
base of the frontal bone with orbital extension of the
lesion (Figure 5.3).
Hamartoma
Figure 5.1: Spindle cells showing wavy pattern in a case of

Neurofibroma. H and E 400
aspect of left eye. Radiological examination including

CT scan and MRI revealed a cranial defect in the
upper orbital wall with small cerebral parenchyma
protrusion into sac covered by meninges. At
operation, CSF leakage was there and the sac contents
along with contents are excised (Figure 5.2).
Hamartoma is a type of congenital lesion composed

of tissues normal to the location. One type of
tissue may be predominantly seen such as vascular
tissue.
Fibrous Dysplasia
These tumors result from embryonic ectodermal

sequestration.
These are rare, found inside the orbit, and most
of them are located in the periorbital region.
Dermoids and epidermoids have the same
histological features as seen elsewhere in the body.
Fibrous dysplasia is a non-neoplastic disease of the

bone that affects children and young adults.
Craniofacial fibrous dysplasia is a benign condition
forming about 3% of all bone tumors. Dysplasia of
frontal, sphenoid, ethmoid, zygomatic and maxillary
bones may involve the orbit causing visual
symptoms. The symptoms may range from
progressive visual loss to proptosis or orbital
distortion. The lesion is usually painless. On plain Xrays the lesion appears as ground glass, milky and
sclerotic and in most cases some areas of radiolucency
may be observed. Histological changes are consistent
with an arrest in the development of immature
woven bone into more mature lamellar bone
Figure 5.2: Histological section showing Neuroglial elements in the

wall of the Meningioencephalocoele. H and E 400
Figure 5.3: Section showing cyst lined by epidermis with dermal

appendages from a case of dermoid cyst H and E 400
Dermoid and Epidermoids
Figure 5.4: Woven bone without osteoblast rimming in a case of

fibrous dysplasia H and E 400
embedded in cellular fibrous stroma. No osteoblastic

rimming is seen around osseous islands. The woven
bone may appear as' Chinese figures' often described
to indicate their focal irregular pattern. The stroma
tends to be fibrous in older individuals (Figure 5.4).
INFLAMMATORY LESIONS
Orbital Cellulitis
Orbital cellulites can remain as a diffuse inflammation
or progress to loculation to form an abscess. There
is usually a profound disturbance of oculomotor
functions, pain and constitutional symptoms.
Occasionally, the abscess may become chronic and
the lesion may manifest like any begin space
occupying lesion. Pathology reveals diffuse
suppurative inflammation composed of neutrophils.
Idiopathic Orbital Inflammation

Idiopathic orbital inflammation is the most common
cause of an intraorbital mass.
The condition is usually diagnosed clinically with
classic symptoms of painful proptosis and restriction
of extraocular muscle often unilateral. It is a
challenging mimicker of an intraorbital neoplasm and
is often a diagnosis by exclusion of other surgically
remediable entities. The exact etiology is not known.
Autoimmune response to antigen has been
implicated. Perhaps the condition is multifactorial.
IOI is often a diagnosis of exclusion of specific
etiologies. Every attempt has to be made to find the

cause prior to labeling the condition as idiopathic
orbital inflammation.
Lymphoid lesions of the orbit continue to pose
problems to the ophthalmologists and pathologists
as well. Despite increase in understanding and
improved methodology the diagnosis is often difficult
and intrigues the treating surgeon.
The inflammatory lymphoid lesions which are
also called orbital idiopathic inflammation add
further confusion to the understanding of the disease
process.
Arnold and Becker (1972) and Hochheim (1900)
first attempted to classify the lymphoid lesions of
the orbit into four categories namely benign
lymphoma, follicular lymphoma, lymphosarcoma
and lymphatic leukemia. In these groups, the first
two possibly belong to the lesions of inflammatory
origin. Blodi and Gass(1968) carefully analysed the
non-neoplastic lymphoid lesions and divided them
into follicular, diffuse lymphocytic and benign
lymphocytic hyper plastic varieties. Morgan and
Harry (1978) studied 98 cases of lymphoid lesions
and considered large number of cases that remained
confined to the orbit are of inflammatory nature.
These lesions have been termed by them as
lymphocytic tumors of indeterminate nature.
These classifications in the present era of
molecular markers, are unscientific and redundant.
Clinically, the inflammatory benign lymphoid
lesions present with exophthalmos with conjunctival
edema, pain/inflammatory signs and occasionally
with palpable mass. There is no specific age or sex
predilection but appears to affect young adults
predominantly. Right eye appears to be involved
more frequently than the left and small percentage
is found to occur bilaterally. The duration of a
symptomatology varies from less than a month to
over several years. These tend to heal or improve
over a period of time with steroids and/or
antibiotics. Small but significant percentage of lesions
may focally recur, but do not necessarily indicate
malignancy in all the cases. However, careful clinical
follow-up is advised and a thorough systemic
examination. It is imperative to rule out any
disseminating malignant lymphoid lesions. Morgan
and Harry (1978) observed in over 25% of cases are
associated lymphoid malignancy elsewhere in the

body. This finding necessitates cautious thinking of
any lymphoid lesion in general.
However, Knowle et al. (1979) using molecular
techniques, made useful and important observation
that benign reactive lymphoid hyperplasias are
immunologically polyclonal whereas malignant
lymphomas are monoclonal in nature.
The lymphoid lesions apparently looking benign
may present primarily in the orbit with its systemic
involvement being observed at a later date. The
pathobiology cannot be clearly defined in the early
stages without application of molecular markers which
are rather defined. Hence the wisdom lies in careful
clinical and histological interpretation (Figure 5.5).
Orbital Infections
Aspergillosis
Aspergillosis is a fungal disease caused by aspergillus
fumigatus, the most common causative species.
Aspergillosis involving the orbit and cranial content
is rare. Paranasal sinus aspergillus infections are
classified as non-invasive and invasive types.
The invasive or fulminant type of aspergillosis
occurs primarily in immunologically compromised
individuals. The clinical manifestations include a
rapidly progressive gangrenous necrosis of
the mucoperiosteum, with destruction of nasal
bones of the paranasal sinuses and orbital wall. There
may be intracranial invasion. The orbital extension
Figure 5.5: Lymphoid aggregates with prominent germinal centers

from a case of pseudo-lymphoma H and E 400
is reflected by soft tissue densities extending from

the sinuses especially the ethmoids into the orbital
cavity. Some degree of bone destruction is identified
in combination with the fungal infiltrate.
The histology in acute form shows necrotising
vasculitis associated with soft tissue invasion by
fungal filaments. In chronic form, there is
granulomatous reaction and fibrosis (Figure 5.6) Intra
and extracellular aspergillus is demonstrable by
fungus stains such as PAS and silver methanamine
(Figure 5.7).
Case report: A 60-year diabetic reported with
painless proptosis of right eye of several weeks
duration. There was restriction of ocular movements
in all directions. Orbital exploration revealed a tough
soft tissue lesion which resisted complete dissection
and excision. Histology showed many granulomas
containg foreign body giant cells containing fungal
filaments characteristic of aspergillous species.
Tuberculosis
Orbital tuberculosis is rare. In endemic areas,
tuberculous infection should be considered as an
important entity in the aetiology of orbital
inflammation. Orbitomorphologically the lesion
shows caseating coalescing granulomas. There may
be variable degree of fibrosis. Similar histoplasmosis
may be encountered in brucellosis, histoplasmosis,
sarcoidosis. Hence thorough clinical examination
Figure 5.6: Histological section showing foreign body type of giant

cells in a granualomatous lesion. From case of Aspergilloma, orbit H
and E 600
Figure 5.7: Fungal filaments of Aspergillus species. Silver

methanamine 400
Figure 5.8: Cross-section of cysticercus. H and E 400
serological and/or cutaneous tests should be

conducted for final diagnosis.
Cysticercosis
Cysticercosis of the orbit is very rare in the western
literature, but Jacobiece and Font (1986) nath et
al. (1977) documented nearly 24 cases in the
literature. Sarada et al. (1981) found one case in
the orbit among 50 cases of cysticercosis of the CNS.
But in endemic areas orbital myocysticercosis is
fairly common. They are usually managed with oral
Albendazole and prednisolone. Only anteriorly
located cysticercosis is excised and is available for
histopathology (Figure 5.8). However, Murthy et
al. (1990) reported on the use of ultrasound in
preoperative diagnosis.
Cysticercus cellulosae, histologically, when
sectioned shows fibrocellular, reaction with
palisading histiocytes around the parasite. The worm
may get calcified in later stages. The cellular infiltrate
may be dominated by eosinophils.
Neoplastic Lesions
Classification of orbital tumors is given in Table 2
Benign tumors.
Table 2: Classification of orbital tumors

Primary
I. Mesenchymal
a. Vascular: hemangioma, lymphangioma, hemangiopericytoma, hamangioendothelioma, Angiosarcoma, Kaposis sarcoma,
b. Lipoma, liposarcoma
c. Fibrous histiocytoma,
d. Fibrosarcoma
e. Rhabdomyosarcoma
f. Leiomyoma, sarcoma
g. Chondroma, chondrosarcoma
h. Giant cell tumor, osteosarcoma.
II. Neural
Glioma, neurilemmoma, neurofibroma, amputation
neuroma.
III. Hemopoietic.
Lymphoma, leukemia, myeloma.
IV. Lacrimal gland
Plemorphic adenoma, adenoid cystic carcinoma.
V. Miscellaneous
Meningioma, nonchromaffin paraganglioma, alveolar
soft part sarcoma, malignant melanoma.
Secondary
I. Direct extension
a. Intraocular tumors retinoblastoma, melanoma
b. Eyelid tumors basal, squamous, sebaceous
carcinoma, melanoma
c. Conjunctival squamous carcinoma, melanoma
d. Metastatic in children neuroblastoma
In adultsLung Ca, Breast Ca.
BENIGN TUMORS
Cavernous Hemangioma
The most common benign intraorbital tumor in the
young and middle aged is cavernous hemangioma
that produces painless proptosis. It is common in
females and may enlarge during pregnancy.
Despite the mention that cavernous hemangioma
is the most common benign intraorbital tumor, there
have not been many reports of a good series of this
condition. Harris and Jacobiec (1979) could find 66
cases recorded during a 40 year period from three
centers dealing specially with ophthalmic problems.
Maroon and Kenerdell (1979) found five hemangiomas among 18 intraorbital tumors subjected to
microsurgical treatment. The same authors reported
17 hemangiomas among 300 cases of orbital
tumors seen from 1975 to 1982. Nath et al. (1977)
found 12 cases among 120 cases of primary orbital
tumors.
Being a benign slow growing tumor, it causes
progressive often painless proptosis. As the lesion is
usually situated behind the globe within the muscle
cone, it produces axial proptosis.
Despite the prominent protrusion of the eyeball,
vision is preserved and movements of the eye are
spared till late in the course. About half of the patients
in the series of Harris and Jacobiec(10979) had
blurred vision and only three of the 66 had diplopia.
Neither bruit nor pulsations were present in any of
their cases.
Microscopically, the lesion is composed of dilated
vascular channels. Some of the channels may contain
prominent smooth muscles when the lesions are
called venous hemangiomas (Figure 5.9).
Capillary Hemangioma
Figure 5.9: Histological section from a case of cavernous

hemangioma, showing widely spaced vascular channels rimed by
fibromuscular tissue. H and E 400
Figure 5.10: Polypoidal tissue composed of proliferating vascular

channels embedded in a inflammatory and edematous stroma from a
case of capillary hemangioma, pyogenic type. H and E 400
Capillary hemangioma is a benign tumor that

manifests usually in first five years of life and tends
to regress thereafter. It is generally single, bright
red and smooth lesion. Histologically, capillaries of
small size are closely packed with no smooth muscle
in between (Figure 5.10).
children and adolescents. Spontaneous hemorrhage

into the cyst leads to abrupt proptosis and formation
of chocolate cysts. Lymphangioma occurs in
extraconal location and presents with pain and
proptosis. Infiltration of muscles and nerves of orbit
also occurs in this condition (Figure 5.11) .
Lymphangioma
Hemangiopericytoma
There are no lymphatics in the orbit. However,

lymphangiomas do occur here and account for 0.5 to
3% of intraorbital tumors. It is commonly seen in
It is a rare lesion occurs in the fourth decade of life.

It may be malignant in about 12% cases. The lesion
consists of increased number of thin-walled vascular
Figure 5.11: Section showing dilated vascular channels with lymphoid aggregates in the mural compartment from a case of
Lymphagioma. H and E 400
channels with perivascular massing of pericytes

separated by tumor cells in a network of extracellular
material (Figure 5.12).
Meningiomas
Orbital meningiomas are of three types based on their
origin
1. Intracranial meningiomas which secondarily
invade the orbit are the commonest.
Meningiomas of the middle cranial fossa
especially those of sphenoid ridge are notorious
to cause proptosis. Meningiomas of the anterior
cranial fossa may invade the orbital roof. In
Figure 5.13: Section shows oval to polyhedral cells with vesicular

nuclei and lightly acidophilic cytoplasm arranged in nests and whorls.
There are scattered psammoma bodies, from a case of meningioma.
H and E 400
addition ectopic meningiomas arising from, for

example, the frontal sinus may encroach into the
orbit.
2. Primary intraorbital meningiomas are rare
tumors which arise from the optic nerve. This is
the most difficult one to treat. The
differentiation between optic nerve glioma
and meningioma is an important clinical
problem.
3. Those which have no apparent connection with
the optic nerve are occasionally found in the
orbit. These probably arise from ectopic
arachnoid cell nests in the orbit.
Intraorbital meningiomas are more common
in women than men. The average age of onset
is about 30 years. About 40% occur below 20
years. The main clinical features are loss of
vision and progressive exopthalmos. Neurofibromatosis may be associated within
16% of cases (Figure 5.13).
Malignant Tumors
Rhabdomyosarcoma
Figure 5.12: Histological section showing thin vascular channels

with oval to spindle cells oriented externally from a case of
Hemangiopericytoma. H and E 600
Rhabdomyosarcoma is the most common

malignant mesenchymal orbital neoplasm and
malignant orbital tumor in children. The average age
of onset is six years and the lesion is rare after 25
years. The onset is rapid and the progression
simulates cellulitis. Histologically, the tumors are of

three types (i) embryonal (ii) adult pleomorphic and
(iii) alveolar.
Of these three types embryonal rhabdomyosarcoma is the most common type of malignant
tumor. Structurally, tumors are composed of round,
oval or stellate rhabdomyoblasts with mitosis in
loose syncitium. Cells contain dense eosinophilic
cytoplasm with striations.
Mortality rate is 40.6%. Prognosis is best in adult
pleomorphic type and it is worst in alveolar type
(Figures 5.14 and 5.15).
Adenoid Cystic Carcinoma

It occurs in adults of either sex in the fourth
decade of life. The lesion is relentlessly
progressive, invading the adjacent tissues with
characteristic tendency to spread along perineural
lymphatics (Figure 5.16).
Lymphoma
Lymphomas in adults are encountered in 10%
biopsies of orbital tumors. They are usually found in
the anterior orbit. About 25% of lymphoma
presenting as NSOID may evolve into lymphomas.
They are essentially B cell lymphomas. Orbital
lymphomas may be the first manifestation of systemic
lymphomas (Figure 5.17).
Histiocytoma
Malignant fibrous histiocytomas are rare, and more
lethal lesions of orbit. These are common in older
age group. The origin of the tumor is from the
histiocytes,and the neoplastic histiocytes are bizarre
with areas of hemorrhage,necrosis and frequent
mitosis. Focal or diffuse storiform pattern
characteristic of histiocytomas is discernible .
Metastasis
Figure 5.14: Cellular lesion showing spindle cells with acidophilic
cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma H and E 400
It accounts for 5% of orbital tumors. In children,

neuroblastoma, ewing's and leukemia and in adults,
carcinoma of bronchus and breast are the common
Figure 5.15: Cellular lesion showing spindle cells with acidophilic

cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma. H and E 600
Figure 5.16: Large cellular islands of basaloid cells with focal cystic
pattern embedded in fibro vascular tissue. From a case of adenoid
cystic carcinoma of the orbit. H and E 400

bilateral, though the patient may present when it is
still confined to one orbit. Microscopic examination
shows edema, lymphocytic infiltration sometimes
forming follicles.
MISCELLANEOUS
Mucoceles
Mucoceles of the paranasal sinuses may require
neurosurgical attention when they involve the orbit
and/or cranial contents. The most frequent site is
the frontal sinus.
Figure 5.17: Cellular lesion composed of monomorphic round cells

with dark nuclei and scant cytoplasm seen in nodular aggregates
form a case of orbital lymphoma. H and E 400
primary sites. In about 50% of the cases the primary

remains unknown. Of all metastases 30% are orbital
and 70% ocular. The main symptoms are pain,
proptosis and limitation of extraocular movements.
Undifferentiated carcinomas arising in a paranasal
sinus account for 4-9% of unilateral exophthalmos.
The carcinoma of ethmoid most commonly involves
the orbit. Pain, proptosis, restricted ocular motility,
with ptosis occurring rapidly is a feature of
malignancy of the orbit.
Optic Glioma
Optic glioma are primary tumors of the optic nerve
and/or chiasma. They are usually low grade pilocytic
astrocytomas which may appear fibroblastic due to
invasion of the leptomeninges (desmoplastic) or
gelatinous with oligodendroglial component. Those
of the optic nerve may be intraorbital or intracranial
(Robertson and Broson, 1980; Alvord and Lofton, 1988).
Those involving the sphenoid and/or posterior

ethmoidal sinuses are rare. Chen et al. (1986) and
Nugent(1970) reviewed 63 cases in whom visual
impairment was noted in 71% of the cases. Optic
nerve damage may be caused by intracanalicular
extension of the lesion with erosion of the canal walls.
Periorbital swelling, pain and displacement of
globe are the frequent symptoms of frontal and
ethmoidal lesions. There may be a swelling over the
frontal sinus with crackling sensation on palpation
due to thinning of its anterior wall. Sphenoidal sinus
mucoceles cause headache, peri or retroorbital pain
and ophthalmoplegias due to extension into the
orbital apex and cavernous sinus (pompili et al. 1990).
Sellar involvement is seen frequently in
sphenoidal mucocele. Surgical intervention and
excision of the lesion is indicated to relieve pressure
on the orbital contents.
Graves Disease
The aetiology of mucoceles is multifactorial. The

basic cause seems to be obstruction of the ostium of
the sinus by a variety of causes such as inflammation,
trauma, polyps, previous surgery, allergy and benign
and malignant tumors. The exact precipitating factor
may not be evident in many cases (Weber and
Mikulis, 1987).
It is one of the most common causes of proptosis

which mostly affects females. Lid retraction is an
early sign. These orbital signs may manifest at any
stage of the endocrine dysfunction. Computed
tomography and ultrasonography may reveal the
characteristic thickening of extraocular muscles
without evidence of mass lesion. The lesion is often
Histological examination (HPE) reveals

fragments of polypoid tissue lined by pseudostratified ciliated epithelium. The sub epithelial
tissue is made up of loose stroma with pools of small
blood vessels and diffuse infiltration of mononuclear
cells and prominent collections of eosinophils
embedded with calcific spicules.
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CHAPTER
Thyroid-Associated
Orbitopathy
Peter J Dolman
Introduction
Thyroid-associated orbitopathy (also known as
Graves Orbitopathy, Graves Ophthalmopathy,
Thyroid Eye Disease) is an immune-mediated
inflammatory disorder causing enlargement of the
orbital muscles and fat (Figure 6.1).1,2 Its clinical
spectrum ranges from eyelid retraction and proptosis
with exposure complaints (Figure 6.2A) to more
serious problems such as orbital soft tissue
inflammation (with discomfort, eyelid and
conjunctival congestion and edema), extraocular
motility restriction, and loss of vision from
compressive optic neuropathy (Figure 6.3A).3
Severe cases may result in lasting cosmetic
disfigurement and functional visual impairment.
Quality of life studies have shown that it may have
Figure 6.2A: Mild TAO in a young female with bilateral upper lid
retraction (worse on the left side) and secondary ocular irritation and
epiphora
Figure 6.1: Axial CT scan of patient with asymmetric TAO

demonstrating right proptosis from marked enlargement of the right
orbital muscles and mild enlargement of the fat compartment. Compare
the affected right orbit with the normal left orbit
Figure 6.2B: Same patient at the time of surgery following a

posterior graded lowering of both upper eyelids

Between 25-50% of patients with immune thyroid
diseases develop orbital involvement, and of those,
5-10% may develop more severe consequences such
as severe inflammation and congestion, impaired
motility, or compressive optic neuropathy.7 A smaller
percentage may develop Graves lower limb
dermopathy (pretibial myxedema, with deposition
of subdermal hyaluronic acid), usually 1-2 years
following the onset of thyroid gland dysfunction and
shortly following severe orbitopathy, and a smaller
subset may develop acropachy (clubbing of the
fingers).8
Figure 6.3A: Patient with severe manifestations of TAO: VISA Score:

V = 1/1 (bilateral optic neuropathy with vision reduced to 20/100,
20/80); I = 9/10 (pain at rest and movement, +2 chemosis, +1 eyelid
edema, +1 caruncular edema, +1 conjunctival redness, +1 eyelid
redness); S = 3/3 (motility restricted to less than 15 upgaze bilaterally);
A = 3/3 (corneal ulceration, Hertel 26 mm bilaterally)
Figure 6.3B: Same patient following intravenous pulsed

corticosteroids, orbital decompression, and upper lid posterior
lowering. VISA Score: V = 0/1 (no optic neuropathy); I = 0/10; S = 1/
3 (eyes aligned but restricted to 30 motility); A = 1/3, mild (some
residual upper eyelid deformity, Hertel 21 mm bilaterally)
more significant lifestyle consequences than chronic

lung disease or diabetes mellitus.4
Incidence and Epidemiology

Thyroid-associated orbitopathy (TAO) is the most
common orbital disease in the Americas and Europe,
with an annual incidence in females of approximately
14 per 100,000 and approximately one-fifth that for
males.5 Anecdotally, it may be less prevalent (or
possibly causes fewer severe complications) in Africa
and South Asia. However, it does occur in all races
and ages, and is most common between the second
and sixth decades.6
Risk Factors, Predictive Variables for

Disease Severity and Associated Immune
Disorders
Risk factors for developing TAO include smoking,
life stressors, poorly controlled hypothyroidism
following radioactive iodine, and a positive family
history of orbitopathy.9,10
Predictive variables for developing more serious
consequences of TAO include male gender,
increasing age, smoking, and a rapid onset of
orbitopathy.11
Cigarette smoking has been shown by numerous
studies to be correlated strongly with the
development of TAO and a progressively higher
incidence of smoking is seen with more severe
disease.11-13
Patients with TAO have an increased probability
of developing associated immune diseases, including
superior limbic keratitis (SLK), myasthenia gravis,
diabetes mellitus, alopecia and vitiligo.14 Psychiatric
conditions such as bipolar affective disorder and
anxiety occur more frequently in patients with
thyroid dysfunction and with TAO.
Pathogenesis
The pathologic hallmark of TAO is a lymphocytic
infiltration of orbital muscle and fat with expansion
of these tissues from edema and deposition of
hyaluronic acid and other glycosaminoglycans.2
Patients exhibiting fat expansion alone may present
with proptosis and lid retraction. Those with muscle
enlargement and more inflammatory features may
develop proptosis, periocular inflammatory features,
and possible restriction of motility or strabismus if
fibrosis develops (Figure 6.4). In a limited number
Thyroid-Associated Orbitopathy 113

of patients with severe myopathy, or with a narrow
boney orbital apex, or with tight lids limiting anterior
displacement of tissues, compression of the optic
nerve may ensue (Figure 6.5A).
TAO is an immune-mediated disease which is
strongly associated with thyroid immune disorders
such as Graves' disease or Hashimoto's thyroiditis.
90% of patients with orbitopathy have a current or
past history of abnormal systemic thyroid hormone
levels, while others may develop abnormal levels in
the future. It is important to explain to patients that
the orbitopathy is associated with, but not caused
by, abnormal thyroid hormone levels, since patients
often believe that the orbital disease should resolve
once a euthyroid state is reached.
Thyroid gland epithelial cells have surface
receptors which bind thyroid stimulating hormone
(TSH, thyrotropin), a hormone secreted in pulses by
the pituitary to control the release of thyroid
hormone. In both Graves disease and Hashimoto's
thyroiditis, circulating thyrotropin-receptor
antibodies (TSH-R Antibodies, TRAb) are present
which can bind to these same receptor sites, and
initiate and perpetuate the disease.2 At least three
subtypes of TRAb have been identified, presumed
to arise from a small population of abnormal
B-lymphocytes: (1) TSI (thyroid stimulating
immunoglobulin), which causes hyperthyroidism;
(2) blocking TRAb, which prevents TSH from binding
to thyroid cells and results in hypothyroidism;
Figure 6.5A: Coronal CT scan near apex showing bilateral optic

nerve crowding. This patient had impaired central vision (20/70, 20/
40), bilateral reduced color vision, and a right afferent pupil defect.
VISA Score: V = 1/1 (optic neuropathy present)
Figure 6.5B: Axial CT scan of same patient showing adequate medial

wall decompression into ethmoid sinuses and resolution of optic
neuropathy (V = 0/1; central vision 20/20 both eyes)
Figure 6.4: Patient with TAO myopathy attempting to look up-wards.

She had bilateral upgaze limitation, the right reaching 30 and the left
reaching 10, based on the light reflexes on her cornea (VISA
strabismus score = 3/3)
(3) binding TRAb, which binds onto TSH receptors

transiently, and has little effect on overall thyroid
hormone levels.
These circulating antibodies are thought to be
mediators in orbitopathy as well, with the likely
target being the orbital fibroblast.2 Orbital fibroblasts
are present in extraocular muscle and in orbital fat.
Orbital fibroblasts from patients with TAO have
increased numbers of TSH-Receptors, which are
thought to bind to circulating autoantibodies (TRAb),
stimulating adipogenesis and deposition of
hyaluronic acid within orbital muscle and fat, the
histologic hallmark of TAO.

Orbital fibroblasts in patients with TAO
have also recently been shown to have an
increased number of receptors to insulin-like growth
factor-1 (IGF-1 R), and serum from patients with
TAO has been found to have circulating antibodies
directed against IGF-1 receptors.15 Binding of IGF-1
receptors has been shown to attract and activate Tlymphocytes and macrophages through inflammatory mediators, which may be the mechanism of
initiating and propagating the inflammatory and
immune cascade in TAO.16
Course of Disease
As with other immune disorders such as rheumatoid
arthritis or Sjogrens disease, TAO typically has a
progressive (active) inflammatory phase followed by
a stable (inactive) postinflammatory phase. This
pattern of the disease was first described by Rundle,
and the plot of orbital disease severity against time
has been called Rundle's curve.17,18 The steepness of
the graph in the active phase reflects the acuity of
progression, with a steeper slope often leading to
more serious sequellae.11
The duration of the active phase may last from
6-18 months, during which the patient may
experience inflammatory symptoms of orbital
discomfort, periocular and conjunctival edema and
redness, and progression in proptosis, strabismus or
optic neuropathy. Management during this phase is
aimed at modulating the immune response and
reducing the inflammation, usually with the use of
steroids, radiotherapy, or other immunosuppressive
agents, and hopefully limiting the destructive
consequences of the active phase.
A useful analogy for patients is that the
inflammatory phase is like a house on fire. While
ignited, efforts are made to staunch the flames or
allow them to smolder if not too severe. Reconstruction is not carried out while the fire is still active.
Once the disease has become quiescent, surgery
may be offered to rectify damage resulting from the
active stage, including reducing proptosis, aligning
muscles, narrowing eyelid apertures, and debulking
fat pockets in the eyelids. This would be similar to
repairs being carried out after the house fire was
suppressed.
Reactivation of disease is fairly uncommon, 19
occurring in less than 5% of individuals, and is
sometimes associated with a major life stressor such

as a family death, divorce or loss of job.
Clinical Classification
One of the challenges in TAO is how to classify and
grade its various clinical manifestations so that
appropriate management can be instituted.
Most ophthalmologists are familiar with
Dr Werners NO SPECS classification that graded
various symptoms and signs associated with the
disease and assigned a global severity score.20 While
this has served as a useful mnemonic for the different
features of TAO, it is weak in terms of defining
management and doesn't assess whether the disease
is in the active or inactive, postinflammatory phase.
In 1989, Drs Mourits et al introduced a clinical
activity score (CAS) to stage and grade the
inflammatory phase of the disease.21
The VISA Classification

We have recently introduced the VISA classification
which is a clinical recording form designed for the
office, and which separates the various clinical
features of TAO into four parameters: V (vision, optic
neuropathy); I (inflammation, congestion);
S (strabismus, motility restriction); A (appearance,
exposure).22
The basic visit form (Figure 6.6) includes the four
sections recording historical symptoms on the left
and signs on the right. After each section is a progress
row (better, same, worse), recording both the
patient's and clinician's impression of the course of
the disease since the last visit. The layout is designed
to simplify data recording and possible later research
data collation. Individual measurements may be
completed in as much or little detail as the clinician
chooses. At the end of the form is a summary grade
for the activity and severity for each of the four
disease parameters and a space for investigations and
management plan.
On the first visit, the date and rate of onset of
both the systemic and orbital symptoms should be
recorded, since this may help predict the ultimate
severity of the inflammatory phase.
a) Vision/optic neuropathy: The focus of this
section is to identify TAO optic neuropathy.
The history includes visual blurring or color
desaturation and the progress and duration of
symptoms.
Figure 6.6: Follow-up VISA classification form

Objective measures of optic neuropathy include
a loss in central visual acuity and color vision, an
afferent pupil defect, and infrequently congestion or
pallor of the optic nerve.
Ancillary testing includes coronal CT or MR Scans
to confirm crowding of the orbital apex,
standardized visual fields, and rarely VEP or optic
nerve head photos. As a summary grade, VISA lists
optic neuropathy as present or absent since most
clinicians would attempt some treatment for this
condition if present. The severity of the neuropathy
is reflected in the individual measurements of central
and color vision.
My usual treatment for TAO optic neuropathy
initially is high dose corticosteroids either by
intravenous route (1 gram Methyl-prednisolone over
30 minutes on alternate days for 3 treatments) or
oral route (100 mg prednisone daily on a tapering
dose) with an expectation that vision should improve
within days of therapy. In most cases, this treatment
will cause incomplete or only temporary visual
improvement so that surgical decompression of the
medial wall is required (through a Lynch,
transcaruncular or endoscopic transethmoidal
approach) in order to relieve pressure more
permanently on the optic nerve at the crowded orbital
apex (Figure 6.5A and B).
I often arrange adjunctive radiotherapy
subsequent to the decompression if the disease is
active to prevent further enlargement of the muscles
and recurrence of neuropathy. Radiotherapy is
administered using a lateral port focused behind the
globe to minimize the risk of retinal or lens exposure;
it is divided into 10 fractions of 200 rads over two
weeks23 and is contraindicated in diabetics because
of the risk of inciting or aggravating retinopathy.24
Although radiotherapy remains controversial, it is
still widely used and many clinicians believe it is
beneficial for certain aspects of TAO, including optic
neuropathy and significant inflammation.25 Success
of therapy for TAO optic neuropathy from both a
clinical or research standpoint is based on specific
improved measurements for central vision, color
vision and visual fields.
b) Inflammation/congestion: Symptoms of ocular
and periocular soft tissue inflammation include
orbital aching at rest or with movement, and eyelid
or conjunctival swelling and redness.
The Clinical Activity Score (CAS) described and

validated by Mourits and the Amsterdam
Orbitopathy group assigns one point for each of the
following: orbital pain at rest, orbital pain with
movement, chemosis, caruncular edema, eyelid
edema, conjunctival injection and eyelid injection.21
The VISA Inflammatory Score modifies the CAS
slightly by widening the grade for chemosis and lid
edema from 0-2. Chemosis is graded as 1 if the
conjunctiva lies behind the grey line of the lid and as
2 if it extends anterior to the grey line (Figures 6.3A
and 6.7A). Lid edema is graded as 1 if it is present
but not causing overhanging of the tissues, and as 2
if it causes a roll in the lid skin including festoons in
the lower lid (Figures 6.3A and 6.7A). The worst
scores from any of the four eyelids are recorded in
the inflammatory score table on the far right section
of the table. The pain score is based on the patient's
report of deep orbit discomfort rather than ocular
surface irritation (0 = no pain, 1 = pain with
movement, 2 = pain at rest). The additional grading
scores for chemosis and lid edema allow for
documentation of more subtle changes in
inflammatory features between visits. An additional
point is assigned for diurnal variation of symptoms,
to reflect the variability in congestion typically seen
during the active phase.
Treatment of active inflammation in TAO
depends on its inflammatory score and evidence of
progression. If the score is less than 4 out of 10, and
there is no deterioration based on history or
sequential clinical examination, conservative
management is offered with reassurance, cool
compresses, nocturnal head elevation, and nonsteroidal anti-inflammatories. In general, if the
inflammatory grade is 5 or more, or if there is
subjective or objective evidence of progression in the
inflammation, more aggressive therapy should be
considered, including oral or intravenous
corticosteroids, radiotherapy, and in refractory cases,
immunosuppressive agents (Figures 6.3A and B, 6.7A
and B). Combination therapy is receiving increasing
attention in severe, progressive cases 26, 27 and interest
is also turning to new immunomodulatory agents,
such as anti-tumor necrosis factor agents (etanercept,
infliximab) or B-lymphocyte directed therapy
(rituximab).28 The hope from greater understanding
of the immunogenic mechanisms in this disease is to
Figure 6.7A: Patient with severe active orbitopathy: V = 1/1 (optic

neuropathy present); I = 10/10 (2/2 pain, 2/2 chemosis with conjunctiva
overlying eyelid margins, 2/2 eyelid edema of left lower eyelid, 1/1
caruncular edema, 1/1 redness of conjunctiva, 1/1 redness of eyelid,
1/1 progression in symptoms over past month)
Figure 6.7B: Same patient 2 days following 3 doses of intravenous

methyl-prednisolone with marked reduction in inflammatory signs.
VISA Score: V = 1/1 optic neuropathy (improved vision, but still showing
impaired central and color vision); I = 4/10 (0/2 pain, 1/2 chemosis
with conjunctiva behind grey line of lower lid, 1/2 eyelid edema, with
resolution of eyelid overhang, 0/1 caruncular edema, 1/1 conjunctival
redness, 0/1 eyelid redness, 1/1 diurnal variation)
identify markers of progressive, more severe disease

so that earlier treatment and more specific
immunotherapy may be developed.
c) Strabismus / motility restriction: The symptoms
for strabismus include a progression from no
diplopia, diplopia with horizontal or vertical gaze,
intermittent diplopia in straight gaze, and constant
diplopia in straight gaze.
Ocular ductions can be graded from 0 to 45 in

four directions using the Hirschberg principle: the
patient is asked to look as far as possible up, down,
right and left while the observer points a bright light
at the eyes and studies the light reflex on the surface
of the eye. If the light reflex hits the edge of the
pupil, the eye has moved 15, between the pupil edge
and the limbus, 30 and at the limbus, 45 (Figure
6.4).
Strabismus can be measured objectively by prism
cover testing in different gaze directions.
Ancillary testing includes using the Goldmann
perimeter to quantify ocular ductions in four
directions.29, 30 The patient keeps both eyes open and
follows the V4e light target, tapping a coin when the
image becomes double.
Management of strabismus depends on whether
the orbitopathy is inflamed (measured in the previous
section) or if there is evidence of progression in
symptoms and signs. If inflammation is present, this
is managed first, either with conservative treatment
or with anti-inflammatories or radiotherapy (Figure
6.8A). During this stage, the strabismus can be
managed with patching one eye or with Fresnel
prisms. Once the inflammatory score has dropped
to zero and there is no evidence of progression,
management of strabismus might include prisms or
surgical alignment (Figure 6.8B).
d) Appearance/exposure: Symptoms in this category
include appearance concerns such as bulging of the
eyes, eyelid retraction and fat pockets, as well as
exposure complaints of foreign body sensation, glare,
dryness or secondary tearing.
Objective measures of appearance change include
eyelid retraction (measured in millimeters), proptosis
(measured with the Hertel exophthalmometer), and
documentation of redundant skin and fat prolapse.
Measures of exposure include corneal staining or
ulceration.
Photographs can document the appearance
changes.
Management of appearance and exposure changes
depend on the inflammatory stage of the disease.
During the inflammatory phase (documented
progression in any of the parameters or an
inflammatory score > 5), lubricant drops and

tempo of disease can be documented to reflect disease
activity. The classification system has been validated
by showing that two clinicians could use the forms
to assess patients independently with different
manifestations of TAO and to choose similar
management plans.22 It is currently being assessed
by members of the International Thyroid Eye Disease
Study (ITEDS) Group and further refinements and
validation will be conducted. A common method of
documenting and classifying the disease parameters
is critical for conducting multicenter clinical trials and
to assess response to different therapies.
Figure 6.8A: Patient with active TAO and strabismus (VISA Score:
I = 7/10, S = 3/3, strabismus in primary gaze). Anti-inflammatory treatment was instituted and one eye was patched for comfort
Figure 6.8B: Once the disease had progressed into the

postinflammatory (inactive) phase, alignment surgery was performed:
(VISA Score: V = 0/1; I = 0/10; S = 0/3, A = 0/3)
ointments can relieve ocular irritation. Rarely a

tarsorrhaphy or emergency orbital decompression
may be required for severe exposure or corneal
ulceration. Once the inflammatory phase has settled,
management for proptosis might include orbital
decompression and for eyelid retraction may include
upper lid lowering from an anterior or posterior
approach or lower lid elevation.31 These surgical
measures often relieve many of the exposure
complaints (Figures 6.2A and B, 6.3A and B).
e) Application of the VISA classification: The VISA
Classification clusters the four functions disrupted
by TAO in a logical sequence for recording and
management. Subjective input and reproducible
objective measurements are recorded for each section
and a global severity grade can be assigned for each
function. The subjective and objective progress and
General Management Guidelines

Patients with TAO are often misdiagnosed initially,
because the majority present with mild, nonspecific
complaints such as tearing, irritation and light
sensitivity as a result of exposure from mild lid
retraction. Occasionally mild inflammatory features
such as eyelid or conjunctival swelling may be
interpreted as allergies or viral infections. Many of
these patients are frustrated with their medical care
by the time the correct diagnosis has been reached.
More serious manifestations of the disease such as
myopathy, proptosis and visual impairment generally
develop rapidly and are more readily recognized.
Understanding what bothers the patient most
about their condition helps to build rapport and to
plan future management. Patients appreciate
reassurance and education about the natural course
of TAO. Clarify that their endocrinologist and family
practitioner will work to control any thyroid
dysfunction, and that while their orbitopathy may
be linked to thyroid immune disorders, a euthyroid
state does not necessarily lead to resolution of the
orbitopathy. Emphasize that they can take positive
steps to help their condition by quitting smoking and
relieving stressors in their lives.
I take the time to explain what is predictable
about the disease, mentioning that the disease is selflimited, and that those with a mild presentation and
younger age are unlikely to progress to more serious
complications. For those with more serious
complications, I use the house-fire analogy to clarify
the two stages of orbitopathy, and the role of antiinflammatory or immunomodulatory therapy in the
active phase and surgical reconstruction if necessary
in the postinflammatory stage. The internet provides
a number of support groups and I also identify

patients who I have treated who are willing to talk
to newly diagnosed patients to allow sharing of their
experiences.
REFERENCES
1. Bahn RS, Heufelder AE. Pathogenesis of Graves'
Ophthalmopathy. N Engl J Med 1993;329(20):1468-75.
2. Garrity JA, Bahn RS. Pathogenesis of Graves'
Ophthalmopathy: Implications for Prediction, Prevention
and Treatment. Am J Ophthalmol 2006;142(1):147-53.
3. Rootman J, Dolman PJ. Thyroid Orbitopathy (Chapter 8)
in: Diseases of the Orbit. A Multidisciplinary Approach.
Hagerstown: Lippincott Williams & Wilings, 2003.
4. Gerding MN, Terwee CB, Dekker FW et al. Quality of life
in patients with Graves' Ophthalmopathy is markedly
decreased: measurement by the medical outcomes study
instrument. Thyroid 1997;7(6):885-89.
5. Bartley GB. The epidemiologic characteristics and clinical
course of ophthalmopathy associated with autoimmune
thyroid disease in Olmsted County, Minnesota. Trans Am
Ophthalmol Soc 1994;92:477-588.
6. Kendall-Taylor P, Perros P. Clinical presentation of thyroid
associated orbitopathy. Thyroid 1998;8:427-28.
7. Burch HB, Wartofsky L. Graves' Ophthalmopathy: current
concepts regarding pathogenesis and management. Endocr
Rev 1993;14:747-93.
8. Fatourechi V, Pajouhi M, Fransway AF: Dermopathy of
Graves disease (pretibial myxedema). Review of 150 cases.
Medicine (Baltimore) 1994;73(1):1-7.
9. Perros P, Kendall-Taylor P. Natural history of thyroid eye
disease. Thyroid 1998;8:423-25.
10. Bartalena L, Marcocci C, Bogazzi F, et al. Relation between
therapy for hyperthyroidism and the course of Graves
Ophthalmopathy. N Engl J Med 1998;338:73-78.
11. Dolman PJ, Rootman J. Predictors of disease severity in
thyroid-related orbitopathy. (Chap18) Orbital Disease.
Present status and future challenges. Taylor and Francis,
2005.
12. Prummel MF, Wiersinga WM. Smoking and risk of Graves'
disease. JAMA 1993;269:479-82.
13. Pfeilschifter J, Ziegler R. Smoking and endocrine
ophthalmopathy: impact of smoking and current vs lifetime
cigarette consumption. Clin Endocrinol (Oxf) 1996;45:
477-81.
14. Cruz AA, Akaishi PM, Vargas MA, et al. Association
Between Thyroid Autoimmune Dysfunction and NonThyroid Autoimmune Diseases. Ophthalmic Plastic &
Reconstr Surg 2007;23(2):104-08.
15. Pritchard J, Horst N, Cruikshank W, et al. Igs from patients
with Graves' disease induce the expression of T cell
chemoattractants in their fibroblasts. J Immunol 2002; 168:
942-50.
16. Pritchard J, Han R, Horst N, et al. Immunoglobulin

activation of T cell chemoattractant expression in fibroblasts
from patients with Graves' disease is mediated through
the insulin-like growth factor 1 receptor pathway. J
Immunol 2003;170:6348-54.
17. Rundle FF. Development and course of exophthalmos and
ophthalmoplegia in Graves' disease with special reference
to the effect of thyroidectomy. Clin Sci 1945;5:177-94.
18. Rundle FF. Ocular changes in Graves' disease. QJM 1960;
29:113-26.
19. Selva D, Chen C, King G. Late reactivation of thyroid
orbitopathy. Clin & Exp Ophthalmol 2004;32(1),46-50.
20. Werner, SC. Classification of the eye changes of Graves'
disease. American J Ophthalmology 1969;68:646-48.
21. Mourits MP, Prummel MF, Wiersinga WM, et al. Clinical
activity score as a guide in the management of patients
with Graves' Ophthalmopathy. Clin Endocrinol 1997;47:
9-22.
22. Dolman PJ, Rootman J. VISA Classification for Graves'
Orbitopathy. Ophthal Plast Reconstr Surg. 2006;22(5):
319-24.
23. Beckendorf V, Maalouf T, George J-L, et al. Place of
radiotherapy in the treatment of Graves' orbitopathy. Int J
Radiation Oncology Biol Phys 1999;43:805-15.
24. Viebahn M, Marricks ME, Osterloh MD. Synergism
between diabetic and radiation retinopathy: a case report
and review. Br J Ophthalmol 1991;75:29-32.
25. Cockerham KP, Mourits MPh, McNab AA, et al. Does
radiotherapy have a role in the management of thyroid
orbitopathy? Debate. Br J Ophthalmol 2002;86:102-04.
26. Kahaly G, Schrezenmeir J, Krause U, et al. Ciclosporin and
prednisone vs prednisone in treatment of Graves'
Ophthalmopathy: a controlled, randomized and
prospective study. Eur J Clin Invest 1986;16(5):415-22.
27. Bartalena L, Marcocci C, Chiovato L, et al. Orbital cobalt
irradiation combined with systemic corticosteroids for
Graves' Ophthalmopathy: comparison with systemic
corticosteroids alone. J Clin Endocrinol Metab 1983;56(6):
1139-44.
28. Leandro MJ, Edwards JC, Cambridge G. Clinical outcome
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lymphocyte depletion. Ann Rheum Dis 2002;61:883-88.
29. Gerling J, Lieb B, Kommerell G. Duction ranges in normal
probands and patients with Graves' ophthalmopathy,
determined using the Goldmann perimeter. Int
Ophthalmol. 1998;21(4):213-21.
30. Dolman PJ, Kendler D, Rootman J. Measuring ocular
excursions in Graves' Orbitopathy. (Abst) International
Congress of Ophthalmology 2004.
31. Looi, A, Sharma B, Dolman PJ. A Modified Posterior
Approach for Upper Eyelid Retraction. Ophthalmic Plast
Reconst Surg 2006;22(6).
Orbital Infections
CHAPTER
Shome Debraj, Walinjkar Jaydeep, Mukherjee Angshuman
Many disease processes such as cancers, infections,

or inflammations present with overlapping clinical
manifestations, because of the confined nature of the
orbital space. Also, with the multitude of muscular,
neurovascular, sensory, and glandular structures
located close to each other in this space, precise
anatomic localization of various biologic processes
can be difficult.
Orbital infections and inflammations present to
the clinician with similar findings: periorbital edema,
erythema, proptosis, and pain. History and clinical
examination determine the work-up required to
better define the disease process. Orbital infections
continue to be associated primarily with diseases of
the paranasal sinuses.1 Hemophilus influenza type B
is no longer a significant pathogen, because of an
effective vaccine.2 Fungal infections extending to the
orbit are becoming more frequent, due to the
increased prevalence of immunocompromised
patients.3
Infections of the orbit are uncommon, but they
are potentially devastating infections that can quickly
result in blindness, meningitis, or death. The
emergency physician must make a rapid and accurate
diagnosis and then quickly initiate therapy because
visual loss is associated directly with the length of
time to definitive treatment.
Smith and Spencer4 classified orbital infections
into 5 tiers which were later modified by Chandler
et al.5
Group I Preseptal cellulitis
Group II Orbital cellulitis
Group III Subperiosteal abscess
Group IV Orbital abscess

Group V Cavernous sinus thrombosis.
This classification system does not necessarily
imply an order of disease progression; however, it
helps explain the physical signs and symptoms of the
various infections and helps organize treatment plans.6
Orbital infections may be divided into preseptal
cellulitis, in which infection is located anterior to the
orbital septum (a thin sheet of fibrous tissue arising
from the periosteum of the orbital margin and
inserting into the tarsal plates), and orbital cellulitis,
in which there is infection of orbital tissues posterior
to the orbital septum.7 Preseptal cellulitis generally
responds to oral antibiotics and rarely has important
sequelae. However, orbital cellulitis is a serious
infection which may be complicated by abscess
formation (subperiosteal, orbital, or brain),
meningitis, septicemia, cavernous sinus thrombosis,
and death. Although orbital cellulitis is related to
ethmoid sinusitis in 70-80% of cases8, it may also
develop after orbital or sinus trauma.9, 10 Prompt and
appropriate management of patients with orbital
cellulitis or at risk of developing this minimises the
risk of complications. 10 The possible causes of
Mortality/Morbidity following orbital cellulitis are:
Cavernous sinus thrombosis
Brain abscess or meningitis
Permanent vision loss.
Demographic Profile
Sex
Males are affected slightly more often than
females.
Orbital Infections 121
Age
Orbital infections are more common in persons
younger than 19 years
Orbital infections are more severe in adults.
Risk Factors
Past medical history significant for HIV, diabetes,
immunosuppression, steroid use, renal disease, and
travel is important.
Chronologic relation with an insect sting, allergic
reaction, or trauma may suggest etiologies that mimic
an orbital infection.
Etiological Causes of Orbital Infections
not uncommon. The causative organisms include E

granulosus, T solium,Trichinella spiralis and the
Onchocerca.15
Protozol infections
Although relatively uncommon, protozoal infections
(most commonly with toxoplasma gondii) are seen
in immunocompromised individuals.16
They have an increased likelihood for more
severe and atypical presentations; this highlights the
need for increased index of suspicion for HIV
infection as ocular or orbital disease may be the first
manifestation of life-threatening systemic
toxoplasmosis.
Bacterial infections
Diagnosis
Bacteria, most commonly Streptococcus species, as

well as Staphylococcus aureus, Hemophilus
influenzae, and anaerobes, cause the vast majority
of orbital infections.11
The incidence of Hemophilus influenzae type B
has decreased since the early 1990s.2 In communities
where CAMRSA is prevalent, ophthalmologists
should maintain high index of suspicion and obtain
microbial cultures and sensitivity studies to help
guide antibiotic therapy for severe ophthalmic
infections.12
The diagnosis of orbital infections includes the

following:
A detailed history
Vision
Slit-lamp examination
Extraocular motility
Examination to document optic nerve function;
including pupillary function
Fundus examination
Resistance to retropulsion
Exophthalmometry: Hertel's exophthalmometry
is the gold standard.
Fungal infections
Fungal infections are less common than bacterial
infections and occur more commonly in patients who
are immunocompromised (e.g., those with HIV or
diabetes). (Rhizopus, Mucor, Aspergillus)13
Phycomycosis, also known as mucormycosis, is
the most common and most virulent fungal disease
involving the orbit. The specific fungal genus involved
is usually Mucor or Rhizopus. These fungi can involve
the blood vessel wall and produce thrombosing
vasculitis.Therapeutic measures include controlling
the underlying metabolic abnormality, local surgical
excision of infected tissues, and administration of
amphotericin B.14, 3
Parasitic infections
Parasitic diseases may be prevalent in endemic areas
(e.g. Mediterranean, Eastern Africa, Australia,
Middle East Asia, South America, Eastern Europe)
and in travelers to these areas, such infections are
Imaging Studies17
Computed Tomography (CT) scan A CT scan
of the orbit, sinuses, and frontal lobe is essential
for every patient showing signs of orbital
involvement. 2 mm cuts are ideal for the orbit.
Both Axial and coronal scans shoud be carefully
looked at, to obtain a 3-dimensional perspective.
Magnetic Resonance Imaging (MRI) While a
CT scan provides enough information in most
cases, a MRI scan may improve visualization of
cavernous sinus thrombosis.
Ultrasonography (USG) B-scan An USG Bscan is mostly supportive in role, due to
difficulty in interpretation of posterior orbital
lesions.
X-rays Waters, Caldwell, submental vertex,
and lateral view are mainly of historical interest.
Other Tests
Fiberoptic nasopharyngeal endoscopy:18 If any
suspicion of mucormycosis (i.e. elevated blood
glucose, leukemia, renal disease) exists,
fiberoptic nasopharyngeal endoscopy should be
performed (usually, by an otolaryngologist) to
seek evidence of black eschar formation.
Endoscopy assisted transnasal drainage is also
useful in cases with evidence of associated sinus
infections.
Rapid plasma reagin (RPR), particularly in cases
of insidious onset or with a history of syphilis.
Cerebrospinal fluid (CSF) analysis for gram stain,
cell count, cultures, and antigens may be
considered in patients with associated central
nervous system signs.
Emergency Department Care

Adults with preseptal cellulitis and no signs of
orbital involvement can be discharged on oral
antibiotics with close follow-up care.19
Adults with orbital signs need admission
and IV antibiotics or antifungals are quickly
initiated, under close supervision.
Surgical intervention should be performed
immediately, in cases with CT evidence of
subperiosteal or orbital abscess for
drainage.19
Surgical drainage is not always necessary
for cellulitis; however, any patient with
compromised vision (20/60 or worse)
should receive immediate surgery for
drainage and debridement.19
Surgical drainage of abscesses (orbital or
subperiosteal) is considered, even without
visual loss. Drainage of sinuses should be
considered in patients with associated
sinusitis.
Some patients can be monitored for 48
hours on IV antibiotics, with surgery
performed for increasing proptosis,
worsening visual acuity, or isolated muscle
weakness. Surgery is performed after 48
hours if fever continues or antibiotics fail.20
Orbital drainage decreases intraorbital
pressure, decreases associated pressure on
the nerve and retinal circulation, creates a
potential path for further drainage (the

wound is closed with spaced sutures) and
provides material for appropriately guided
antibiotic therapy. We recommend orbital
drainage by a trained orbital specialist as
tissue in these cases are extremely vascular,
friable and susceptible to damage.
All children are admitted prior to initiating
therapy21 even if they lack orbital signsbecause children are deficient in IgG2 and
are predisposed to bacteremia.
For orbital cellulitis, oxacillin or nafcillin can be used
with the addition of ampicillin and sulbactam in
children to cover H influenzae.21 Patients who are
allergic to penicillin can use vancomycin, clindamycin,
or chloramphenicol. More and more organisms in
countries like India are becoming resistant to
conventional antibiotics. Methicillicin resistant
Staphylococcus Aureus (MRSA) strains are not only
prevalent in the nosocomial environment but are also
found in the community. MRSA orbital cellulitis can
quickly progress to irreversible blindness, despite
antibiotic treatment.
Alternatively, a cephalosporin (e.g. cefuroxime,
cefoxitin, cefotetan) can be used alone.
Nasal decongestants can be used to help drain
the sinuses.
Concomitant steroid therapy, once clinical
improvement is documented on antibiotics, is
started to decrease inflammation associated
collateral damage to tissue and edema, thereby
further decreasing intraorbital pressure.
Further Inpatient Care

Underlying disorders, if present (e.g. hyperglycemia, acidosis, infection, immunosuppression) are
corrected.
Consultations: Consultation with an ENT
surgeon or a neurosurgeon must be considered in
cases without improvement, or in cases with
involvement of sinuses or the nervous system.
Necrotizing fascitis, Orbital Tuberculosis and
Community Acquired MRSA (CAMRSA) induced
orbital cellulitis are rare entities in the spectrum of
orbital infections. Although these infections are not
so common, ophthalmologists should be well aware
of these conditions, especially in endemic areas as

the morbidity and mortality of selected cases are
reduced with prompt and appropriate antimicrobial
therapy. Keeping in mind this fact we find it
appropriate to present the following case reports:
CASE ILLUSTRATIONS
Case 1
A 25-year-old systemically healthy male patient
presented with complaints of severe photophobia,
redness, discharge, pain and severe swelling of the
lids in the left eye, since 2 days. Past history was
significant for a boil on the lower eyelid, 2 days ago.
On examination, the best-corrected visual acuity was
6/6 and 6/9, in the right and left eyes respectively.
Right eye examination was unremarkable. The left
eye showed severe lid edema with scales on the skin
and was diagnosed as having left sided periocular
necrotising fascitis with associated keratoconjunctivitis (Figure 7.1A). The cornea showed
multiple marginal infiltrates. Photographic
documentation of the anterior segment condition was
impossible because of the severe photophobia.
Extraocular movements were full. A conjunctival
swab and a periorbital skin swab were sent for
culture and sensitivity. The corneal infiltrates were
also cultured on Blood agar and Sabouraud's
dextrose agar. The patient was seen by our infectious
diseases expert and started on intravenous coamoxiclav 1 gram twice daily, intravenous ceftriaxone
1 gram twice daily and oral metronidazole 500 mg
three times daily, pending sensitivity reports. Topical
Lotepred eye drops every 3 hourly and ciprofloxacin
(0.3%) eye drops 6 times a day were started, in the
left eye.
On follow-up 2 days later, the patient was
symptomatically much better. The skin scabs had
fallen off, revealing violaceous, sub-epidermal
necrosis. The conjunctival inflammation had reduced
and the corneal marginal infiltrates had almost
disappeared (Figure 7.1B). Culture and sensitivity
results showed Staphylococcus aureus, sensitive to the
administered medications. The culture plates for
corneal infiltrates showed negative growth and were
discarded after 3 weeks. The patient was seronegative for HIV.
Five days later, the skin lesions had healed and
the conjunctivitis had resolved. Intravenous
antibiotics were stopped and the patient was started

on oral antibiotics for a week.
On final follow-up a month later, periocular
skin discoloration was the only sequalae noted
(Figure 7.2).
Discussion
Necrotising fascitis (NF) is a serious life threatening
condition, with reported mortality of more than 20%.
The limbs, perineum and abdomen are frequently
involved with facial involvement being rarely
involved. The organisms most closely linked to NF
are Group A beta-hemolytic streptococci, though
these bacteria are isolated in only a minority of the
cases.20
Figure 7.1A: Periocular necrotising fasciitis: External photograph of

patient at presentation, showing left sided severe lid edema, erythema
and necrotic tissue with overlying skin scabs
Figure 7.1B: Periocular necrotising fasciitis: Slit lamp photograph of

the left eye, in diffuse illumination (with upper eyelid retracted),
showing periocular skin erythema and kerato-conjunctivitis

ocular NF and the hitherto unreported anterior
segment involvement.
Case 2
Figure 7.2: Periocular necrotising fasciitis: External photograph of

patient, a month post presentation, showing healed skin lesions, with
symmetrical palpebral apertures
Periocular NF is reported to have a better

prognosis.22 Though reports of resolution of periocular NF postsurgical debridement are common, a
detailed Medline and Embase search revealed only
one case report which was reported to resolve with
conservative management.23 Infections in the periocular region occur postsurgical procedures, post
trauma, postfurunculosis or even without any
antecedent cause.23
Ideally, a combination of intensive parenteral
antimicrobial therapy and prompt surgical
debridement of necrotic tissue should be performed.
Intravenous pooled immunoglobulin and heparinisation may also have beneficial roles by neutralising
super-antigen activity and aiding antibiotic
perfusion.24 Mild cases especially those restricted
to the eyelids alone may respond to medical
therapy.23
We report the case of a 25-year-old male patient
who presented with periocular NF associated with
keratoconjunctivitis. We report this case to highlight
the successful conservative management of peri-
A 60-year-old systemically healthy female was

referred with a 2-month history of irritation in the
left eye associated with a mass in the lower lateral
orbit. Her visual acuity was 20/20 in both eyes. The
right eye was unremarkable. The left eye was 2 mm
enophthalmic by Hertels exophthalmometry but
showed no displacement (Figure 7.3A). Ocular
motility was full in range. There was a nontender,
hard, lobulated mass measuring about 30 mm 10
mm in size in the inferior orbit, felt separate from
the inferior orbital rim. The mass appeared to be
contiguous with the globe. General physical
examination including chest roentgenogram was
unremarkable.
Computed tomography scan showed an illdefined extraconal anterior orbital mass located
inferotemporal in the left orbit, with minimal contrast
enhancement (Figure 7.3B). Differential diagnoses of
scirrhous breast cancer metastasis or sclerosing
orbital pseudotumor were considered and a biopsy
was performed by the inferior conjunctival fornicial
approach.
Histopathology revealed features of noncaseating granulomatous inflammation and fibrosis
(Figure 7.4A). AFB was negative both by smear and
culture. PCR for Mycobacterium tuberculosis DNA
was positive (Figure 7.4B). Orbital tuberculosis was
diagnosed and the patient received four-drug
combination antitubercular therapy for 6 months. The
orbital mass completely regressed and there was no
local recurrence at two years.
Figures 7.3A and B: Orbital tuberculosis manifesting with enophthalmos: External photograph of the face showing mild enophthalmos of the
left eye (left) and a coronal computed tomography scan showing an ill-defined anterior orbital mass located inferotemporally in the left orbit,
with minimal contrast enhancement (right)
Figures 7.4A and B: Orbital tuberculosis manifesting with

enophthalmos: Histopathology of the orbital mass showing features
of non-caseating granulomatous inflammation and fibrosis
(hematoxylin and eosin, x 500) (left) and polymerase chain reaction
of the specimen positive for mycobacterium tuberculosis
deoxyribonucleic acid (right)
Discussion
Orbital tuberculosis is rare, even in endemic
countries.25 Erosion of a parenchymal pulmonary
tuberculous focus in a blood or a lymph vessel may
lead to dissemination of the organism; with systemic
involvement. 26 Orbital tuberculosis may involve
orbital soft tissues, lacrimal gland, periosteum, and
bones, and may extend to a contiguous paranasal
sinus or intracranial cavity.25, 27 The disease course is
generally slow and indolent. 25,27 Clinical manifestations include orbital tuberculoma and cold
abscess presenting with proptosis, and orbital
osteomyelitis manifesting with discharging sinus and
inflammation.25, 27 Orbital tuberculosis presents with
proptosis and is not known to present with
enophthalmos.28
Common manifestations of orbital tuberculosis
are proptosis or discharging sinus. Our patient,
however, presented with an orbital mass and
enophthalmos. Orbital tuberculosis presents with
proptosis and is not known to present with
enophthalmos.25,27 Herein we report a case of orbital
tuberculosis presenting with an orbital mass and
paradoxical enophthalmos.
On examination, the visual acuity was perception

of light with inaccurate projection and 6/9 in the right
and left eyes respectively. The right eye was severely
proptotic, with greatly increased resistance to
retropulsion. There was an upper lid wound, with
purulent drainage. The bulbar conjunctiva was
severely chemosed, making the remaining slitlamp
examination difficult (Figure 7.5A). Extraocular
movements were severely limited in all directions
of gaze. Left eye examination was unremarkable.
The patient was mildly febrile. Remaining
systemic examination was unremarkable. An orbital
Computed Tomography (CT) scan, routine blood
investigations and culture and urine examination
and culture were ordered. The blood and urine
examination reports were unremarkable, except for
mild leucocytosis. The CT scan showed a superior
orbital mass suggestive of an abscess. The paranasal
sinuses were clear of any obvious infection. Tenting
of the ocular contents was seen on axial CT,
suggestive of increased orbital pressure.
A superior orbitotomy for drainage of the abscess
was performed, under general anesthesia.
2 millilitres of pus was drained and sent for culture
and antibiotic sensitivity testing and Pulsed field Gel
Electrophoresis (PFGE), which revealed CAMRSA,
resistant to all antibiotics, except vancomycin,
cotrimoxazole (trimethoprin/sulfamethoxazole
combination) and amikacin. The patient was
admitted and started on intravenous vancomycin
1 gram every 6 hours and intravenous amikacin
1 gram daily, after consultation with our internist.
The patient started improving three days post
therapy. Intravenous dexamethasone 4 mg every 8
hours was started to decrease associated
inflammation. This was subsequently increased to
Case 3
A 55-year-old systemically normal female patient
presented to us with right-sided sudden onset severe
proptosis, pain and dimunition of vision, of 2 days
duration. Past history was significant for a boil on
the right upper eyelid, 2 days prior. The patient was
on oral amoxicillin 750 mg three times daily, at
presentation.
Figure 7.5A: CAMRSA orbital cellulitis: External photograph of patient,

showing severe right sided proptosis, with conjunctival chemosis
and draining wound of the right upper eyelid

8 mg every 8 hours, 2 days later. Serum creatinine
and urea were monitored every 72 hours. Seven days
posttherapy, the vision had improved to 6/24 and
the ocular movements had normalized almost
completely. However, lagophthalmos due to the
earlier lid wound persisted. The patient was
discharged on oral cotrimoxazole 960 mg every 12
hours and oral steroids 60 mg every day. The
antibiotic was stopped a week later and steroids
gradually tapered.
At 3 months follow-up, the vision in the right
eye was 6/9. The lid wound had completely healed
(Figure 7.5B).
Discussion
Methicillin-resistant Staphylococcus aureus (MRSA)
has been recognized as a cause of nosocomial
infections since the 1960s. Recently, MRSA infections
have been reported among patients with no history
of hospitalization. These infections have affected
prison inmates, athletic teams, military recruits,
children attending day care, and patients within no
identifiable risk group. 29,30 Community acquired
MRSA (CAMRSA) is no longer a pathogen unique to
certain high-risk populations such as prison inmates.
Most patients presenting in an outpatient setting with
an MRSA soft-tissue infection are not linked to any
distinct high-risk group.30 These CAMRSA strains
have different genetic background and antibiotic
susceptibility profiles than hospital strains. Despite
their broader antibiotic susceptibility, in comparison
to hospital-acquired strains, CAMRSA strains can
cause severe infections, such as necrotizing
pneumonias, large soft-tissue abscesses, and
necrotizing fasciitis, in otherwise healthy patients.
Figure 7.5B: CAMRSA orbital cellulitis: External photograph, 3 months

postorbitotomy, showing complete resolution of the condition and
complete healing of the lid wound
CAMRSA causing orbital cellulitis is rare, but

with an increasing incidence.31
CAMRSA is geographically widespread, as it has
been reported in many regions of the US as well as
Europe, Japan, and Australia.30, 31 Within the orbit;
this infection is extremely virulent and being resistant
to most antibiotics, causes severe damage.
We report a rapidly evolving orbital cellulitis
caused by CAMRSA. A rapidly evolving orbital
cellulitis with an abscess in an adult from any cause
should undergo prompt surgical drainage and
treatment with susceptible antibiotics. So in this
respect a MRSA orbital cellulitis is no different. The
real teaching point in this case is to include MRSA as
a significant possible cause of the cellulitis and to
start empiric therapy that includes coverage for
MRSA, until antibiotic susceptibilities come back. It
is further recommended to maintain suspicion for
MRSA in community patients with possible
staphylococcal infection, failing -lactam class
antibiotic therapy, to obtain culture and sensitivity
studies in cases of severe ophthalmic infections, and
to be informed about the rates of MRSA in the local
community.
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1. Henning Bier, and Uwe Ganzer. Involvement of the orbit
in diseases of the paranasal sinuses. Neurosurgical Review
1990;13: 109-12.
2. Ambati BK, Ambati J, Azar N. Periorbital and orbital cellulitis
before and after the advent of Haemophilus influenzae
type B vaccination. Ophthalmology 2000; 107: 1450-53.
3. Kronish JW, Jhonson TE, Gilberg SM, et al. Orbital infections
in patients with human immunodeficiency virus infection.
4. Smith AF, Spencer JF. Orbital complications resulting from
lesions of the sinuses. Ann Otol Rhinol Laryngol 1948; 57:
5-27.
5. Chandler JR, Langenbrunner DJ, Stevens ER: The
pathogenesis of orbital complications in acute sinusitis.
Laryngoscope 1970; 80: 1414-28.
6. Uzcategui N, Warman R, Smith A, Howard CW. Clinical
practice guidelines for the management of orbital cellulitis.
J Pediatr Ophthalmol Strabismus 1998;35:73-79.
7. Givner, Laurence B. Periorbital versus orbital cellulitis.
Concise Reviews Of Pediatric Infectious Diseases. Pediatric
Infectious Disease Journal 2002.21:1157-58.
8. Shovlin JP. Orbital infections and inflammations. Curr Opin
Ophthalmol. 1998; 9: 41-48.

9. Mannor G. Unusual orbital infections: Oculoplastic and
orbital surgery. Current Opinion in Ophthalmology
2000;11: 357-60.
10. Shuttleworth GN. (Clinical review)Orbital trauma.do not
blow your nose. BMJ 1999;318:1054-55.
11. Wald ER. Periorbital and Orbital Infections. Pediatrics in
Review 2004; 25: 312-20.
12. Jeyaratnam D, Reid C, Kearns A, Klein J. Community
associated MRSA: an alert to paediatricians. Arch Dis Child
2006; 91: 511-12.
13. Hendrickson RG, Olshaker J, Duckett O. Rhinocerebral
mucormycosis: a case of a rare, but deadly disease. J Emerg
Med 1999;17:641-45.
14. Ferry AP, Abedi S. Diagnosis and management of
rhinoorbitalcerebral mucormycosis. A report of 16
personally observed cases. Ophthalmology 1983;90:
1096-104.
15. Mortada A. Orbital pseudo-tumors and parasitic infections.
Bull Ophthalmol Soc Egypt 1968; 6: 393-9.
16. Mun-Wai L, Kee-Siew F, Li-Yang H, Wee-Kiak L. Optic
nerve toxoplasmosis and orbital inflammation as initial
presentation of AIDS. Graefe's Archive for Clinical and
Experimental Ophthalmology 2006;244:1542-44.
17. Luis Gorospe, Arnzazu Royo, Teresa Berrocal, GarciaRaya P, Moreno P, Abelairas J. Imaging of orbital disorders
in pediatric patients. European Radiology 2003;13:2012- 26.
18. Wendy L. Wright MS. Viral or Acute Bacterial
Rhinosinusitis? Determining the Difference. The Nurse
Practitioner: The American Journal of Primary Health Care
2005;30:30-41.
19. D B Jones and P G Steinkuller. Strategies for the initial
management of acute preseptal and orbital cellulitis. Trans
Am Ophthalmol Soc. 1988;86:94-112.
20. Urschel JD. Necrotizing soft tissue infections. Postgrad Med
J 1999;75:645-49.
21. Donahue SP, Schwartz G. Preseptal and orbital cellulitis in

childhood. A changing microbiologic spectrum.
Ophthalmology 1998; 105:1905-06.
22. Kronish JW, McLeish WM. Eyelid necrosis and periorbital
necrotizing fasciitis. Report of a case and review of the
literature. Ophthalmology 1991; 98; 92-8.
23. Luksich JA, Holds JB, Hartstein ME. Conservative
management of necrotizing fasciitis of the eyelids.
Ophthalmology 2002; 109; 2118-22.
24. Seal DV. Necrotizing fasciitis. Curr Opin Infect Dis 2001; 14:
127-132.
25. Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis
with abscess. J Neuro-Ophthalmol 2002; 22: 208-10.
26. Biswas J, Shome D. Choroidal tubercles in disseminated
tuberculosis diagnosed by the polymerase chain reaction
of aqueous humor: a case report and review of the literature.
Ocul Immunol Inflamm 2002;10: 293-98.
27. Sen DK. Tuberculosis of the orbit and lacrimal gland: a
clinical study of 14 cases. J Pediatr Ophthalmol Strabismus
1980;17:232-38.
28. Shome D, Honavar SG, Vemuganti GK, Joseph J. Orbital
tuberculosis manifesting with enophthalmos and causing
a diagnostic dilemma. Ophthal Plast Reconstr Surg 2006;
22: 219-21.
29. Weber JT. Community-associated methicillin-resistant
Staphylococcus aureus. Clin Infect Dis 2005; 41: S269 - 72.
30. Rutar T, Chambers HF, Crawford JB, Perdreau-Remington
F, Zwick OM, Karr M, Diehn JJ, Cockerham KP. Ophthalmic
manifestations of infections caused by the USA 300 clone
of community-associated methicillin-resistant Staphylococcus aureus. Ophthalmology 2006;113:1455-62.
31. Rutar T, Zwick OM, Cockerham KP, Horton JC. Bilateral
blindness from orbital cellulitis caused by communityacquired methicillin-resistant Staphylococcus aureus. Am J
Ophthalmol 2005;140:740-2.
Orbital Inflammatory Disease
CHAPTER
E Ravindra Mohan, Moupia Goswami, Vinathi Mutyala
The orbits represent a microcosm of the body in

terms of tissues present-muscle, adipose tissue, blood
vessels, nerves, skin and bone as also the eyeball
with its unique architecture, histology and spectrum
of diseases affecting it. Inflammatory conditions of
the orbit represent the commonest afflictions of the
orbits and thyroid associated orbitopathy and
infections constitute the bulk of these.
Excluding the above causes of orbital
inflammation, the causative entities range from
vasculitis, like Wegeners granulomatosis to
granulomatous conditions like sarcoidosis and the
entity of idiopathic orbital inflammation, earlier
popularly labelled as pseudotumor. As evident from
the diversitry of underlying causes, the clinical
picture, natural history, treatment and outcome of
these conditions vary greatly.
The age profile of patients with orbital
inflammation also varies greatly, ranging from the
paediatric age group for juvenile xanthogranuloma
to adulthood, mostly the 3rd-5th decades of life for
the vast majority of inflammatory conditions. Orbital
inflammatory disorders are less common in the
elderly, and must be diagnosed only after ruling out
metastatic disease and infections, by tissue diagnosis
if needed.
Orbital inflammation affects both sexes and all
races across continents.
Broadly speaking, all patients with orbital
inflammation present with one or more of the
inflammatory symptoms of pain, swelling around the
eye, proptosis, double vision or reduced vision,
redness or watering. Pain is a common symptom and
is variably described as dull, aching or throbbing

and is poorly localized, with headache being a
common complaint. Periocular swelling and
puffiness, more pronounced in the mornings after
rising from sleep is not uncommon. While double
vision is reported by some of these patients, it is a
primary complaint only in a small proportion of the
patients and at times elicited only on examination.
Orbital disease, particularly resulting from
inflammatory conditions is one of the few remaining
areas in the practice of ophthalmology, where
detailed and meticulous history taking still has an
important role and bearing on arriving at a diagnosis.
Details of symptoms, associated systemic features,
response to medications and side effects of treatment
are important areas which need to be probed
carefully. For example, merely knowing that a
patients orbital symptoms improved on oral steroids
may be inadequate to arrive at a diagnosis of
idiopathic orbital inflammation. If the details are
sought, and suggest rapid relief starting within
hours, and near total resolution, one would be more
definite in making the diagnosis.
Examination of a patient with orbital
inflammatory disease needs to be done with
meticulous attention to detail. Careful documentation
including photographic documentation is invaluable
in studying the natural history of an individual
patients condition as also in prognostication and in
titrating medical therapy.
Close co-ordination with other specialists
treating a patient is clearly essential in the
management of patients with orbital inflammatory
Orbital Inflammatory Disease 129

conditions. In addition to primary care givers, like
rheumatologists in managing a patient with a
vasculitic condition, other specialists often need to
be involved in care as the disease progresses or
complications develop as a result of medical therapy,
as in the need for orthopaedic care for aseptic
necrosis of neck of femur developing from prolonged
systemic steroid therapy.
Overall, the disease entities covered in this
chapter are diverse and may have little in common
except the propensity to produce orbital
inflammation. Achieving a precise diagnosis, by
obtaining orbital tissue for diagnosis if needed, is
vital. With present day techniques and
instrumentation, obtaining an adequate specimen for
histopathological and immunohistochemical
diagnosis is safe. Except for cases where the disease
is localized to a relatively inaccessible region of the
orbit like the orbital apex, or resolves fully on medical
therapy based on presumptive diagnosis, an incisional
biopsy is essential to achieve diagnosis and rule out
other causes for orbital inflammation like
masquerade syndrome related to malignant tumor.
A fine needle aspiration biopsy is often inadequate
for the purpose.
The treatment of orbital inflammations remains
centred on the control of inflammation and
prevention of the sequelae of persistent and
prolonged inflammatory reaction. A host of
immunosuppressive drugs like cyclosporine,
methotrexate and cyclophosphamide are used in
addition to intravenous methylprednisolone and the
widely used oral steroid medications.
Surgery in patients with orbital inflammatory
disease is primarily to obtain tissue diagnosis.
Surgical debulking of the involved orbital tissue is
rarely needed, and the use of destructive operations
like orbital exenteration is only in extremely
disfigured orbits with no visual potential and
severely troubling symptoms. Radiation therapy has
limited role.
Treatment of orbital inflammatory disease needs
to be tailored and titrated to the individual disease
entity and patient. Since inflammation often tends
to relapse and recur, long term follow-up is needed
as also monitoring to assess for complications of
orbital disease like ocular motility restriction or
complications of therapy, like steroid induced cataract.
The prognosis for patients with orbital

inflammatory disease is variable and depends on the
underlying disease and its severity.
ORBITAL AMYLOIDOSIS
Amyloidosis refers to a heterogenous group of
disorders of protein metabolism characterized by the
extracellular deposition of abnormal insoluble protein
fibrils. Deposition of amyloid in the eye and its
adnexal structures may occur as part of systemic
amyloidosis or as a localised form. Local orbital
amyloidosis is a rare condition, comprising only 4%
of cases of local amyloidosis seen in the head and
neck regions.1
Ocular findings in primary generalised
amyloidosis include purpura of the eyelids, which
can frequently be the presenting sign; bilateral,
symmetrical, small amyloid papules of the skin of
the eyelids, nodules in the lids, ptosis; proptosis,
globe displacement with or without visual
impairment, ophthalmoplegias or amyloid
neuropathy affecting pupillary function or both and
subconjunctival hemorrhages.
Histologically the specimen shows fibrous
connective tissue and massive amyloid deposit
infiltrated with lymphocytes, plasma cells, and
foreign body giant cells. Amyloid deposits are
identified histologically by congo red staining
(Figure 8.1) and viewing under polarized light where
amyloid deposits produce a distinctive apple green
birefringence. The pathogenic mechanisms leading
to local tissue deposition of amyloid are not clear.
The universal constituent of amyloid is the amyloid
P component (AP). It is derived from normal
Figure 8.1: Congo red staining showing amyloid (Congo red x 200)

circulating plasma protein, serum amyloid P
component (SAP). The isolated pure human SAP
radiolabelled with I 123 is a highly specific tracer for
all types of amyloidosis.2
The best method of orbital imaging is CT scan,
for its ability to detect calcification which
differentiates it from other lesions. Radionuclide SAP
scans help in anatomical localisation of amyloid
deposits.
Management of orbital amyloidosis is difficult.
Standard treatment aims to reduce reproduction of
the monoclonal immunoglobulin precursor via
chemotherapy or radiotherapy or surgery of the
localized lesion. Total excision is usually difficult and
surgery is aimed at debulking the mass with
preservation of palpebral gland of the lacrimal gland,
levator and rectii muscles.
It is mainly a diagnosis of exclusion based on

clinical picture, laboratory tests, biopsy and
radiologic evidence. Up to 90 percent of patients with
ocular sarcoid have abnormal chest radiographs.
Hilar lymphadenopathy is seen (Figures 8.2A and
B). Lung biopsy by tracheobronchial fiber optic
techniques is 90 percent accurate. Biopsy of an
enlarged, potentially infiltrated lacrimal gland or
conjunctival granuloma is an acceptable alternative.
REFERENCES
1. Gean-Marton AD, Kirsh CFE, Vezina LG, et al. Focal
amyloidosis of the head and neck: evaluation with CT and
MR imaging. Radiology 1991;181:521-5.
2. Murdoch IE,Sullivan TJ, Moseley I, Hawkins PN, Pepys
MB, Tan SY, Gamer A, Wright JE. Primary localised
amyloidosis of the orbit. Br J Ophthalmol 1996;80:1083-6.
SARCOIDOSIS
Sarcoid (from the Greek sark and oid meaning
flesh-like) or Besnier-Boeck disease or Schaumanns
syndrome.
Sarcoidosis is an idiopathic chronic non
necrotizing granulomatous multi-systemic disease
that affects skin, brain, eyes, lungs, spleen, thyroid,
and liver. It commonly affects young adults, who
frequently present with hilar lymphadeno
pathy, pulmonary infiltration, ocular and cutaneous
lesions.
Ocular involvement manifests in 25-60% of
patients with systemic sarcoidosis. 1 The most
common ocular manifestation in sarcoidosis is uveitis
and the most common orbital manifestation is
dacryoadenitis, which is frequently bilateral.2 Other
manifestations include eyelid swelling and palpable
eyelid masses, conjunctival nodules, retrobulbar
masses, proptosis, optic nerve, chiasma or sheath
involvement, optic radiation infiltration, bone
destruction and rarely extraocular muscle
involvement.
B
Figures 8.2A and B: CT chest showing hilar
lymphadenopathy in sarcoidosis

Typical histologic findings from biopsy include
accumulation of T lymphocytes and mononuclear
phagocytes, diffuse non-caseating epitheloid
granulomas and derangements of the normal tissue
architecture. Infectious etiologies need to be excluded
by culture and/or staining.
Corticosteroids are the mainstay of treatment in
symptomatic cases. Severe symptoms are generally
treated with steroids. In cases of steroid intolerance
or resistance, steroid sparing agents such as
azathioprine and methotrexate are often used.
Cyclophosphamide has also been used. As the
granulomas are caused by collections of immune
system cells, particularly T cells, there has been some
early indication of success using immunosuppressants, interleukin 2 inhibitors or anti tumor
necrosis factor-alpha treatment (such as infliximab).
Lofgrens syndrome represents an acute
presentation with erythema nodosum, bilateral hilar
lymphadenopathy and polyarthralgia. This entity has
a relatively good prognosis.
The combination of anterior uveitis, parotitis and
fever is called uveoparotid fever and in association
with cranial nerve palsies is referred to as HeerfordtWaldenstrom syndrome.
REFERENCES
1. Hunter DG, Foster CS. Ocular manifestations of sarcoidosis.
In: Albert DM, Jakobiec FA, eds. Principles and practice of
ophthalmology. Philadelphia: WB Saunders, 1994; 443-50.
2. Jakobiec F, Bilyk JR, Font RL Non infectious orbital
inflammations. In: Spencer WH, editor. Ophthalmic
pathology - An Atlas and Textbook. WB Saunders,
Philadelphia, 1996; 2810-58.
NONSPECIFIC ORBITAL
INFLAMMATORY SYNDROME (NSOIS)
Nonspecific orbital inflammatory syndrome (NSOIS),
commonly referred to as Idiopathic Orbital
Inflammation, Orbital Pseudotumor is defined as a
benign, non infective clinical syndrome characterized
by features of nonspecific inflammatory conditions
of the orbit without identifiable local or systemic
causes. Idiopathic orbital inflammation is the third
most common non infectious orbital disease,
following Gravess orbitopathy and lymphoproliferative diseases. It accounts for 4.7 to 6.3% of
orbital disorders.1
Idiopathic orbital inflammation has highly

variable clinical features, from a diffuse to very focal
process targeting specific orbital tissues, such as the
lacrimal gland, extraocular muscles, optic nerve and
orbital fat. Presentations vary according to the
specific location and the degree of inflammation,
fibrosis, and mass effect. Ptosis, chemosis, motility
dysfunction, and optic neuropathy may also be
found. Entrapment, compression, and destruction of
orbital tissues may occur in patients with extensive
sclerosis. Unilateral presentation is typical, but
bilateral presentations are not uncommon.
Radiological imaging studies allow tissue
characterization and localization without surgical
intervention and thereby have become invaluable
diagnostic tools. Computed tomography is the
preferred mode of imaging. Idiopathic orbital
inflammation is typically seen on CT scans as a focal
or diffuse mass, usually poorly demarcated and
enhancing with contrast. Common CT findings include
enhancement with contrast medium, infiltration of
retrobulbar fat, proptosis, extraocular muscle
enlargement, muscle tendon or sheath enlargement,
apical fat edema, optic nerve thickening, uveal-scleral
thickening, edema of the Tenon capsule, and lacrimal
gland infiltration. Tendons of the extraocular muscles
may be involved or spared.
The histopathological spectrum of idiopathic
orbital inflammation is typically non diagnostic, wide,
and diverse, ranging from the typical diffuse
polymorphous infiltrate to the atypical granulomatous inflammation, tissue eosinophilia, and
infiltrative sclerosis. In the absence of systemic fibro
inflammatory, granulomatous, and vasculitic disease,
these atypical presentations are considered to be
subclasses of idiopathic orbital inflammation.2
NSOIS respond rapidly to high dose steroid
therapy in tapering doses but recurrences are
common. In such cases, chemotherapy (e.g.
methotrexate, cyclosporine) and low-dose radiation
(e.g. 1500-2500 cGy EBRT) may be needed to control
the inflammation.3
REFERENCES
1. Henderson JW: Orbital tumors, Newyork, Ravin press,
1994, (3rd ed), pp 13-14; 47;317-411.
2. Root man J: The classification and management of acute
orbital pseudotumors: Ophthalmology 1982, 89;1040-48.
3. Leone C: Treatment protocol for orbital inflammatory
diseases; Ophthalmology 1985, 92; 1325-31.
KIMURAS DISEASE
Kimuras disease is a chronic inflammatory disorder
of uncertain etiology which typically presents as
multiple cutaneous nodules in the head and neck
region particularly the preauricular regions. It was
first described in China in 1937 by Kim and Szeto as
eosinophilic lymphogranuloma.1
Kimuras disease is most commonly seen in
patients between 20-40 years of age with a striking
male predominance and is endemic among the
oriental population. It is characterized by a triad of
insidious onset of painless subcutaneous nodules in
the head and neck region, blood and tissue
eosinophilia and markedly elevated serum
immunoglobulin levels.2 In the orbit, presentation is
in the form of proptosis, upper lid swelling and
orbital masses usually in the lacrimal gland.
The histomorphology of Kimuras nodule is
characterized by intense infiltration of lymphocytes,
vascular proliferation and plasma cells with a variable
number of lymphoid follicles with germinal centers.
Typically, there is a moderate to intense eosinophilic
infiltration mainly in a perivascular pattern.
Immuohistochemical stains would typically show IgE
reticular network in the germinal centers.3
Following initial presentation, surgical excision
and biopsy with debulking is the preferred mode of
treatment, but recurrence is common. Other
treatment options include radiation, systemic
corticosteroids, cyclosporine and pentoxyfylline.
REFERENCES
1. Kim HT, Szeto C. Eosinophilic hyperplastic
lymphogranuloma, comparison with Mikuliczs disease.
Chin med J. 1937 23:699-700.
2. Hui PK, Chan JK, Ng CS, Kung IT, Gwi E.
Lymphadenopathy of Kimuras disease. Am J Surg Pathol.
1989; 13:177-86.
3. Motoi M, Wahid S, Horie Y, Akagi T. Kimuras disease:
clinical, histological, and immunohistochemical studies.
Acta Med Okayama.1992;46:449-55.
WEGENERS GRANULOMATOSIS
Wegeners granulomatosis (WG) is a fulminant
systemic disease of unknown aetiology consisting of
necrotizing granulomatous vasculitis of the upper and
lower respiratory tracts, focal necrotizing

glomerulonephritis, and systemic small vessel
vasculitis involving multiple organ systems.
Incidence of ocular involvement in WG varies
from 29 to 79%.1, 2 Ocular involvement can be either
due to an extension from the adjacent paranasal
sinuses (contiguous) or as a result of focal vasculitis
(noncontiguous).1,2 Presentation can be in the form
of proptosis, dacryocystitis, scleritis with peripheral
keratopathy, kerato-conjunctivitis sicca, uveitis,
retinitis, retinal vascular occlusions, exudative retinal
detachments, and optic neuritis.
Laboratory findings support or confirm the
diagnosis of WG. In the active stage of the disease
raised ESR and leucocytosis are seen. Chest X-ray
and computerised tomography detect pulmonary
involvement. Routine urine analysis detects renal
involvement. Serum antibodies against the
cytoplasmic component of neutrophils and monocytes
(cANCA) form a useful adjunct in the diagnosis of
WG. Indirect immunofluorescence is currently the
standard test for ANCA screening.3 Between 80%
and 95% of all ANCA found in WG is cANCA.
Diagnosis is established by biopsy in orbital and
paranasal sinus involvement. The characteristic
histopathologic picture is that of necrotising vasculitis
of the blood vessels, usually with granuloma
formation in the surrounding infiltrates.
Management of WG requires a multisystem
approach. Oral corticosteroids along with a cytotoxic
agent, of which cyclophosphamide is the most
efficacious, is the treatment of choice. Early treatment
with cyclophosphamide and corticosteroids reduces
both ocular and systemic morbidity.
Exacerbations are common in the first two years
after diagnosis. In cases of remission, azathioprine,
cyclosporine A and methotrexate may be used.
REFERENCES
1. Straatsma BR. Ocular manifestations of Wegeners
granulomatosis. Am J Ophthalmol 1957; 144:789-99.
2. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular
complications of Wegeners granulomatosis.
Ophthalmology 1983;90:279-90
3. Harman LE, Margo CE. Wegeners granulomatosis. Survey
of Ophthalmology 1998; 42:458-80.
LANGERHANS HISTIOCYTOSIS
Langerhans cell histiocytosis (LCH) is a group of
idiopathic disorders characterized by the proliferation
of specialized bone marrowderived Langerhans cells
(LCs) and mature eosinophils. These can be
subdivided into three clinico-pathological entities:
acute disseminated LCH, unifocal and multifocal
unisystem LCH, and multisystem LCH. There are
three forms of presentation.
Eosinophilic Granuloma: single organ involvement.
Hand-Schuller-Christian Syndrome: lytic bone
lesions, diabetes insipidus and exophthalmos.
Letterer-Siwe disease: severest form of the disease
found in infants, involving lesions in the liver, bone
marrow, spleen and skin.
Langerhans cell histiocytosis (LCH) accounts for
less than 1% of all orbital tumours. Orbital
involvement in LCH is characterised by osteolytic
lesions with sclerotic margins along with soft tissue
involvement.
The typical cytopathological picture consists of
Langerhans cells along with eosinophils and a
varying number of neutrophils, lymphocytes,
macrophages and multinucleated giant cells with pale
ill-defined eosinophilic cytoplasm and lobulated
nuclei with longitudinal grooves, best visualized in
Papanicolaou-stained smears.1 A definitive diagnosis
is made by presence of Birbeck granules on electron
microscopy (rod-like structures with a striated core
having dilated ends giving a tennis racket
appearance) or positivity for CDI antigen
determinants on cryostat sections. In an appropriate
clinicoradiological setting, a typical pathology alone
can be used for effective diagnosis and definite proof
of LCH.2
Management modalities vary from observation,
curettage, intralesional steroids, low-dose radiation,
high-dose systemic corticosteroids and chemotherapy, bone marrow transplantation and antibody
therapy for recalcitrant cases. The most effective
treatment is chemotherapy with Vincristine,
Vinblastine, Etoposide and steroids. Low dose
radiation in 4-6 fractions may be used when the
disease is extensive, inaccessible or if it threatens an
important organ.3
REFERENCES
1. Ayala AG, RO-JY, Famming CV, Flores JP, Yaskee AW.
Core needle biopsy and fine needle aspiration in diagnosis
of bone and soft tissue lesions. Hematol Oncol Clin North
Am Jun 1995; 9:633-51.
2. Pohar-Marinsek Z, Us-Krasovec M. Cytology of
Langrehans cell histiocytosis. Acta Cytol 1996; 40:1257-64.
3. Sessa S, Sommelet D, Lascombes P, Prevol J. Treatment of
Langerhans cell histiocytosis in children - Experience at the
childrens Hospital of Nancy. J Bone Joint Surgery-Am 1994;
76:1513-25.
ROSAI-DORFMAN DISEASE
Synonyms: Sinus Histiocytosis with Massive
Lymphadenopathy, Destombes Rosai-Dorfman
disease.
Sinus Histiocytosis with Massive Lymphadenopathy (SHML) otherwise known as Rosai
Dorfman Disease, (RDD), is a rare, benign systemic,
idiopathic reactive proliferation of distinctive
histiocytes, characterised by massive lymphadenopathy, particularly in the head and neck region,
and often associated with extra nodal involvement.
The orbit is a common extranodal site of RDD.1
Widespread dissemination with liver, kidney,
respiratory organs, orbit, and eyeball involvement
has been reported rarely. Lymphoproliferation in the
soft tissues of the orbit and in the lids has been
reported in 12% of cases but intraocular involvement
is rare.
The histologic hallmark of sinus histiocytosis with
massive lymphadenopathy (SHML) are large
intrasinusoidal cells exhibiting cytophagocytosis.
Microscopic examination of the lymph nodes
shows a polymorphous infiltrate composed of plasma
cells, neutrophils, lymphocytes, and histiocytes. The
histiocytes often contain phagocytised lymphocytes,
a histological finding termed emperipolesis. An
immunohistochemical staining panel that includes
CD31 and S100 facilitates the diagnosis of SHML.2
Most effective regimen is a vinca alkaloid
combined with an alkylating agent and a
corticosteroid. The most commonly used regimen is
a combination of cyclophosphamide, vincristine,
mercaptopurine, and prednisolone. Treatment causes
regression of the tumor and resolution of

lymphadenopathy with minimal recurrence. Surgery
is indicated rarely, in life or function threatening
situations.
REFERENCES
1. Friendly DS, Font RL, Rao NA. Orbital involvement in
sinus histiocytosis. Arch Ophthalmol 1977;95:200611.
2. Slone SP, Fleming DR, Buchino JJ. Arch Pathol Lab Med
2003 Mar;127(3):341-4.
ORBITAL XANTHOGRANULOMA
Adult xanthogranulomatous diseases are nonLangerhans histiocytic disorders (type II) involving
the ocular or orbital tissues and constitute a group
of entities with varying manifestations. They are
adult onset xanthogranuloma (AOX), which is
isolated with no systemic associations, adult onset
asthma with periocular xanthogranuloma (AAPOX),
necrobiotic xanthogranuloma (NBX) and ErdheimChester disease (ECD). Juvenile xanthogranulomas
usually present with skin or intraocular lesions and
orbital involvement which is rare, occurs almost
exclusively in children.1
Ocular involvement maybe in the form of eyelid
or orbital mass, proptosis, orbital bone mass and
extraocular muscle involvement,epibulbar mass,
uveal mass, uveitis and rarely retinal and choroidal
involvement. While the orbit or adnexal
xanthogranuloma tends to be anterior in AOX,
AAPOX, and NBX, it is often diffuse in ECD and
leads to visual loss.
Diagnosis is confirmed by biopsy. The
characteristic appearance of xanthogranulomas on
histopathology is proliferation of histiocytes, plasma
cells and lymphocytes with Touton giant cells that
stain positive for lipid. Touton giant cells are
multinucleate cells with the nuclei arranged in a
wreath around a nidus of eosinophilic cytoplasm and
separated from the cell membrane by a rim of
translucent foamy cytoplasm.2 Necrosis (necrobiosis)
with pallisading epitheliod histiocytes is mostly seen
in NBX whereas large lymphoid aggregates with
germinal centers are often found in cases of AAPOX.
ECD exhibits florid fibrosis with fewer follicles and
more dispersed lymphocytes and lipid laden
histiocytes.
The clinical course is chronic and often

progressive. Various treatment modalities include
local excision, periocular and systemic steroids,
chemotherapy with low dose chlorambucil, nitrogen
mustard, cyclophosphamide, melphalan, local
radiation and plasma exchange.
REFERENCES
1. Zelger B, Cerio R, Orchard G, et al. Juvenile and adult
xanthogranuloma. A histological and immunohistochemical comparison. Am J Surg Pathol 1994;18:12635.
2. Murthy R, Honavar SG, Vemuganti GK, Naik M, Burman
S. Isolated giant xanthogranuloma of the orbit. Indian J
Ophthalmol [serial online] 2007 [cited 2007 Jul 22]; 55:
156-58.
CASE ILLUSTRATIONS
Case 1
An 18-year-old female, presented with recurrent
swelling of the right upper lid of 4 months
duration.There was no history of pain, redness,
diplopia or defective vision. A cystic mass was excised
elsewhere 8 months back, histopathology reports
were not available.
On examination, best corrected visual acuity in
both eyes was 6/6,N6. Ocular movements were full
and painless.There was fullness of the upper temporal
part of the right orbit. A firm to hard cord like mass
was felt preseptally. The mass was adherent to the
bone at the lateral canthus. (Figure 8.3) There was
no lymphadenopathy.
CT scan (Figure 8.4) showed enlarged mass, not
separate from lacrimal gland, surrounding the globe.
It was heterogenous in density.
Figure 8.3: Showing fullness of upper temporal

part of the right orbit in a patient with sarcoidosis

Blood tests for serum calcium, phosphorus,
proteins, angiotensin converting enzyme and serum
lysozyme were within normal limits.
No systemic medications were started in view
of normal blood investigations.
The patient was asymptomatic and there was no
recurrence during the postoperative follow up of two
years.
Case 2
Figure 8.4: CT scan showing anteriorly located heterogenous

mass in the upper temporal quadrant in patient with sarcoidosis
Total excision of the mass was performed

through anterior orbitotomy.Intraoperatively the
mass was found to be arising from the palpebral part
of the lacrimal gland.
Histopathological examination revealed chronic
granulomatous inflammation with no evidence of
caseation. Large amounts of granuloma with
epitheloid cells and giant cells were observed,
suggestive of sarcoidosis (Figure 8.5).
Special stain for fungus and acid fast bacilli were
negative.
Figure 8.5: Photomicrograph showing non-caeseating

granulomas (Hematoxylin Eosin x 200)
A male farmer, 45 years of age, presented with

complaints of protrusion of right eyeball for the past
2 months. His general health was good. Previous
thyroid profile was normal.
Examination revealed best corrected visual acuity
of 6/6p, N6 and 6/5, N6 in the right and left eyes
respectively. There was periocular fullness and
inferior scleral show of 2 mm.The globe was pushed
forwards by 9 mm,outwards by 3 mm and upwards
by 2 mm. Ocular motility was normal. There was no
relative afferent papillary defect.There was increased
resistance to retropulsion (Figure 8.6). A firm mass
was palpable in the inferomedial orbit, posterior
extent of which could not be felt.
Slit lamp examination of the anterior segment and
intraocular pressure were within normal
limits.Fundus examination revealed disc edema with
no choroidal folds. CT scan revealed an illdefined
heterogenous mass lesion in the right inferomedial
orbit extending upto the apex.Medial rectus and
inferior rectus were included in the mass lesion
(Figures 8.7 and 8.8). We did a right orbital biopsy
through a subciliary incision, with debulking of the
tumor under general anesthesia. Intraoperatively an
infiltrating grey white firm mass was seen, which
Figure 8.6: Photograph showing periocular fullness, proptosis and

scleral show in a patient with nonspecific inflammation of right orbit

followed by 400 mg BD for one month. There was
significant clinical improvement postoperatively with
vision improving to 6/6, and complete resolution of
proptosis.
Case 3
Figure 8.7: Axial CT scan showing illdefined heterogenous mass

lesion extending upto apex in a patient with nonspecific inflammation
A young female aged 15 years, presented with small

swelling in the upper outer quadrant of the left eye
since 2 years. The swelling was progressively
increasing in size. There was no pain, visual
disturbance or diplopia.
On examination, fullness of superotemporal
region with displacement of the globe downwards
and medially was seen (Figure 8.9). A soft to firm
swelling with illdefined margins was palpable. It was
compressible, non reducible, non pulsatile and non
tender. There was increased resistance to
retropulsion. There was no lymphadenopathy.
CT scan revealed well defined, heterogeneously
dense, non enhancing enlargement of lacrimal gland.
(Figures 8.10A and B). We excised the mass in toto
through lateral orbitotomy. Histopathology
examination revealed focal collection of chronic
inflammatory cells with abundant fibrocollagenous
tissue suggestive of nonspecific inflammation of
lacrimal gland (Figure 8.11). Postoperatively, patient
was symptom free on follow up of 5 years.
Case 4
Figure 8.8: Coronal CT scan - Medial rectus and Inferior rectus

muscles involvement in the nonspecific inflammation of orbit
A 32-year-old female presented with complaints of

protrusion, pain and redness in the right eye
associated with progressive dimunition in vision since
1 year.
On examination, the best corrected visual acuity
in right eye was 2/60, N36. Marked lid edema and
was difficult to cut and hence was removed

piecemeal. Histopathological examination of the
specimen was consistent with a diagnosis of non
specific inflammation with extensive fibrocollagenous
tissue. In view of this finding,ultrasound abdomen
was done to rule out retroperitoneal fibrosis. ANCA
test was advised to rule out Wegeners granulomatosis. Both test reports were found to be within
normal limits. Patient was started on tapering dose
of oral steroids, starting with prednisolone
50 mg/day, reducing it by 10 mg every 3 days and
tab pentoxyfylline 400 mg TDS for one month
Figure 8.9: Photograph showing fullness of superotemporal region

with downward displacement of the right globe in a patient with
nonspecific inflammation of lacrimal gland
Figures 8.10A and B: Axial (A) and Coronal (B) sections of CT scan showing well defined, heterogeneously dense nonenhancing
enlargement of lacrimal gland in a patient with nonspecific inflammation
Figure 8.11: Photomicrograph showing specimen of lacrimal gland

with chronic inflammatory cells surrounding it, suggestive of
inflammation of lacrimal gland (Hematoxylin Eosin x 100)
conjunctival congestion was seen, globe was

displaced forwards and downwards. Elevation and
abduction of the globe was restricted. A palpable
mass was felt in the superotemporal quadrant.
Preauricular and submandibular lymphadenopathy
was present on the same side. Ultrasound abdomen
was normal, FNAC showed reactive changes with
mild eosinophilia. CT scan showed large extraconal
orbital mass surrounding the globe all round,
infiltrating the periocular structures (Figure 8.12). We
Figure 8.12: CT scan showing large extraconal orbital mass

surrounding the globe and infiltrating the periocular structures in a
patient with Kimura's disease
performed a lateral orbitotomy. Intraoperatively, the

mass was firm in consistency and was extensively
infiltrating the periorbital, lateral orbital and the
lacrimal gland region extending upto the apex.
Histopathological report confirmed the diagnosis
of angiolymphoid hyperplasia with eosinophilia

(Kimuras disease) (Figure 8.13). A course of systemic
steroids was given. Visual acuity improved to 6/6,
N6 in the affected eye. She came back 4 months later
with severe pain and headache, with massive
proptosis and keratinization of conjunctiva and
cornea. Right eye had no perception of light. Patient
was referred to oncologist for chemotherapy and
radiotherapy. The response was not satisfactory. In
view of the above, exenteration of the right orbit
was done. (Figure 8.14) Patient was fitted with a
spectacle mounted prosthesis. Recurrence was seen
the form of tiny nodular subcutaneous lesions. CT
scan evidence of orbital recurrence was seen in the
form of soft tissue filling orbit (Figure 8.15). As the
lesion was non malignant, it was decided to watch
the lesion for 6 months. Patient thereafter was lost
Figure 8.15: Photomicrograph showing blood vessels with scattered

eosinophils and lymphocytes in a patient with Kimura's disease
(Hematoxylin Eosin x 200)
to follow up. This case shows that some of the benign

orbital inflammations may be very aggressive and
severe disease may even necessitate orbital
exenteration.
Case 5
Figure 8.13: CT scan post exenteration of the

patient with Kimura's disease
A male aged 41 years, presented with recurrent left

cheek swelling with protrusion of left eye since 5
months. He had undergone partial maxillectomy
surgery of the left side 1 year back.
On examination, his best corrected vision was
6/9, N6 in both the eyes.Left proptosis with upward
displacement of the globe was seen. A firm to hard
swelling was palpable in the left inferior orbit and
cheek area. Elevation and depression of the left eye
was restricted. (Figure 8.16 ).No afferent papillary
defect was present. CT scan revealed soft tissue
Figure 8.14: Appearance following orbital exenteration

for severe Kimura's disease
Figure 8.16: Photograph showing left proptosis, upward displacement

of globe and fullness in periocular area in a patient with Wegener's
Granulomatosis

lesion in the left maxillary sinus with extraconal
extension into the orbit (Figures 8.17A and B). The
CT scans showed evidence of partial resolution of
findings following treatment with oral steroids
Histopathological examination showed deposits

of fibrocollagenous tissue with areas of necrosis with
vessel obstruction and dense collection of chronic
inflammatory cells (Figure 8.19). A diagnosis of
Wegeners granulomatosis was kept in mind. Chest
B
Figures 8.17A and B: CT scan pre-treatment - showing soft tissue lesion due to Wegener's
Granulomatosis in the left maxillary sinus extending to left orbit
B
Figures 8.18A and B: CT scan, post-treatment with systemic steroids in a patient with
Wegener's Granulomatosis, showing partial resolution of the lesion
Figure 8.19: Photomicrograph showing diffuse inflammation with granulomatous reaction (giant cell formation).
Arrow shows healed vasculitis. This is suggestive of Wegener's Granulomatosis (Hematoxylin Eosin x 100)

X-ray was normal. p ANCA and c ANCA were
borderline.Patient was evaluated by rheumatologist
and based on clinical diagnosis of Wegeners
granulomatosis, was started on oral steroids. Patient
had resolution of symptoms and decrease in swelling
following treatment.
Case 6
A male child aged 4 years was referred with CT scan
of orbit as a case of malignant lacrimal gland tumor.
He had painful swelling of right upper lid of 2 months
duration and raised ESR. On examination, his best
corrected vision was 6/18 in the right eye. Tender
swelling of the right upper lid, more on lateral aspect

was noticed (Figure 8.20). There was no proptosis.
Ocular motility was normal.Rest of anterior segment
and fundus examination was normal. CT scan
(Figures 8.21A and B) revealed an irregular
heterodense mass in the lacrimal gland region,
associated with lysis of lateral wall and temporal part
of roof of orbit. With the presumptive diagnosis of
Langerhan cell histiocytosis, he underwent a
complete evaluation including bone marrow which
was normal. Chest X-ray revealed interstitial
pneumonia. An anterior orbitotomy revealed
yellowish black material. Impression cytology and
permanent sections confirmed the diagnosis of
Langerhans cell histiocytosis. On pediatricians
advise, patient was started on oral prednisolone and
6-Mercaptopurine. Patient has been free of symptoms
on a followup of 3 years.
Case 7
Figure 8.20: Photograph showing swelling of the lateral part of

right upper eyelid in a patient with Langerhans cell histiocytosis
A male child aged 2 years presented with

gradual onset of swelling in the right upper lid since
3 months.There was sudden increase in swelling in
the last 2 days. On examination, a well circumscribed,
non tender, firm lesion was palpable in the superior
orbit.Posterior limit could not be felt (Figure 8.22).
CT scan showed heterogenous soft tissue in the
B
Figures 8.21A and B: CT scan shows an irregular heterodense mass in the lacrimal gland area associated with
lysis of lateral wall and temporal part of roof of orbit in a patient with Langerhans cell histiocytosis
Figure 8.22: Photograph showing swelling of the right upper eyelid in another patient with Langerhans cell histiocytosis
Figures 8.23A and B: CT scan showing heterogenous soft tissue in the anterior superior aspect of the right orbit
(predominantly preseptal) with lytic lesion in the temporal bone in a patient with Langerhans cell histiocytosis
anterior superior aspect of the right orbit

(predominantly preseptal) with lytic lesions in the
temporal bone (Figures 8.23A and B). Anterior
orbitotomy with excision of mass was done. It was
cream to black in colour and soft in consistency.
Histopathological examination showed large
collection of histiocytes, in addition to lymphocytes,
eosinophils, plasma cells and few multinucleated
giant cells (Figure 8.24). He was treated with
intravenous steriods followed by oral steroids for
one month. There was complete resolution of mass
following this. He was asymptomatic during two
years of follow-up.
Figure 8.24: Photomicrograph showing numerous eosinophils and
histiocytes suggestive of Langerhans cell histiocytosis
(x100,Hematoxylin Eosin)
Case 8
A 31-year-old female, presented with recurrent
episodes of swelling and prominence of left eye since
8 months.She had been on steroids off and on. On
examination, fullness of left side of face was seen.
There was 4mm left axial proptosis. Firm irregular
mass was palpable in the left inferior orbit.There was
no lymphadenopathy (Figure 8.25). MRI scan showed
a huge fusiform retrobulbar mass (Figures 8.26A to
D). Histopathological and immunochemistry study
of the biopsy specimen revealed features consistent
Figure 8.25: Photographs showing left proptosis, upward globe

displacement and fullness of the left side of the face in a patient with
Rosai-Dorfman disease
Figures 8.26A to D: MRI scans of the patient with Rosai-Dorfman disease shows illdefined extra and intraconal heterogenous mass lesion,
isointense in T1 and hypointense in T2 weighted images with homogenous contrast enhancement. Inferior and lateral rectus muscles are
thickened and displaced
Figure 8.27: Photomicrograph showing histiocytes with lymphocytes

and plasma cells in Rosai-Dorfman disease ( x 40, Hematoxylin Eosin)
Figure 8.28: Photograph showing downward displacement of right

eyeball, with proptosis in a patient with Juvenile Xanthogranuloma
with Rosai Dorfman syndrome (Figure 8.27). Patient

was seen by oncologist who advised radiation to the
orbit. Patient refused treatment and was lost to
follow-up. She came back one and a half years later
with double vision and massive proptosis. There was
increased retrobulbar resistance. Disc edema was seen
on fundus evaluation. In view of the huge size of the
lesion and the high dose of radiation that would be
required with its potential complications, it was
decided to perform a surgical debulking.
Intraoperatively, a firm grey tumor was seen
infiltrating the inferior orbit. Inferior rectus was
densely adherent to it. Patient was advised tapering
dose of oral steroids. Patient was free of symptoms
on 2 years of follow-up.
Case 9
A male child, age 1 year, presented with prominence
of right eye since last 20 days. There was no pain,
redness, fever or weight loss. On examination, there
was downward displacement of right eyeball with 4
mm proptosis (Figure 8.28). Resistance to retropulsion was felt. Restriction of elevation and
abduction was seen. Fundus examination revealed
mild disc edema with dilated tortuous retinal veins.
CT scan revealed a large slightly hyperdense
retrobulbar mass with excavation of medial wall of
orbit (Figure 8.29). The mass was removed piecemeal
by anterior orbitotomy. Histopathological examination showed features of juvenile xanthogranuloma
(Figure 8.30 ). He was started on tapering dose of
Figure 8.29: CT scan showing a large slightly hyperdense retrobulbar

mass with excavation of medial wall in the patient with Juvenile
Xanthogranuloma
oral steroids. On 6 months followup, complete

resolution of proptosis was seen. He was
asymptomatic at follow-up of 3 years.
Case 10
A young female of age 35 years, presented with
painless progressive protrusion of both eyes since
five years. She had occasional double vision. She
had been treated with oral steroids off and on with
dramatic improvement in symptoms, and recurrence
on stopping the same. She had received anti

tuberculosis therapy earlier. She also reported
dryness of the mouth. On examination, her best
corrected visual acuity was 6/36, N36 and 6/24, N6
in the right and left eye respectively.Axial proptosis
was noted with firm palpable orbital masses in both
orbits, most prominent in the superonasal
orbit.Ocular movements were restricted in all
gazes.There was no lagophthalmos (Figure 8.31).
Slit lamp examination revealed reduced tear
meniscus, superficial punctuate keratopathy and
posterior subcapsular cataract in both the eyes.
Fundus examination revealed striae in the right eye
and choroidal folds in the left eye. Bilateral parotid
enlargement was noted on both sides. Thus the
possibility of Sjogrens syndrome with underlying

autoimmune disease was considered. CT scan of the
orbit revealed bilateral diffuse illdefined extra and
intraconal soft tissue with clumps of calcification
within (Figures 8.32A and B). Transeptal orbital
biopsy was done under general anesthesia. Histopathological examination suggested xanthogranulomatous inflammation. Medical oncology and
dermatology opinion was sought. In view of side
effect of long term steroids, patient was started on
oral anti-inflammatory drugs and steroid sparing
immunosuppressive therapy (Cyclophosphamide,
Endoxan 50 mg daily), with biweekly monitoring of
WBC and platelet counts. Surgical debulking was not
Figure 8.30: Photomicrograph shows numerous Toutan giant cells,

(Hematoxylin Eosin 40 ) suggestive of Xanthogranuloma
Figure 8.31: Photograph showing bilateral proptosis

in a patient with Xanthogranuloma orbit
B
Figures 8.32A and B: CT scan in the patient with Xanthogranuloma showing bilateral diffuse illdefined periocular, extra
and intraconal soft tissue lesion. Extraocular muscles cannot be identified separately.Clumps of calcification noted

considered in view of potential risks. Oral antiinflammatory drugs had to be stopped after a month
due to increase in dry eye symptoms. On last followup, patient was symptomatically better.The was
considerable decrease in proptosis and improvement
of extraocular motility. Patient was symptomatic due
to dry eyes, but had much reduced proptosis and
orbital symptoms.
ACKNOWLEDGEMENTS
We are grateful to Dr J Biswas and Dr S Krishna Kumar, Ocular
Pathology Service, Sankara Nethralaya for their help with the
photomicrographs and Dr Veena Noronha, Radiology Service,
Sankara Nethralaya for help in the interpretation of radiologic images.
We are also grateful to Dr Nirmala Subramaniam, Emeritus
Professor of Oculoplasty, Sankara Nethralaya for providing some
of the photographs.
Orbital Lymphoma
CHAPTER
Christopher s, Laurence Brown, Raghavan Sampath
The Lymphoproliferative diseases, including

malignant lymphoma, are not a single disease entity
but a collection of disorders ranging from benign
reactive hyperplasia, atypical lymphocyte infiltrate
to malignant lymphoma.1 Some consider idiopathic
orbital inflammation (pseudotumor) as part of the
lymphoproliferative disease spectrum. 1 Approximately 75% of patients with purely orbital lymphoma
will develop systemic disease, whilst 1-5% of those
with systemic disease will have orbital involvement.1
Overall orbital lymphoma accounts for less than 1%
of all lymphoma.2 Interestingly those patients with
atypical orbital lymphocytic infiltrate are at an
increased risk of systemic lymphoma.1
Various classifications of Lymphomas are in
vogue. They include the following:
Revised European American Lymphoma

Classification (REAL Classification)3
1. Leukemias and Lymphomas of B-cell Origin
(Pan B CD 19,20+)
A. Indolent B-cell malignancies:
(i) Small lymphocytic lymphoma
(ii) Hairy cell leukemia
(iii) Follicular lymphomas
(iv) Lymphoplasmacytoid lymphoma
(v) Marginal zone lymphoma.
B. Aggressive B-cell malignancies:
(i) Diffuse large cell lymphoma
(ii) Follicular large cell lymphoma
(iii) Mantle cell lymphoma
(iv) Burkitt's lymphoma
(v) Plasmacytoma / Myeloma
2. Leukemias and Lymphomas of T-cell Origin

(CD 2, 7+)
A. Indolent T-cell malignancies:
(i) T-CLL
(ii) Cutaneous T-cell lymphoma (Sezary
syndrome)
B. Aggressive T-cell malignancies:
(i) Peripheral T-cell NHL
(ii) Angioimmunoblastic T-cell lymphoma
(iii) Intestinal T-cell lymphoma
(iv) Adult T-ALL
WHO Classification of NHL

1. B-cell Neoplasms
A. Precursor B-cell ALL
B. Mature B-cell malignancies:
(i) B-cell CLL
(ii) Plasmacytoma
(iii) Extranodal marginal B-cell lymphoma
(iv) Mantle cell lymphoma
(v) Follicular lymphoma
(vi) Diffuse large B-cell lymphoma
(vii) Burkitt's lymphoma
(viii) B-cell promyelocytic leukemia
(ix) Hairy cell leukemia
(x) Lymphoplasmocytic lymphoma
(xi) Monocytoid B-cell lymphoma.
2. T-cell Neoplasms
A. Precursor T-cell ALL
B. Mature T-cell malignancies:
(i) Mycosis fungicides
Orbital Lymphoma 147

(ii) Adult T-cell lymphoma
(iii) Anaplastic or Null cell lymphoma
(iv) Peripheral T-cell lymphoma, not
specified
(v) T-cell prolymphocytic leukemia
(vi) Aggresive NK cell leukemia
(vii) T-cell gruanular lymphocytic
leukemia.
Modified Rye's Classification of Hodgkin's

Lymphoma4
1. CLASSIC HD:
A. Lymphocyte-predominance
B. Nodular sclerosis
C. Mixed cellularity
D. Lymphocyte depletion.
2. Nodular Lymphocyte-predominant HD
Clinically it is difficult to differentiate the
malignant and non-malignant tumors since age, sex,
presenting symptoms and radiographic findings are
similar. Even utilising immunohistochemical and
molecular biology techniques it is difficult to separate
the conditions. 1 Polymerase chain reaction is a
method of amplifying target genetic material in tissue
samples to identify a gene re-arrangement diagnostic
of lymphoma. This is particularly useful when the
microscopic morphology resembles chronic inflammation, but the clinical presentation is suggestive of
lymphoma.5
Orbital lymphoma is typically a non-Hodgkins
B-cell lymphoma arising from mucosa-associated
lymphoid tissue (MALTOMA)1,6 and accounts for
approximately 10-15%1,7 of all orbital masses. When
only malignant masses are taken in to account, they
account for 55% of the lesions. 1 Other types of
lymphoma identified in the orbit include extranodal
marginal zone lymphoma (MZL), follicular (FL),
diffuse large B-cell (DLBCL), mantle cell (MCL), Bcell chronic lymphocytic leukaemia (CLL)/small
lymphocytic lymphoma, peripheral T-cell lymphoma
(PTCL) and natural killer cell lymphoma (NKCL).8
Orbital lymphoma tends to be a bilateral disease
and can affect the conjunctiva, lacrimal gland, be
found in the nasolacrimal duct, as well as intraconal
and extraconal space.1,9 The orbital lesions tend to
present insidiously with painless proptosis in the sixth
decade,1,7,10 whilst visual impairment is relatively rare

occurring in only 13% of patients. 7 It can rarely
present like acute orbital inflammation or cellulitis.
MZL has the lowest risk of accompanying extraorbital disease and consequently, the lowest risk of
lymphoma-associated death.3
Radiographically orbital lymphomas tend to be
homogenious in nature and mould themselves
around orbital structures such as the globe and optic
nerve. 1,9 Bone erosion is rare although bone
destruction can occur with aggressive tumors.7
Radiotherapy is an effective treatment for orbital
MALT lymphoma using doses in the order of
30Gy.11,12 The 5 and 10 years survival rates for MALT
lymphoma is 100% and 88%.12
Orbital inflammatory disease include a wide
spectrum of conditions ranging from idiopathic
orbital inflammation to orbital involvement of specific
systemic inflammatory disorders such as Wegeners
granulomatosis, sarcoidosis, systemic lupus
erythematosus (SLE) or Tolosa Hunt syndrome.13 In
the case of Wegeners granulomatosis and sarcoidosis
ocular involvement occurs in approximately 50% of
affected subjects. The inflammation may affect
multiple or localized orbital tissues and can involve
the sclera. 13 Investigations are therefore guided
towards identifying these specific diagnoses. These
include a battery of blood tests including full blood
count (FBC), urea and electrolytes (U&E), C reactive
protein (CRP) and/or erythrocyte sedimentation rate
(ESR), autoantibody screen, anti-nuclear antibody
screen (ANA), ANCA, rheumatoid factor (RhF),
thyroid function tests (TFT), thyroid peroxidise
antibody screen and serum ACE. Radiological tests
include a chest X-ray, and orbital imaging (CT and/
or MRI). An orbital ultrasound scan can be beneficial.
Where no underlying cause can be identified the
diagnosis of idiopathic orbital inflammation can be
made.
When to Suspect Lymphoma

Age of onset: more common in the elderly
Insidious onset
Bilateral disease with no evidence of
indentation of the globe (choroidal folds)
Lacrimal gland involvement, lesions are typically
firm/rubbery on palpation

Conjunctival involvement, lesions are typically
salmon colored
Orbital imaging will reveal the lesion to be
moulded around the globe.
When to Suspect
Inflammatory Disease
Idiopathic
Orbital
Onset is usually acute

Symptoms include; pain, erythema, proptosis,
diplopia and blurred vision
Involvement of the extraocular muscles and the
sclera (scleritis)
Differential diagnosis includes; orbital cellulitis,
a systemic inflammatory disease such as
Wegeners granulomatosis, sarcoidosis,
polyarteritis nodosa and neoplasms such as
lymphoma
Investigations include; FBC, U & E, CRP,
antibody screen, serum ACE, ANA, ANCA,
RhF, thyroid function and thyroid peroxidase
antibodies
Imaging; orbital CT and/or MRI scan,
ultrasound scan to rule out scleritis and a chest
X-ray where sarcoidosis is suspected.
CASE ILLUSTRATIONS
Case 1
A 52 years old diabetic female presented to the orbit
clinic with enlargement of the left lacrimal gland.
Examination revealed proptosis of 5 mm on the left
side, whilst oculomotility was full, there was no
RAPD and funduscopy was unremarkable. Routine
bloods were taken to rule out autoimmune conditions
and inflammatory conditions such as Wegner's
granulomatosis, systemic lupus erythematosus (SLE),
Sjorgren's syndrome, and sarcoidosis. These were
all negative. An urgent CT scan revealed a discrete
soft tissue mass arising from the left lacrimal gland
(Figure 9.1) extending in to the orbit and displacing
the lateral rectus muscle. The right orbit was normal.
The differential diagnosis included plemorphic
adenoma and lymphoma.
Excision biopsy via a lateral orbitotomy approach
was performed since pleomorphic adenoma was
suspected.
Initial examination of the biopsy specimen
identified a dense exudate of lymphoid cells forming
prominent germinal centers. The appearance was
Figure 9.1: Left lacrimal gland enlargement
suggestive of reactive lymphoid change. Subsequent

immunohistochemistry identified T and B cell
proliferation and in one specimen kappa light chains
could be identified. A low grade lymphoma was
suspected. Molecular genetics confirmed the
diagnosis of low grade B cell lymphoma.
The wounds settled well postoperatively and
vision was maintained at 6/9 in the left eye and 6/5
in the right. A referral was made to the lymphoma
service for a course of radiotherapy. There was no
evidence of systemic lymphoma. 40 Gy was given to
the right orbit in 20 fractions.
Postradiotherapy she suffered from a dry eye
and was prescribed lubricants. A combination of
radiation and diabetic retinopathy subsequently
developed. However there was no evidence of
recurrence at 6 years.
Case 2
A 31 years old male attended the eye casualty with a
3 weeks history of bilateral orbital inflammation
(Figure 9.2) and right sided proptosis (Figure 9.3).
Visual acuity was 6/5 in both eyes and IOPs were
normal. Examination revealed bilateral conjunctival
chemosis, associated with reduced upgaze and
abduction. Fundoscopy was unremarkable. Routine
bloods were taken to rule out inflammatory
disorders. C-reactive protein and plasma viscosity
were elevated as was the white cell count. An urgent
MRI scan was arranged which revealed bilateral
enlargement of the lacrimal glands (Figure 9.4)
suggestive of lymphoma.
Urgent lacrimal gland biopsy was performed.
Histology confirmed the diagnosis of Hodgkins
lymphoma and referral to the lymphoma service was
made.
Orbital Lymphoma 149
Figure 9.2: Bilateral orbital inflammation
revealing only chronic inflammation. Lymphoma was

still suspected, and after a discussion with the patient,
a more extensive orbital biopsy was performed.
Histology was once again inconclusive, revealing only
scant lymphoid exudates. Subsequent immunohistochemistry identified B and T cells. No evidence
of lymphoma was found.
Referral was made to the lymphoma team to rule
out systemic lymphoma. None was identified. The
patient continues to be followed up.
Case 4
Figure 9.3: Right sided proptosis
Figure 9.4: Bilateral lacrimal gland enlargement
Case 3
A 63 years old male was referred to the eye casualty
with a 3 month history of diplopia and right sided
proptosis. Onset had been gradual and visual acuity
was 6/9 in both eyes. Examination revealed 3 mm of
proptosis in the right eye which was associated with
reduced ocularmotility in all directions of gaze.
Intraocular pressure was normal and there was no
evidence of an RAPD. Fundoscopy revealed evidence
of choroidal folds on the right although both discs
were healthy. There was no associated ocular pain
or headache and there was no history of weight loss,
fever or cough. He was an ex-smoker having stopped
6 years previously. There was no significant past
medical history. Routine bloods were taken to rule
out systemic causes. Urgent CT and MRI scans were
performed, revealing orbital inflammation suggestive
of lymphoma.
An urgent orbital biopsy was performed within
3 days and the patient started on a reducing dose of
steroids postoperatively. Histology was inconclusive
A 26 years old female presented to eye casualty with

a three day history of right orbital swelling. Visual
acuity was 6/9 in the right eye and 6/6 in the left.
There was marked chemosis (Figure 9.5) and
proptosis of the right eye associated with and
reduced upgaze and adduction. Fundoscopy was
normal and there was no evidence of papilloedema.
No RAPD was noted. The patient was apyrexial. A
diagnosis of orbital cellulitis was made and
appropriate treatment commenced. CT scan of the
orbits revealed preseptal and orbital inflammation
with evidence of mild proptosis. The extraocular
muscles and optic nerve were normal and sinuses
clear. There was no evidence of any abscess
formation. An orbital ultrasound scan was normal.
Despite intravenous antibiotics no improvement
in the symptoms was seen. Referral was made to the
orbital service and idiopathic orbital inflammation
diagnosed. Routine bloods were taken to rule out
sarcoidosis, Wegeners granulomatosis, and syphilis,
along with an auto-antibody screen, full blood count,
thyroid function tests and C-reactive protein screen
(CRP). All these tests were normal other than an
elevated CRP. Systemic steroids were commenced
(Prednisolone 1mg/kg) along with a histamine H2
receptor antagonist. Over the next few days the
symptoms and eye movements started to resolve.
Figure 9.5: Right sided chemosis and lid edema

The patient was discharged home on a reducing dose
of steroids. Urgent immunology and rheumatology
out patient appointments were arranged. No
underlying immunological or rheumatological
abnormalities were detected.
Three months following the initial episode, the
patient returned to eye casualty with a flare up in
the left eye, and then a further six months and two
years later in the right eye. All episodes settled with
a short course of systemic steroids. Biopsy is
normally recommended for all suspected idiopathic
orbital inflammation but in this particular patient the
features were typical with no localising lesion so a
biopsy wasn't performed.
Surgical Approach
Surgery is generally indicated to obtain tissue for
histology and to aid diagnosis of suspicious lesions.
Incisional biopsy is the treatment of choice.
Incisional Biopsy
The lateral 1/2 of the upper lid skin crease is marked
with pen and extended down parallel to the lid
margin, level with the lateral canthus, where it is
extended in a horizontal plane just past the orbital
rim. Local anesthetic is injected subcutaneously. A
skin incision is made along line with a cutting
diathermy. Both the palpebral and orbital parts of
the lacrimal gland are identified and an incisional
biopsy is made. Any other suspicious lesions are also
biopsied and sent for histology.
The deep tissues are closed with 5.0 vicryl and
skin closed with 6.0 prolene.
Suspicious visible conjunctival (bulbar and
palpebral) and sub-conjunctival lesions should also
be biopsied.
A reducing dose of steroids is given for 18 days
along with a histamine H2 receptor antagonist such
as ranitidine.
A head-light can be worn throughout the

procedure to ensure adequate illumination of the
operating field.
REFERENCES
1. Akansel G, Hendrix L, Erickson BA, et al. MRI patterns in
orbital malignant lymphoma and atypical lymphocytic
infiltrates. Eur J Radiol 2005;53(2):175-81.
2. Norton AJ. Monoclonal antibodies in the diagnosis of
lymphoproliferative diseases of the orbit and orbital
adnexae. Eye 2006;20(10):1186-8.
3. Jenkins C, Rose GE, Bunce C, et al. Histological features of
ocular adnexal lymphoma (REAL classification) and their
association with patient morbidity and survival. Br J
Ophthalmol 2000;84(8):907-13.
4. Schnitzer B. Classification of lymphomas. CRC Crit Rev
Clin Lab Sci. 1978;9(2):123-78. Review.
5. Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112
cases. Ophthalmology 1998;105(8):1430-41.
6. White WL, Ferry JA, Harris NL, Grove AS, Jr Ocular adnexal
lymphoma. A clinicopathologic study with identification
of lymphomas of mucosa-associated lymphoid tissue type.
Ophthalmology 1995;102(12):1994-2006.
7. Selva D, Rootman J, Crompton J Orbital lymphoma
mimicking optic nerve meningioma. Orbit 2004;23(2):11520.
8. McKelvie PA, McNab A, Francis IC, Fox R, O'Day J Ocular
adnexal lymphoproliferative disease: a series of 73 cases.
Clin Experiment Ophthalmol 2001;29(6):387-93.
9. Sullivan TJ, Valenzuela AA Imaging features of ocular
adnexal lymphoproliferative disease. Eye 2006;20(10):
1189-95.
10. Demirci H, Shields CL, Shields JA, Honavar SG, Mercado
GJ, Tovilla JC Orbital tumors in the older adult population.
Ophthalmology 2002;109(2):243-8.
11. Bhatia S, Paulino AC, Buatti JM, Mayr NA, Wen BC. Curative
radiotherapy for primary orbital lymphoma. Int J Radiat
Oncol Biol Phys 2002;54(3):818-23.
12. Hasegawa M, Kojima M, Shioya M, et al. Treatment results
of radiotherapy for malignant lymphoma of the orbit and
histopathologic review according to the WHO classification.
Int J Radiat Oncol Biol Phys 2003;57(1):172-6.
13. Gordon LK Orbital inflammatory disease: a diagnostic and
therapeutic challenge. Eye 2006;20(10):1196-206.
Vascular Lesions of Orbit 151
10
CHAPTER
Vascular Lesions
of Orbit
To comprehend the vascular lesions of the orbit, it

is very important to have embryological,
pathological and clinical concepts very clear in mind.
There have been numerous ways of classifying these
lesions, the most common being to divide them into
malformations, shunts and new growths.1 Many
entities have been placed under these three headings
which we will discuss in detail.
MALFORMATIONS
Malformations are present since the time of birth,
though they may not manifest at that time. Flat
endothelium lines their wall, and in contrast to
neoplastic lesions, do not show any growth in-vitro.
The orbital society has classified malformations as:2
a. No flow or hemodynamically isolated
malformations. For example: Lymphangioma.
b. Venous flow malformations. For example:
Varices.
c. Arterial flow malformations. For example:
Cavernous hemangioma.
d. Other congenital malformations. For example:
Phakomatosis.
Lymphangioma
These are benign vascular lesions seen usually in the
early childhood and commonly confused with orbital
venous anomalies and hemangiomas. Though they
are hemodynamically isolated, they arborize the orbit
and bleeding into their lumen causing chocolate cyst
is not very uncommon. Lymphangiomas often
enlarge during the upper respiratory infections

probably due to the inflammatory response of the
lymphoid tissue within the lesion.3
Superficial lymphangiomas are lesions of the lid
or conjunctiva, readily visible on inspection as
multiple serous or blood filled cysts. These are usually
purely lymphatic in character. Indication for
management is due to cosmetic reasons and can be
removed easily.4
Deep Lymphangiomas have in addition venous
connections and may cause slowly progressive
proptosis. They may present with increase in size
during upper respiratory infection. It can also present
with sudden proptosis due to bleeding into its lumen
causing a chocolate cyst. A significant number of
these patients may present with signs of optic nerve
compression like decreased visual acuity,
dyschormatopsia, diminished contrast and light
brightness sensitivity and visual field defects.
Imaging modalities used include Ultrasonography, CT or MRI. Of these MRI is the diagnostic
modality of choice.1 USG demonstrates cystic masses
in the retrobulbar space. CT shows low density
masses in intra and extraconal compartments with
minimal ring enhancement on contrast. No vascular
component is noted on angiography. MRI is useful
to delineate the lesions well and is also helpful in
timing the chocolate cyst as being acute, subacute or
chronic which may have clinical implications.5
Management is usually conservative since
spontaneous regression of the cysts is common.
Surgery though ungratifying because of incomplete

removal and recurrences, should still be carried on
in the presence of signs of optic nerve compression.
Carbon dioxide and contact Nd:YAG lasers are useful
surgical adjuncts.6
Other recent modalities that are gaining
upperhand in the initial management includes the
use of sclerosing agents. Many sclerosing agents in
use include Picibanil (OK-432)7,8 percutaneous ethanol9
and bleomycin.10 Certain other agents like 5% sodium
morrhuate 11 and sodium tetradecyl sulfate, 12
considered to be more effective by some for the
management of low flow vascular lesions have been
recommended as the first line therapy for
lymphangiomas.11
Orbital Varices
Orbital varices are weakened, dilated segments of
orbital venous system. Age at presentation varies
from childhood to middle ages .Most of the cases
are unilateral and upper nasal quadrant is the
favoured site. Clinical signs include visible lesions in
the eyelid or conjunctiva, or the patient may present
with a non-pulsatile proptosis which is accentuated
with increasing venous pressure like while straining,
assuming a dependant posture like sitting with a head
down position or by a valsalva maneuver. Since the
orbital venous channels are devoid of valves, a
reversible proptosis occurs.13 Rarely varices may
threaten the vision by optic nerve compression due
to acute hemorrhage or thrombosis. Chronic lesions
may present as enophthalmos.14
Imaging modalities used include CT scan,
Doppler ultrasonography and angiography. Doppler
demonstrates the flow of blood. Rapid spiral CT
during valsalva maneuver shows characteristic
enlargement of the engorged varix. Uniform contrast
enhancement is seen. Sometimes phleboliths may also
be seen. Angiography shows connection of the lesion
to the venous system and completely fills up
following injection.1
Management is usually conservative. But in the
presence of signs of optic nerve compression, surgical
removal is attempted .Complete removal is usually
not possible since the lesions are friable,
unencapsulated and bleed easily. Embolization using
coils through a distal vein is another method to
diminish symptoms.13
Cavernous Hemangioma
Cavernous hemangioma is the most common benign
orbital tumor in adults predominantly affecting
middle aged females. Most frequently it develops in
the intraconal space though it may also develop
elsewhere in the orbit.15
The patients present with slowly progressive
unilateral axial proptosis which may be associated
with decreased visual acuity, hyperopia, optic nerve
compression, optic disc edema, choroidal folds and
gaze-evoked amarousis, raised intraocular pressure
and strabismus. Bilateral cavernous hemangiomas
have also been reported.16,17
Imaging modality used commonly is a CT scan
which shows a well defined intraconal mass with
smooth margins that enhances either homogenously
or inhomogenously with intravenous contrast.
Sometimes small areas of calcification are seen.18 On
MRI the lesion is isointense and hyper intense to the
muscle on T1 and T2 weighted images respectively.
With Gadolinium contrast, the lesion fills up
homogenously.19
Management is surgical excision Lateral
orbitotomy is the most common approach if the lesion
is intraconal. Anterior orbital approaches are useful
for extraconal lesions. Surgical removal is much
simpler, since cavernous hemangiomas are well
encapsulated. Cryo is a very useful adjunct. In very
large lesions, passing a suture through the lesion
helps in two ways, for exsanguination of the tumor
reducing its size and to hold the tumor.
Other Congenital Malformations

Many other congenital malformations commonly
termed as phakomatosis and include 'Sturge-Weber
Syndrome', 'Wyburn-Mason Syndrome', 'KlippelTrenaunay Syndrome', 'Osler-Weber-Rendu
Syndrome' and many more rare malformations.1
Sturge-Weber Syndrome: It is also called as
encephalofacial angiomatosis and is a sporadic
phakomatosis that involves the leptomeninges, brain
and eyes. Struge-Weber is characterized by a naevus
flammeus or port wine stain over the area of
trigeminal nerve distribution, ipsilateral leptomeningeal hemangiomas and contralateral seizures
and hemiparesis. Ophthalmological features

include ipsilateral episcleral hemangiomas, glaucomas
and diffuse choroidal hemangioma and homonymous
hemianopia.
Diffuse choroidal hemangioma gives a
characteristic 'tomato-catsup' fundus.20 Imaging
modalities like CT and MRI are used. CT scan
may show 'tramline markings' of cerebral
calcification.
Management includes the use of Erbium laser for
naevus flammeus, external beam radiotherapy for
diffuse choroidal hemangiomas and medical control
of intraocular pressure followed by a combined
trabeculotomy-trabeculectomy for glaucoma.21
Wyburn-Mason Syndrome: This is a rare A-V
malformation of retina, optic nerve head and
posterior fossa involving a direct communication
between arteries and veins without the intervening
capillary bed. Orbits are occasionally involved with
ipsilateral portwine pigmented naevi over the
trigeminal course.1 CT scans may show enlarged optic
canal and bony orbit with a poorly defined enhancing
mass.22
Klippel-Trenaunay Syndrome: This rare
syndrome encompasses cutaneous hemangiomas,
venous varicosities and bony and soft tissue
hypertrophy of usually a single limb. Rarely is the
orbit involved with vascular anomalies.23
SHUNTS
Carotid-Cavernous Fistula
As obvious from the name, it is an abnormal
communication between the carotid artery and the
cavernous sinus. The blood in the cavernous sinus
becomes arterialized thereby raising the venous
pressure and at the same time the arterial perfusion
suffers. The fistula can be classified as 'direct or
indirect', 'high flow or low flow ' and 'spontaneous
or traumatic'. Barrow standardized the classification
in 1985.24
Trauma is the most common cause of direct or
type A fistulas usually seen in basal skull fractures.
Indirect or type C, D, E, are due to congenital
anomalies or spontaneous rupture of the artery
Barrow's types of carotid-cavernous fistulas24

Barrow
Type
Origin
Vessels Involved
Type A
Trauma
Internal carotid
Type B
Spontaneous
Meningeal branches of internal

carotid
Type C
Spontaneous
Meningeal branches of external

carotid
Type D
Spontaneous
Meningeal branches of internal

and external carotids.
secondary to aneurysm, atherosclerosis and severe

hypertension.
Clinical features include classical triad of
conjunctival chemosis, pulsatile proptosis and bruit.
Bruit is best heard as a flushing noise with the bell
of the stethoscope, reduced by ipsilateral carotid
compression in the neck. Other features include
ptosis; increased intraocular pressure due to elevated
episcleral pressure; 25 anterior segment ischemia
hallmarked by corneal edema, ischemic pseudoiritis,
rubeosis iridis and cataract; ophthalmoplegia, most
frequently affecting the 6th cranial nerve due to its
intracavernous location; diplopia Fundus examination
reveals dilated veins, optic disc edema and
intraretinal hemorrhages.
Imaging modalities used are CT scan, MRI and
angiography. CT scan shows enlarged superior
ophthalmic vein, enlarged extraocular muscles and
enlargement of the cavernous sinus.26 The definite
test in selective internal or external carotid
angiography which will demonstrate the fistula and
its hemodynamics.
Carotid cavernous fistulas are managed by
interventional radiologists.Usually these patients are
first seen by an ophthalmologist/orbital surgeon who
makes the diagnosis and refers to interventional
radiologist for management. Management indications
include secondary glaucomas, ophthalmoplegia,
severe proptosis and intolerable bruit.27 Balloon/
coils or surgical occlusion of the fistula is
recommended for the Type A. Balloon/coil can be
introduced either by an arterial or a venous route.

Type B and C can be treated with selective
embolization of a single feeder vessel whereas Type
D requires embolization of all the multiple feeder
channels. Various complications of interventional
radiology like vascular perforations, hemorrhage and
permanent neurological deficit have been reported.28
New Growths
New growths can be further subdivided into
'Hamartomas' and 'Neoplasms'. The hamartomas are
exemplified by cavernous hemangiomas whereas
the neoplasms include 'hemangiopericytoma',
'Angiosarcoma', 'Kaposi Sarcoma', 'angiomyomas',
etc.1
Capillary hemangiomas are hamartomas
characterized by growth of blood vessels along with
proliferation of endothelium. These are common
benign primary tumors of the orbit in children. It
usually presents in the first or second week after
birth and enlarges during the first year of life, after
which they begin to involute. About 70% regress by
7 years of age.
Clinical presentation are in the form of
strawberry naevus when the hemangioma involves
the lid. Involvement of the conjunctiva is important
from diagnostic point of view. Within the orbit
anterior and superior quadrant of the orbit is a
favoured site. Orbital lesions may present with a
progressive non-pulsatile proptosis, which may
increase following straining and crying.29 Capillary
hemangiomas have important systemic implications
like high output failure, 'Kasabach-Meritt Syndrome'
(Anemia + thrombocytopenia + low coagulant
factors)30 and 'Maffuci Syndrome' (Hemangiomas +
enchondromatosis).
Imaging modalities used include CT scan, MRI
and angiography.
CT scans demonstrates moderately well defined
lesion with finger like projections that may be present
in any orbital space. There is a moderate to intense
enhancement on contrast. Gadolinium enhanced T1
wieghted images with fat suppression shows diffuse
homogenous or heterogenous enhancement. Multiple
feeder vessels are seen on angiography.
Treatment is indicated when vision is threatened

by amblyopia as a result of anisometropia, ptosis or
strabismus.
Intralesional injection of steroids is the most
frequently used method. Usually 40-80 mg of
triamcinolone with 25 mg of methylprednisolone is
directly injected into the lesion. 1 Alternatively
Triamcinolone 40 mg along with betamethasone
4 mg can also be used. The tumor usually begins to
regress in two weeks but if necessary injection may
be repeated after about two months. Early
recognition and prompt treatment with intralesional
steroid prevents early occlusion amblyopia, but
follow-up and management of refractive amblyopia
with glasses and patching is necessary in the longer
term. Potential complications include skin
depigmentation, fat atrophy, eyelid necrosis and
rarely central retinal artery occlusion.
Systemic steroids are indicated for extensive
lesions especially if associated with visceral
involvement. Recommended dosage used is 1.5 to
2.5 mg/kg prednisolone daily over a few weeks with
titration downward depending on response.1
Though steroids are effective in large majority
of patients, a recurrence is not infrequent. Recurrent
or resistant cases are being treated with recombinant
interferon alpha-2a and 2b with variable results.31
Recent studies have demonstrated good efficacy of
interferons when given subcutaneously in a dose of
3 million units/m 2 . During clinical follow-up
diagnostic ultrasound evaluation ( the depth
dimension) proved helpful. One report suggested
high efficacy of treatment when a combination of
interferon alpha-2a with a low dose of
cyclophosphamide.
In the presence of very large platelet-consuming
lesions as seen with Kasabach-Meririt syndrome,
systemic antifibrinolytics like aminocaproic acid or
tranexemic acid are used.30
Surgical resection is carried out in cases where
vision is threatened or there is a failure of medical
management. Surgery should be carried out under
hypotensive anesthesia with constant hemostasis
during removal.32
Hemangiopericytoma
These are uncommon vascular tumors of the orbit
occurring in the middle ages. They are divided into
benign and malignant based upon the histopathology.
Clinical features include progressive painless
proptosis of usually less than one year duration,
predominantly in the superior part of orbit.
Hemodynamically there is rapid circulation with
significant shunting of blood. CT and MRI shows
well defined lesions with homogenous contrast
enhancement. 33 The microscopic features include
cellular, myxoid and storiform components with
spindle shaped pericytes, which stain positively with
vimentin upon immunohistochemistry.34
Management includes careful and complete
excision. The tumor has a pseudocapsule and is
notorious for recurrences. Histologically benign
tumors have been reported to metastasize. A very
aggressive local behavior may warrant an
exenteration.35
Angiosarcoma
Angiosarcomas are malignant tumors of the
endothelial origin, with an affinity for the head and
neck regions. Mostly affects males in 6-8th decades
of life. They are ill defined, multiple, involving the
skin of the lids and the orbit.36 They may present
with orbital apex syndrome and other neurological
deficits37 Since these tumors are aggressive, a wide
surgical excision is adviced.
Kaposi's Sarcoma
Kaposi sarcoma has generated considerable research
after the advent of AIDS.38 Though it is reported to
be common in the western literature, we are yet to
see a single case. Ocular involvement is usually of
the skin, lids or conjunctiva as reddish or purple
lesions and rarely lacrimal gland is involved. 39
Histologically vascular slit channels lined by
endothelium are seen.
Management includes the use of chemotherapy

and extended field radiotherapy.
Hemangioendothelioma
These are very rare tumors of the orbit. It is known
to affect all age groups with no age or sex
prelidiction. Multifocality is present in 9-14% of the
cases.40
It presents as a very rapidly enlarging mass with
edema or erythema of overlying skin. The tumor is
highly vascular and bleeds significantly on biopsy.
Unlike rhabdomyosarcoma, it has a mass effect rather
than being invasive. Imaging modalities used are
CT and MRI, which demonstrates lytic, multiloculated, expansile lesions of the orbit.
Histopathologically the tumor is composed of
irregular vascular elements lined with immature
endothelial cells with prominent anaplasia. All
hemangioendotheliomas are positive for at least one
endothelial marker. (CD31, CD34, factor VIII ).
Management includes histological grading followed
by treatment with radiotherapy, chemotherapy and
surgical removal.41
Hemangioblastoma
Hemangioblastoma is a rare tumor of the orbit. It
presents with progressive proptosis which can be axial
and abaxial as hemangioblatomas have been reported
both from the recti muscles42 and optic nerve.43 Optic
nerve hemangioblastomas are frequently familial,
presents with visual loss and RAPD and are associated
with infratentorial hemangioblastomas, angiomatosis
retinae, and cysts of the abdominal viscera. CT
and Magnetic resonance imaging reveals a wellenhanced mass, with an enlargement of optic canal
in cases of optic nerve lesions. Management includes
surgical removal with appropriate orbitotomy
approaches.43
Age
Early
childhood
childhood to
middle age
Middle ages
Variable
Ist year of
life
Middle ages
Name of the lesion
Lymphangioma
Orbital varices
Cavernous
hemangioma
Carotid-Cavernous
fistula
Capillary hemangioma
Hemangiopericytoma
Painless, progressive eccentric proptosis

of less than a year duration
Strawberry nevus on lids and conjunctiva. Nonpulsatile proptosis that increase with valsalva
maneuver. Additional features of associated
Kasaback-Meritt and Mafucci syndromes
Conjunctival chemosis, pulsatile proptosis with

a bruit, anterior segment ischemia and glaucoma
Slowly progressive unilateral axial proptosis,

decreased vision, hyperopia, optic nerve compression, choroidal folds and gaze evoked
amaraosis
Non-pulastile, reversible proptosis, increases

with valsalva maneuver
Progressive proptosis that increases with

upper respiratory infections, sudden proptosis with or without optic nerve compression
due to bleeding into the lumen (chocolate cyst)
Clinical features
Orbital Vascular Lesions at a Glance
CT and MRI shows well defined lesions

with homogenous contrast enhancement
CT, MRI and angiography. Finger like

projections into the orbit with moderate
to intense enhancement on contrast
CT, MRI and angiography. Enlarged

superior ophthalmic vein is a feature.
Definite test is angiography
CT and MRI. Well defined intraconal

mass with smooth margins and
enhancement with contrast
CT, Doppler USG and angiography.

Phleboliths may be observed on CT
USG, CT and MRI. MRI is modality of

choice as it delineates the leison well
and also helps in timing the chocolate
cyst
Imaging modalities
Careful excision. Aggressive

tumors require exenteration
Topical, intralesional and systemic

steroids. Interferons and cyclophosphamide. Surgical excision
Interventional radiology Balloon

or coil embolization
Surgical excision with a lateral

orbitotomy approach
Conservative, Embolization
Surgical removal
Conservative, Sclerosing agents,

Surgical with adjunctive lasers
Management

CASE ILLUSTRATIONS
Case 1
Mrs. B, female 45 years in age, has presented with
proptosis of her right eye since 5 years and
progressive loss of vision since 3 years. There was
no history of pain, trauma, change with posture, or
any systemic disease. She consulted an
ophthalmologist elsewhere 2 years back, who
ordered CT scan of orbit which was reported as
Meningioma of optic nerve sheath by the radiologist
and hence was advised conservative management
by the ophthalmologist.
On inspection, (Figure 10.1) she had a nonpulsatile proptosis of her right eye with displacement
of globe by 8 mm axially, and outwards by
5 mm.There was no change with Valsalva maneuver.
Fullness was seen with obliteration of superior sulcus.
Minimal mechanical restriction of ocular motility was
noticed. Pupil was dilated in size and direct light
reaction was absent. There was no perception of light.
Fundus exam revealed optic atrophy.
Non-tender, firm mass was palpable in the
superior peripheral space, extending into Orbit. Its
posterior border could not be felt. Orbital rim was
normal. Retropulsion was positive.
General examination was within normal limits.
Clinical Impression: In view axial proptosis, the
lesion should be in the intraconal space. The long
duration of proptosis, absence of visual symptoms
for a long period after the onset of proptosis, and
the severe degree of proptosis exclude lesions arising
from optic nerve or its sheath. (We are yet to see a
case of Meningioma of optic nerve sheath causing
such a huge proptosis). In view of the long duration,
sex (female) and the location (Cavernous
hemangioma is the most common intraconal lesion
in our experience), we made a diagnosis of
Cavernous hemangioma.
CT scan of orbit revealed a huge, hyper dense
lesion, occupying entire intraconal space and
extending into the peripheral space, more on the
medial compartment, pushing the globe temporally
(Figures 10.2 and 10.3). The lesion is very well
encapsulated. It has caused excavation of bony orbit.
It is not enhancing on contrast. All these features are
suggestive of Cavernous hemangioma. The tumor

was excised through lateral orbitotomy. On gross
examination it was very well encapsulated, measuring
55 mm 45 mm (Figure 10.4). Histopathology
confirmed it to be Cavernous hemangioma (Figure
10.5). Postoperative recovery was smooth and
satisfactory but for mild enophthalmos which was
due to increased orbital volume because of excavation
of orbital walls (Figure 10.6).
Figure 10.1: Proptosis left eye with globe pushed temporally
Figure 10.2: CT Coronal view well Figure 10.3: CT Axial view:

defined mass filling the entire conal Note the increase in orbital
space
volume
Figure 10.4: Gross specimen Figure 10.5: Histopathology shoof excised Well encapsulated wing dilated vascular channels
tumor
(H and E) cavernous hemangioma
Figure 10.7: Eccentric proptosis with inferior fullness
Figure 10.6: Postoperative picture showing relief from proptosis
Case 2
Mr. K, male, 32 years of age presented to us with
painless progressive proptosis of right eye since 3
years. There was no history of defective vision, or
diplopia.
On examination (Figure 10.7) we noticed eccentric
proptosis of the right eye, with fullness inferiorly.
The proptosis was nonpulsatile. Ocular motility was
normal. Pupil was normal. There was no RAPD.
Vision was 20/20 and color vision was normal. CT
scan showed a well defined lesion in the inferior
peripheral space with minimal contrast enhancement
and bony excavation of the floor of the orbit
(Figures 10.8 and 10.9), suggestive of Cavernous
hemangioma.
Anterior inferior orbitotomy was performed
through subciliary approach, and the tumor was
exsanguinated by passing a suture through the
substance of it to shrink its size (Figure 10.10).
This suture also helps in applying traction to assist
the excision of the tumor through a smaller
incision .
The excised tumor was pinkish in color and was
very well encapsulated. (Figure 10.11) The cut-section
of the tumor (Figure 10.12) showed honey-comb like
appearance with blood oozing out from the entire
cut surface. On histological examination the
encapsulated mass was made-up of dilated vascular
channels, filled with blood (Figure 10.13), confirming
the clinical diagnosis of Cavernous hemangioma. The
patient recovered well (Figure 10.14). The proptosis
disappeared completely. His vision remained 20/20.
The ocular motility was full.
Figure 10.8: CT scan shows well Figure 10.9: CT scan well defdefined lesion in inferior space
ined lesion with bony excavation
Figure 10.10: Suture through Figure 10.11: Well encapsulated

exsanguinated and shrinks Aids
tumor
excision by traction
Figure 10.12: Cut section showing Figure 10.13: Dilated vascular

honeycomb appearance with blood
channels filled with blood
oozing from it
Figure 10.15: Axial proptosis

of right eye
Figure 10.16: CT scan of brain

showing calcified lesions
Figure 10.14: One week postop, recovery from proptosis
There was no recurrence of the tumor. The points

to consider in this case were the location of cavernous
hemangioma in the inferior peripheral space, the
passing of suture through the hemangioma to bleed
the tumor and shrink its size, which facilitates to
remove the tumor through a smaller incision. Cryo
is other wise an excellent tool to hold the tumor
during its dissection and removal.
Figures 10.17A and B: Axial and Coronal sections of CT orbit showing

well encapsulated Intraconal lesion with contrast enhancement
Case 3
Mrs. J, a female 28 years of age, presented with
history that her friends and family members were
commenting that her right eye was looking prominent
since 3 months (Figure 10.15). She had no pain and
did not complain of any visual disturbances. Past
history was significant in that she had convulsions 4
years back and CT scan of brain showed 2 calcified
lesions (Figure 10.16). She was on carbamazepine
(Tegretol) since then. There was no relapse of
convulsions. On clinical evaluation, she had 3 mm of
axial and nonpulsatile proptosis, which did not
increase with Val-salva maneuver. The ocular motility
was normal. The pupils were brisk and the vision
was 20/20. CT scan of orbit revealed a hyper-dense,
contrast enhancing lesion of size 15 12 mm in the
intraconal space (Figures 10.17A and B). In view of
the short duration and contrast enhancement,
provisional diagnosis of a vascular tumor like
hemangioendothelioma/hemangiopericytoma was
considered. Lateral orbitotomy was performed and
the tumor was excised. Histopathology and Immunohistochemistry revealed it to be hemangioblastoma.
Further evaluation of the patient did not show any
Figure 10.18: Postoperative status showing complete recovery
other lesions in the posterior segment of the globes,

brain or elsewhere. The calcified lesions are probably
intracranial hemangioblastoma and since they were
inactive, neurosurgeon did not contemplate excision.
The patient has no recurrence during the past 3 years
of follow-up (Figure 10.18).
Case 4
Miss P, female child of 6 years presented with acute
proptosis of 2 weeks duration associated with severe
pain and defective vision. There was no history of
trauma.She never had similar problem previously.
On examination she had a non-pulsatile, proptosis
of 7 mm, associated with severe periocular fullness,
chemosis grade III associated with subconjunctival

hemorrhage, and restricted ocular motility in all
gazes .Pupil was dilated. RAPD was present. Her
best corrected vision was 20/200. Retropulsion was
positive. Tenderness was present on palpation
(Figure 10.19).
In view of acute onset associated with pain,
chemosis, restricted motility, subconjunctival
hemorrhage and chemosis, in a child, Lymphangioma
was the clinical diagnosis. CT scan showed a contrast
enhancing lesion with a huge hemorrhage in the
lesion, supporting the clinical diagnosis (Figure
10.20). Normally lymphangioma is managed
conservatively since it is not possible to excise it
completely and hence recurrence is very common.
But in this child as the vision is being compromised,
the parents were informed that though recurrence is
very common, surgery was to be performed to save
vision. Antero-lateral orbitotomy was performed.
About 6 cc of blood was aspirated from the lesion to
shrink it and excised as much as possible (Figure
10.21). The histopathology confirmed the diagnosis
of lymphangioma (Figure 10.22). Postoperative
recovery was smooth and the vision improved to
20/20 (Figure 10.23). After 3 years, she had a
Figure 10.23: Postoperative status. Proptosis

disappeared vision improved to 20/20
recurrence which was surgically managed. There was

no recurrence in the 2 year postoperative follow-up
after second surgery.
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8. CM Giguere, NM Bauman, Y Sato, DK Burke, JH Greinwald,
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Figure 10.21: Blood being

drawn from the lesion
intraoperatively
Figure 10.22: Histopathology

showing channels filled with lymph
9. JP Deveikis. Percutaneous Ethanol Sclerotherapy for

Vascular Malformations in the Head and Neck Arch Facial
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10. Mathur NN, Rana I, Bothra R, Dhawan R, Kathuria G,
Pradhan T Bleomycin sclerotherapy in congenital lymphatic
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Otorhinolaryngol. 2005;69(1):75-80.
11. Schwarcz RM, Ben Simon GJ, Cook T, Goldberg RA
Sclerosing therapy as first line treatment for low flow
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2006;141(2):333-9.
12. Wojno TH Sotradecol (sodium tetradecyl sulfate) injection
of orbital. Lymphangioma. Ophthal Plast Reconstr Surg.
1999;15(6):432-7.
13. Wright JE, Sullivan TJ, Garner A, et al. Orbital venous
anomalies. Ophthalmology. 1997; 104:905-13.
14. Cline RA, Rootman J. Enophthalmos: a clinical review.
Ophthalmology. 1984; 91:229-37.
15. Harris GJ, Jakobiec FA. Cavernous hemangioma of the
orbit: A clincopathological analysis of sixty-six cases. In:
Ocular and adnexal tumors. Birmingham, AL: Aesculapius,
1978:741-81.
16. Fries PD, Char DH. Bilateral orbital cavernous
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Cavernous hemangiomas of the orbit. Br J Ophthalmol.
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18. Forbes GS, Sheedy PF, Waller RR. Orbital tumors evaluated
by Computer tomography. Radiology 1980;136:101-11.
19. Ohtsuka K, Hashimoto M, Akiba H. Serial dynamic
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23. Good WV, Hoyt CS Optic nerve shadow enlargement in
Klippel. Trenaunay-Weber syndrome. J Pediatr Ophthalmol
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the orbital bone. Ophthalmol 1992. 99:1773-98.
42. Cockerham KP, Sachs DM, Cockerham GC, Hidayat AA,
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11
CHAPTER
Orbital Tumors of
Neurological Origin
Christopher M Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath
Optic Nerve Glioma

The first clinical report of optic nerve tumors was
by Antonio Scarpa in 1816, however it wasn't until
1912 that Hudson described optic nerve gliomas and
optic meningiomas as separate lesions.1 These tumors
are relatively rare, accounting for less than 4% of all
orbital tumors. Overall optic nerve gliomas account
for 65%1,2 of optic nerve tumors and meningiomas
35%. 1 Optic nerve gliomas appear to be more
frequent in females, whereas for optic pathway
gliomas, there may be an equal sex distribution.2
Lesions typically become symptomatic in childhood.3
The mean age of presentation is 8.8 years with only
10% presenting after 19 years of age.2 Most lesions
are sporadic although there is an association with
neurofibromatosis (NF 1). Adult optic nerve gliomas
may behave like those presenting in childhood,3
however some may show an aggressive behavior,
being clinically distinct from those seen in childhood.
These patients are typically middle aged with a slight
male bias.3
Benign optic nerve gliomas are typically pilocytic
astrocytomas originating from astrocytic glia and can
involve the visual pathways anywhere from the optic
nerve to the visual cortex.1,4 Histologically they are
composed of delicate, hair-like elongated eosinophilic
cells in an interwoven pattern. The nuclei may be
round or oval. Mitoses are rare, but nuclear atypia
may be observed. Malignant change is very rare.
The proliferating astrocytes in an optic glioma
may be associated with worm-like densely
eosiniophilic bodies, known as Rosenthal fibers,
surrounded by hyalinized connective tissue, a
distinctive (but not diagnostic) feature of pilocytic

astrocytomas. 5 Orbital gliomas, particularly in
patients suffering from NF 1, can extend through
the pia and arachnoid matter in to the subdural space.
They tend to remain intradural, however when
incompletely excised can recur diffusely invading
adjacent orbital structures. Where the lesion has
spread to the subarachnoid space reactive
proliferation of fibrovascular and meningeothelial
tissue can occur resulting in arachnoid hyperplasia,
which can resemble an optic nerve sheath
meningioma.1 Two patterns of growth are identified,
perineural (circumferential growth) and intraneural
growth. Perineural growth results from proliferating
astrocytes and fibrovascular tissue within the dural
sheath, widening the epipial-subdural space and
subsequently compressing the optic nerve. Intraneural growth results from growth of intra-axial
astrocytes causing the subarachnoid space to be
obliterated. Perineural growth appears to occur in
patients with NF1.1 Enlargement of optic gliomas
occurs by a combination of neoplastic cell
proliferation, reactive arachnoid proliferation and
accumulation of PAS positive mucinous substance.
Rapid growth can result from cystic degeneration
or intra-lesional hemorrhage. Malignant change can
occur however this is extremely rare.1
Malignant optic nerve gliomas seen in adults
show evidence of malignant astrocytomas and are
thought to originate within the optic pathways.
Extension is usually subpial along the optic pathways
although can directly invade the substance of the
brain.1
Orbital Tumors of Neurological Origin 163

Approximately 30-50% of benign optic gliomas
are associated with neurofibromatosis type 1
(NF 1)2, 4, the rest occur sporadically. The incidence
of optic nerve glioma in patients with NF 1 is 15-20%
of which only about 20% have visual symptoms.1, 4
The presenting symptoms depend very much on
the location of the lesion. Those involving
predominantly the intraorbital optic nerve present
with proptosis, ocular motility abnormalities, vision
loss and visual field loss. Intracranial tumors
including those involving the chiasm, tend to have
reduced vision and endocrine abnormalities
including precocious puberty.1-4 Fundoscopy may
reveal either optic atrophy or papilloedema
depending on the location of the lesion. Optocilliary
shunt vessels, central retinal vein occlusion and
venous stastis retinopathy are occasionally seen.1,3
Hemorrhage in to the tumor can result in acute
proptosis and sudden loss of vision.3
Investigation of optic nerve gliomas involves
computerized tomography (CT) and magnetic
resonance imaging (MRI). The appearance of the
lesion depends on whether the patient has NF 1 or
not. Fusiform swelling of the optic nerve is typically
seen in patients without NF 1, whereas those with
NF 1 tend to have more irregular nerves with areas
of kinking or buckling. 3 Cystic spaces may
occasionally be seen representing mucinous
accumulations. With perineural growth patterns,
there may be thickening of the dura with preservation
of the compressed optic nerve, mimicking the tramtracking seen in optic nerve sheath meningiomas.
Calcification is a rare finding.1 MRI shows the lesions
to be hypointense on T1 weighted images, whereas
on T2 images the glioma tends to be hyperintense.3
Contrast enhancement and fat suppression technique
can help to differentiate optic nerve meningiomas
from gliomas, since arachnoid hyperplasia associated
with gliomas does not enhance with gadolinium.1
Treatment options depend very much on the
location and extent of the lesion. Most optic nerve
gliomas have an indolent growth pattern and can
remain stable for many years, with some
spontaneously regressing.1, 3 Those lesions confined
to the optic nerve show a mortality rate of 5% from
intracranial extension. Tumor resection is curative
whilst the lesion remains confined to the optic nerve.

Lesions involving the chiasm have a mortality rate
of approximately 28% from intracranial spread, whilst
hypothalamic or 3rd ventricle involvement increases
the mortality rate to more than 50% at 15 years.1
Patients with isolated optic nerve involvement and
good vision should be reviewed on regularly with
repeat serial MRI scans of the optic nerve. When there
are signs of posterior progression the lesion should
be excised en block.1 Those lesions confined to the
optic nerve in blind, painful or cosmetically
unacceptable eyes should be considered for surgical
excision. 1,3,4 Optic nerve lesions extending to the
chiasm or those primarily involving the chiasm or
optic tracks could be considered for chemotherapy
or radiotherapy. Children 5-7 years and younger
should be treated with chemotherapy 2,4 since
radiotherapy at this age may result in damage to the
endocrine system and affect the future intellectual
development of the child.1,4 Children aged 10 and
above may be considered for radiotherapy using
various delivery techniques. Doses typically given
are in the range of 45 to 56 Gy given in 180cGy
fractions.
Malignant glioma seen in adults is invariably
fatal.1 Neuro-imaging of an involved optic nerve is
non-specific and the condition shows a rapid rate of
progression. Lesions affecting the proximal part of
the optic nerve can progress to affect both eyes within
5-6 weeks.3
Optic Nerve Meningioma

Optic nerve meningiomas account for one third of
primary optic nerve tumors and were first identified
as a pathological entity in 1835 by Jean Cruveilheir.
It wasn't until 1912 that Hudson clearly differentiated
optic nerve gliomas from meningiomas. 6 Most
meningiomas involving the orbit are extensions from
intracranial sites such as the sphenoidal wing
(secondary optic nerve sheath meningiomas).
Primary optic nerve meningiomas originate from the
cap cells of the arachnoid surrounding the intraorbital
or intracanalicular portion of the optic nerve.3 The
typical age at presentation is 40 years of age6,7 with
bilateral cases presenting much earlier at
approximately 13 years of age.6 There is a small sex
bias with 61% of cases occurring in females.6

Histologically most orbital meningiomas are of
the meningiothelial or syncytial variety. The cells are
eosinophilic with abundant cytoplasm and indistinct
cell margins. The nuclei are small and vesicular with
occasional pseudoinclusions. Cells may be wrapped
with or without psammoma bodies in tight whorls.5
Approximately 90% of meningiomas involving the
orbit originate from intracranial sites, primarily the
olfactory groove and the sphenoidal ridge.6 Ectopic
lesions have been described in the orbit, seemingly
independent of the optic nerve, these may have
developed from orbital mesenchymal cells or may
be a case of mistaken identity, since lesions such as
fibroxanthomas and hemangiopericytomas closely
resemble meningiomas.6
NF 1 is associated with optic nerve meningioma,
although the relationship is no where near as high as
for optic nerve glioma. The incidence of NF 1 in
patients with optic nerve meningioma is 2%, whereas
the incidence of NF 1 in the general population is
0.03-0.05%.6
The classical triad of clinical findings in optic
nerve meningioma are visual loss, optic atrophy and
optociliary shunt vessels. 3,6 The simultaneous
occurrence of these three findings is however rare.
The most common finding is vision loss which can
take the form of visual obscurations. Other findings
are reduced color vision and visual field defects.3,6
Proptosis seems to follow the onset of visual loss,
tending to be reasonably mild.3,7 It is thought to occur
because tumor growth results in a straightening of
the optic nerve and may also account for any ocular
motility defects with up gaze being most commonly
affected.6,7 Orbital pain may occur. Examination may
reveal disc swelling although the only signs may be
optic atrophy. Optociliary shunts which present in
less than one third of patients3,7 are shunts between
the retinal and choroidal circulations and thought to
be a result of a reopening of regressed vestigial
embryonic retinociliary anastomoses.6 They may be
seen in eyes with other causes of disc swelling. The
disc swelling and central retinal vein congestion may
proceed the shunt vessels by 1-2 years becoming
noticeable as the swelling starts to resolve3 and then
regress as optic atrophy sets in.6
Meningiomas arise from meningothelial cells
along the meninges. Large collections are known as
pachionian bodies or arachnoid bodies whereas

smaller ones are known as arachnoid villi. Optic
nerve meningiomas are thought to arise from
meningiothelial cap cells of the arachnoid villi.6 Two
patterns of growth are seen, a syncytial pattern in
which polygonal cells are arranged in sheets
separated by vascular trabeculae and a transitional
pattern where spindle cells are arranged in concentric
whorls. Mitoses are uncommon. Psammoma bodies
are occasionally seen in which calcium salts can be
deposited. Meningiomas may spread in the
subarachnoid spaces along paths of least resistance.
They can extend in to the surrounding tissues3 and
the haversian canal system of bone resulting in
hyperosteosis. They do not seem to invade the brain
and are not associated with raised intracranial
pressure or pituitary dysfunction.6 Growth is slow
although may accelerate with pregnancy and results
in compression of the optic nerve.6
Investigation involves CT and MRI imaging.
Calcification when present is useful in distinguishing
optic nerve gliomas from meningiomas. Typically the
lesions show 'tram-tracking' in which a thickened
optic nerve sheath surrounds a central lucent optic
nerve.3,6,7 On coronal views this is seen as a dense
ring surrounding the central optic nerve.6 MRI with
gadolinium enhancement is particularly sensitive in
detecting meningiomas.7
Treatment options include surgery and
radiotherapy, however observation is not unreasonable since the mortality rate is low.7 Surgery
may be indicated for aggressive lesions however it
is associated with a high degree of local recurrence
and orbital invasion. Typically surgical excision
results in blindness, since the tumor and optic nerve
often share their blood supply.6,7 Decompression of
the nerve has been attempted by opening the dural
sheath, which in some cases can arrest the visual
deterioration.3,6 Fractionated radiotherapy with a
total of 40-54Gy given in divided doses seems to be
effective in stabilizing and in some cases improve
vision, whilst at the same time limiting the risks of
optic nerve or chiasm damage to less than 5%.3,7,8
Interestingly the tumor volume appears unchanged
following radiotherapy, despite an improvement in
neurological function.8 The side effects of treatment
including headache, nausea and hair loss can be

limited by fractionating the doses. Later side effects
include pituitary dysfunction, retinopathy, iritis and
temporal lobe atrophy.8 The risk of optic nerve or
chiasm damage associated with high doses of
radiotherapy. When doses greater than 59Gy are
given in fractions of less than 1.9Gy there is an 11%
risk of injury whilst doses greater than 1.9Gy have a
47% risk according to a University of Florida study.9
Low dose steroids at the time of radiotherapy may
limit damage from radiation induced peritumor
edema.8 The risk of a second malignancy following
radiotherapy is 0-2% at 10-20 years.8 When a lesion
is treated with a single dose of radiation the
threshold for injury is 8-10 Gy.8
Orbital Schwannoma (Neurilemmoma) and

Neurofibroma
Schwannomas also known as neurilemmomas
account for approximately 1-4% of orbital tumors,
occurring most commonly in men between the second
and fifth decades.10,11 They occasionally arise from
the optic nerve, probably originating from schwann
cells of sympathetic nerves tightly adherent to the
optic nerve.3 More frequent sites are the other cranial
nerves including the oculomotor, lacrimal, trigeminal
and zygomaticotemporal nerves. 10,11 The most
commonly affected cranial nerve is the vestibular
nerve.12
Schwannomas are slow growing lesions that do
not invade surrounding tissues.11 Clinical features
include proptosis and diplopia. Globe compression
can induce a hypermetropic shift, whilst optic nerve
compression results in reduced visual acuity.
Malignant transformation is rare.10
Gross examination reveals a well encapsulated
yellowish grey lesion, with cysts containing clear
fluid. Histologically schwannomas show two growth
patterns: Antoni A where densely packed spindleshaped cells are arranged with palisaded nuclei
sometimes forming Verocay bodies, and Antoni B
where cells are separated by an abundant myxoid
stroma with no alignment of nuclei. Mitoses are
usually absent. The associated nerve may sometimes
be seen in one side of the lesion, in contrast to a
neurofibroma where the originating nerve is
expanded by the lesion and cannot be seen. So-called
"ancient schwannomas" may feature bizarre enlarged
or multiple nuclei, but there are no malignant
connotations.10,13 The lesions may be very vascular,

causing diagnostic confusion.
Surgery is the treatment of choice11 although it is
associated with usual risks of orbital surgery
including loss of sight. Recent advances in steriotactic
radiosurgery and fractionated radiotherapy in the
treatment of vestibular schwannoma and nonvestibular schwannoma have achieved high levels of
tumor growth control whilst preserving cranial nerve
function.12,14 Future advances may mean that this
treatment option could be used to treat and control
orbital schwannomas.3
Neuro-imaging reveals a homogenious lesion isointense with rest of the surrounding neural tissue
mimicking other lesions such as optic nerve gliomas,
cystic spaces are sometimes seen.3,11 Histopathology
is required to make a definitive diagnosis.10,11,15
Neurofibromas are another group of benign
nerve sheath tumors which can occur in the orbit
region.16 In a retrospective review by Rose et al. in
1991,17 looking at peripheral nerve tumors in the orbit,
they found that 93% of these lesions were either
schwannomas (neurilemmomas) or neurofibromas.
In their series they found that most affected nerve
was the first division of the trigeminal nerve.
Neurofibromas are typically associated with
neurofibromatosis, although they can occur in
isolation. Approximately 25 to 45% of all lesions are
found in the head and neck region.16 Other sites
typical affected by neurofibromas include the eyelids,
the orbit and rarely the lacrimal sac.18
Clinically they tend to present with painless
proptosis and diplopia. Pain and altered sensation
are rare.17
Grossly, the lesions appear as an encapsulated
firm white mass. Histologically neurofibromas consist
of wavy cells with basophilic nuclei.16
The treatment of choice is surgical excision. Even
when incompletely excised recurrence is low.17
When to Suspect an Orbital Tumor of

Neurological Origin
Insidious onset
Proptosis typically axial
Symptoms may include: Pain with and without
eye movement, diplopia, blurring of vision,
reduced color vision and increased hypermetropia

Fundoscopy may include: Unilateral disc swelling
and choroidal folds
Firmness of the globe to retropulsion.
Investigations
Bloods to rule out an inflammatory cause
Orbital imaging: CT and/or MRI imaging
preferably with contrast enhancement
Orbital biopsy where imaging is suspicious or
the diagnosis is in doubt.
CASE ILLUSTRATIONS
Case 1
A 2 years old child with Neurofibromatosis type 1
was referred to the eye department by the pediatric
oncology service following surgery for a cerebellar
astrocytoma and hydrocephalus.
At presentation she had an unrelated right
convergent strabismus for which she underwent
convergent squint surgery. Fundoscopy including
the optic disc was unremarkable and there was no
RAPD. There was no evidence of proptosis.
Routine MRI scans (Figures 11.1 and 11.2)
revealed sub-clinical bilateral optic nerve
gliomas.
Over the following 8 years optic disc cupping

developed, with the left disc being paler and more
cupped. Color vision remained normal in both eyes.
Case 2
A 66 years old female presented to the orbit clinic
with a 6 months history of horizontal diplopia. Visual
acuity was 6/9 in the right eye and 6/12 in the left.
Ocular motility appeared normal however diplopia
was reported on dextroversion. There was proptosis
of 4 mm on the left and a left relative afferent
papillary defect. Only 2 of 13 ishihara plates were
correctly identified by the left eye whereas color
vision in the right eye was normal. There was no
evidence of papilloedema, although the left disc was
slightly pale. Fundoscopy revealed signs of age
related macular degeneration in both eyes. Routine
bloods were taken.
Urgent MRI and CT scans were performed,
revealing an intraconal soft tissue mass which involved
the sphenoidal wing and extending intracranially to
the temporal lobe (Figure 11.3). The findings were
strongly suggestive of a meningioma. A subsequent
CT scan showed evidence of hyperostosis of the
sphenoidal wing, as well as bone loss in the posteriorlateral aspect of the orbit. The diagnosis of a
sphenoidal wing meningioma was made.
A neurosurgical referral was made and the
patient was offered the option of a craniotomy and
surgical debulking of the tumor. As there was no
guarantee that this would improve her vision,
surgery was declined. The condition is being
managed conservatively with regular clinic follow
up and routine repeat MRI scans.
Case 3
Figure 11.1: Bilateral optic nerve glioma (axial view)
Figure 11.2: Bilateral optic nerve glioma involving the chiasm

(coronal view)
A 66 years old female was referred from a district

hospital with retro-orbital pain. An MRI had revealed
Figure 11.3: Left optic nerve meningioma

an intraconal retrobulbar mass displacing the optic
nerve superiorly, with no evidence of bone
destruction. Visual acuity was 6/9 in the right eye
and 6/6 in the left. Color vision slightly reduced in
the right eye, seeing 13 out of 15 ishihara plates, the
left was normal. A mild right RAPD was noted. Both
optic discs were normal and there was no proptosis.
A diagnosis of optic nerve meningioma was made.
Following discussion with the patient and oncology/
radiotherapy department a dose of 50.4 Gy was given
in 14 fractions to the orbit.
Subsequent MRI scans revealed a reduction in
the tumor following the radiotherapy. After
treatment the patient experienced a dry right eye,
which was treated with lubricants, and there was
evidence of mild radiation retinopathy.
Figure 11.4: Low power meningioma H and E stain
Case 4
A 74 years old female with a known optic nerve
meningioma was referred to the orbital clinic with a
recurrence of orbital symptoms and enlargement of
the lesion. Visual acuity in the affected eye was NPL,
whereas the right was 6/6. Proptosis was noted on
the left side. The left disc was atrophic and there
was marked chorioretinal atrophy. An MRI confirmed
enlargement of the lesion with extension towards
the orbital apex. Following discussion with the
patient a decision was made to debulk the
meningioma via a lateral orbitotomy. Histology
confirmed the diagnosis of a meningioma (Figures
11.4 and 11.5).
Figure 11.5: High power meningioma H and E stain
Case 5
A 36 years old male attended the orbit clinic
with an 18 months history of worsening left sided
proptosis (Figure 11.6). Visual acuity was unaffected;
left eye 6/6, right 6/5, although color vision was
slightly reduced, with only 11 out of 13 ishihara color
vision plates being correctly identified in the left eye.
Color vision was normal in the right eye. The visual
field on the left showed generalized depression on
the left side although no relative afferent
papillary defect was identified. Oculomotor
function was reduced on abduction and upgaze in
the left eye. Fundoscopy of both eyes was normal
with no evidence of papilloedema. The central
nervous system examination was unremarkable and there was no past medical history of
relevance.
Figure 11.6: Left sided proptosis
An urgent MRI scan of the orbits was arranged

identifying a 2.5 cm rounded intraconal lesion which
was enhanced following intravenous contrast
administration (Figure 11.7). The optic nerve, lateral
and inferior rectus were displaced and that the lesion
was intraconal (Figure 11.8). The optic nerve was
separate from the lesion. An MRI scan was also
Surgical Approach
Where orbital surgery is considered to obtain a tissue
diagnosis or to debulk an orbital tumor, a lateral
orbitotomy approach is chosen since this gives good
access to the retro-orbital spaces and any lesions
found there. If there is evidence of intracranial
extension, referral to a neurosurgeon is indicated, a
craniotomy may be indicated.
Lateral Orbitotomy
Figure 11.7: Left orbital schwannoma axial view
Figure 11.8: Left orbital schwannoma sagittal view
organized overall the MRI and CT findings

suggested the lesion was a possible cavernous
hemangioma. An urgent excision biopsy was
arranged via a lateral orbitotomy approach.
Macroscopically the tumor was found to be cystic
and wrapped around optic nerve. The lesion was
debulked. As the surgery proceeded the pupil
became fixed and dilated. No further debulking was
attempted and the wound closed.
Postoperatively the pupil gradually recovered
and vision remained 6/6.
Histopathological examination revealed the
lesion to be a cystic Schwannoma.
Referral was made to the oncology service and
the tumor treated with radiotherapy.
A lateral orbitotomy approach is employed to biopsy

tumors arising in the anterior third of the orbit. The
lateral 1/3 of the skin crease is marked with pen and
extended down parallel to the lid margin to a height
level with the lateral canthus. The line is then
extended laterally in a horizontal plane for 1 cm.
Local anesthetic is injected subcutaneously in this
region. A skin incision is made along this line with a
cutting diathermy. The deep tissues are blunt
dissected down to the periosteum. This is incised
with the diathermy and the periosteum blunt
dissected off the bone exposing the lateral wall from
the frontozygomatic suture down to the lower border
of the lateral wall just above the opening of the
zygomaticofacial foramen. At the orbital rim the
periobita is elevated off the internal aspect of the
lateral wall of the orbit. The orbital contents are
displaced nasally with a malleable retractor. Two
holes cm apart and cm back from the orbital
margin are drilled parallel to the orbital rim at the
superior border of the exposed bone and 2 at the
bottom. A gap of approximately 3 cm will exist
between the higher of the 2 lower holes and the lower
of the upper 2 holes. The lateral orbital wall is incised
with a power saw between the drilled holes both
superiorly and inferiorly. The cuts extended for
approximately 2 cm in a radial direction to an area
where the bone thins. A malleable retractor should
be used to protect the globe during this procedure.
The section of bone is dissected off with forceps and
stored in normal saline.
Once the lesion is identified care should be taken
to avoid the globe, optic nerve and rectus muscles,
while incision biopsies are taken using a 15 blade
and forceps. Pupil reactions are monitored
throughout the procedure. Hemostasis is achieved
with bipolar cautery. The lateral orbital wall is
replaced by threading 4.0 prolene through the

pre-holes at the orbital rim and tied in place. The
tissues are closed with 5.0 vicryl and skin closed with
5.0 prolene. A reducing dose of steroids is given for
18 days along with a histamine H2 receptor antagonist
such as ranitidine.
A head-light is worn throughout the procedure
to ensure adequate illumination of the operating field.
Where a drain is used this can be removed the
following day.
REFERENCES
1. Dutton JJ. Gliomas of the anterior visual pathway. Survey
of Ophthalmology 1994;38(5):427-52.
2. Jahraus CD, NJ Tarbell. Optic pathway gliomas. Pediatric
Blood and Cancer 2006;46(5):586-96.
3. Miller NR. Primary tumours of the optic nerve and its
sheath Eye 2004;18(11):1026-37.
4. Kaufman LM, O Doroftei. Optic glioma warranting
treatment in children Eye, 2006. 20(10):1149-64.
5. Rosenblum MK, Bilbao JM Ang L Neuromucular system
Chap 28 in Surgical Pathology Ed Rosai (9th ed) 2004;2:2461682.
6. Dutton JJ. Optic nerve sheath meningiomas. Survey of
7. Carrasco JR, RB Penne. Optic nerve sheath meningiomas
and advanced treatment options. Current Opinion in
8. Melian E, WM Jay. Primary radiotherapy for optic nerve

sheath meningioma. Seminars in Ophthalmology,
2004;19(3-4):130-40.
9. Parsons JT, et al. Radiation optic neuropathy after
megavoltage external-beam irradiation: analysis of timedose factors. International Journal of Radiation Oncology,
Biology, Physics, 1994;30(4):755-63.
10. Subramanian N, et al. Cystic schwannoma of the orbita
case series Orbit 2005;24(2):125-29.
11. Tezer MS, et al. Schwannoma originating from the
infraorbital nerve: a case report. Auris Nasus Larynx 2006;
33(3):343-5.
12. Pollock BE, RL Foote, SL Stafford. Stereotactic radiosurgery:
the preferred management for patients with nonvestibular
schwannomas? Int J Radiat Oncol Biol Phys, 2002;
52(4):1002-7.
13. Rutherford SA, AT King. Vestibular schwannoma
management: What is the 'best' option? Br J Neurosurg,
2005;19(4):309-16.
14. Tsuzuki N, et al. Cystic schwannoma of the orbit: case
report. Surg Neurol, 2000;54(5):385-7.
15. Rosai J Soft Tissues Chap 25 in Surgical Pathology Ed Rosai
(9th ed) 2004;2:2237-371.
16. Chua CN, Alhady M, Ngo CT, Swethadri GK, Singh A, Tan
S Solitary nasal neurofibroma presenting as compressive
optic neuropathy. Eye 2006;20(12):1406-8.
17. Rose GE, Wright JE. Isolated peripheral nerve sheath
tumours of the orbit Eye 1991;5(6)668-73.
18. Dailey RA, Sullivan SA, Wobig JL. Surgical debulking of
eyelid and anterior orbital plexiform neurofibromas by
means of the carbon dioxide laser. American Journal of
Ophthalmology, 2000;130(1):117-19.
12
Mesenchymal Tumors
CHAPTER
E Weis, J Rootman
Mesenchymal Soft Tissue Tumors

Tumors that are believed to arise from mesenchymal
tissues occur in multiple sites with variable biologic
behavior. In the orbit mesenchymal tissues include
striated and smooth muscle, fibrous tissue, fat,
cartilage, and bone. Bone tumors are discussed
separately in Chapter 13. Despite orbital mesenchyme
arising from neural crest, lesions do not differ from
other locations in the body in which mesenchymal
tissues arise from mesoderm. In our orbital center
mesenchymal tumors account for 1.6% of orbital
lesions and 9% of neoplasias; in children they
constitute 5% of all disease and 19.4% of childhood
neoplasia.1
Striated Muscle Tumors

Rhabdomyosarcoma
Epidemiology
Rhabdomyosarcoma accounts for approximately 20%
of all soft-tissue sarcomas making it the most
common soft tissue sarcoma in children.2, 3 The head
and neck is the principal location and the orbit the
second most common site in the head and neck (the
most common being the parameninges) accounting
for about 10% of all rhabdomyosarcomas. 4-6
Rhabdomyosarcoma is thus the most common
primary orbital malignancy of childhood. In our clinic
it constitutes 1% of all orbital neoplasias, and 6% in
children.1
It has occurred from birth to the seventh decade,
but 70% present in the first decade with a mean age
of 8. The embryonal subtype affects mainly children,
the alveolar mainly adolescents (median age 16), and
the much rarer anaplastic (pleomorphic) subtype most

commonly presents in older people (median age is
54).7-11 Embryonal rhabdoymyosarcoma is the most
common subtype accounting for 49% of all
rhabdomyosarcomas and their predominance in the
orbit is even greater since this subtype has a
predilection for the orbit and parameninges.12
Most cases are sporadic although familial,
congenital, and multiple tumors in the same patient
including retinoblastoma have been reported.13 Most
evidence suggests that they arise from primitive
mesenchyme and not from skeletal muscle as they
can develop in areas with no skeletal muscle.14
Presentation
The typical presentation is rapidly developing
exophthalmos over weeks (mean of 5) with 60%
presenting with signs of inflammation including
conjunctival and eyelid swelling.9,10,15 Two-thirds of
primary orbital rhabdomyosarcoma present with a
mass in the superonasal quadrant.15,16
The differential diagnosis is that of a childhood
progressive rapidly developing mass with or without
inflammation: infantile hemangioma, lymphangioma,
neuroblastoma, chloroma, cellulitis, and non-specific
orbital inflammation.
Imaging
There are no specific radiologic findings in
rhabdomyosarcoma.17 Local bone invasion has been
reported in 24% of cases,18 with destruction of the
orbital wall without orbital expansion seen in 30%.15,19
CT imaging typically shows a homogeneous (92%)
well-defined soft tissue mass without bone
Mesenchymal Tumors 171

destruction that takes up contrast in a moderate to
marked amount; rarely the mass is poorly defined
as it invades surrounding structures. It is most often
extraconal (87%), in the supero-nasal quadrant (66%),
(Figures 12.1A and B) and may displace but does
not appear to arise from the extraocular muscles.15,16,20
Invasion of the sinus is noted in 20%. 15 Focal
hemorrhage or necrosis may result in heterogeneity.17
MRI typically shows isointensity to skeletal muscle
and hypointensity to orbital fat on T1, hyperintensity
on T2 to orbital fat and muscle, decreased signal
intensity on all pulse sequences, and moderate to
marked uptake of gadolinium.16,17,20
Classification
The International Classification of Rhabdomyosarcoma combined previous histologic
classification schemes to provide a system based on
prognosis.12 The Pleomorphic subtype was excluded
from this classification system because of its rarity
in children (Table 1). The WHO classification has
divided rhabdomyosarcoma into embryonal,
alveolar, and pleomorphic subtypes. The spindle cell
and botryoid are considered variants of the
embryonal subtype.21 The alveolar subtype more
commonly presents in the inferior orbit.
included the lung (66% of metastasis), lymph nodes,

and bone marrow. 22 Interestingly, lymph node
metastasis is highly related to site of origin with lower
rates seen in orbital tumors.22 Since the development
of combination therapy, including biopsy/
conservative surgery, radiation, and multi-agent
chemotherapy, the majority of children are now
surviving. 14 Survival is related to pre-treatment
tumor extension past the site of origin, size, nodal
involvement, and metastasis at presentation. 6
Anatomic site is related to prognosis with the orbit
having the best prognosis (92% survival). 5,23,24
Histologic type, as previously mentioned, is also
associated with survival.12 (Table 1).
Management begins with pathologic confirmation and staging. Two contrasting clinical
philoshophies have emerged from the main clinical
trial groups. The American Intergroup RhabdoTable 1: The International classification of
Rhabdomyosarcoma (1995)
Management
Before the 1960s rhabdomyosarcoma was an almost
uniformly fatal disease. Common metastatic sites
Superior prognosis
Botryoid
Spindle cell
Intermediate prognosis
Embryonal
Poor prognosis
Alveolar
Undifferentiated
Subtypes whose prognosis
Rhabdomyosarcoma with
is not presently available
rhabdoid features
Figures 12.1A and B: This 11-year-old child presented with a 3-week history of swelling of the left upper lid associated with ptosis and
intermittent diplopia. It was a nonpainful swelling. She had an interpalpebral fissure of 5 mm on the left compared to the right at 8 mm, with 3 mm
downward and 1 mm axial displacement of the left globe. This was associated with a 2 diopter left hypotropia in primary position, which
increased to 6 diopters in upgaze. There was a solid, rubbery, palpable mass just behind the superior oblique tendon adjacent to the trochlea.
On CT scan, there was a well-defined, homogeneous, hyperdense extraconal mass in the superomedial orbit displacing the eye and medial
rectus muscle downward. The superior muscle group appeared displaced laterally. Because of the rapid development of this mass, an
incisional biopsy was performed. Histopathologically, the mass was consistent with an embryonal rhabdomyosarcoma. Repeat investigations
revealed no other evidence of tumor, and the patient underwent chemotherapy and radiotherapy. She is alive and well 17 years later with
pseudophakos (20/25-2) and some enophthalmos

myosarcoma Study Group (IRSG) have tended to be
more aggressive by utilizing routine radiotherapy,
except for tumors that are totally excised, followed
by prolonged chemotherapeutic regimes.25,26 Whereas
the European International Society of Pediatric
Oncology (SIOP) have attempted to use short course
chemotherapy and have avoided surgery or
radiotherapy if possible.27-29 The best treatment option
is likely somewhere between these two philosophies.30 The vast majority of recurrences occur
within 3 years of presentation and can often be
treated with chemotherapy and repeat excision. In
cases with refractory orbital tumors, salvage surgery
has been shown to be beneficial.31
Complications related to treatment include
cataract (55-82%), dry eye (30-36%), radiation
retinopathy (6%), and bony hypoplasia (24-59%)
secondary to radiation induced damage. 15,32
Secondary malignancies are rare (1.2-3%) and most
commonly occur in patients who have received
alkylating agents and radiation.15,33
Rhabdomyoma
Tumors of skeletal muscle differentiation are atypical
in that malignant are more common than the benign.
Rhabdomyoma is a rare benign tumor that has had
only 6 cases described in the orbit.34-38 Extracardiac
rhabdomyomas are divided into 4 categories: adult,
fetal, genital, and the rhabdomyomatous mesenchymal hamartoma types. The adult and fetal type
have a predilection for the head and neck and the
rhabdomyomatous mesenchymal hamartoma occurs
mainly in the periorbital and perioral subcutaneous
tissue in children.14 We have reported the only case
of rhabdomyomatous mesenchymal hamartoma
reported in the orbit.38 Tumor excision or debulking
with observation have been described if
symptomatic, since they can regress with time. 39
Recurrences are extremely rare and are typically
associated with incomplete removal.
Very little has been written about the radiologic
findings of extracardiac rhabdomyoma.17 CT imaging
demonstrates an ill defined homogeneous lesion that
does not show signs of necrosis or hemorrhage with
heterogeneous enhancement.37 The adjacent bone can
be remodelled secondary to pressure but destruction
is absent.37 MRI demonstrates a well defined mass
similar to muscle on T1 and T2 weighted images with
variable enhancement patterns.37,40-42
Smooth Muscle Tumors

Smooth muscle tumors of the orbit are exceedingly
rare and can arise from Mullers muscle and the smooth
muscle overlying the inferior orbital fissure.
Leiomyoma is a benign slow growing lesion with
three distinct clinical groups: (1) leiomyoma
cutis, (2) deep dermal (genital leiomyomas), and
(3) leiomyoma of deep soft tissue (musculoskeletal).14
The most common location for these tumors is the
female genital tract.44 About 20 cases in the orbit have
been reported with a mean age of presentation in the
orbit of 36 years.43 Complete excision is the preferred
treatment, since they are not sensitive to radiation,
and no orbital recurrences have been found with
complete excision. 43 Interestingly, regression is
commonly seen with leiomyomas in other body sites.14
Leiomyosarcomas are rapidly growing infiltrative malignancies that can vary in their natural
behavior. They account for 5-10% of soft tissue
sarcomas2,45,46, are more common in females, present
at a median age of 60,47 and they can occur in younger
individuals who have received radiation therapy.48,49
Most commonly they are retroperitoneal, thus lesions
outside this location are poorly understood; yet
certain differences have been consistently documented. Extra-peritoneal location seems to have a
better prognosis with a 5 year survival of
approximately 64% and equal gender incidence.45,50
Although extremely rare in the orbit exenteration is
the preferred treatment.51-55 Adjuvant chemotherapy
and radiotherapy may be beneficial.56
Imaging of subcutaneous leiomyoma typically
show a well-defined mass on MR with T1 intensity
similar to skeletal muscle and T2 displaying a
heterogeneous high or mixed signal intensity.17 Deep
leiomyomas often show calcification, T1 intermediate
signal intensity, T2 variable intensity, and marked
contrast enhancement.17 Leiomyosarcomas do not
display specific features on imaging. Hemorrhage,
necrosis, and cystic change are common. A
hypervascular mass with arteriovenous shunting is
often seen on arteriography.17
Adipose Tumors
Despite fat making up the majority of the orbital
volume and lipomas being the most common soft
tissue tumor in the body 14, tumors arising from
adipose tissue in the orbit are rare. Lipomas should

be differentiated from prolapsed orbital fat since
they are often well circumscribed, slightly more
yellow, and displace orbital structures.57 (Figures
12.2A to D) They present commonly in mid-life, are
more frequent in men, are not related to race, and
after an initial growth phase typically do not change
in size significantly.58,59 Lipomas are divided into deep
and subcutaneous/superficial lesions which are much
more common.
Lipomas are identical to subcutaneous fat on CT

and MRI.17 In the orbit they can have lower density
than the surrounding orbital fat simulating a cyst.57
When the tumor is encapsulated a low signal fibrous
capsule may be visualized on MR and CT. 17
Nonencapuslated lipomas have been reported making
it difficult to localize them when adjacent to normal
fat.67
Figures 12.2A to D: (A) This 21-year-old female noted a nonpainful, mildly tender swelling of the left lower lid for 2 years. There was no
history or findings of increase in size with Valsalva maneuver and no ecchymotic episodes. On physical examination, the vision was normal.
There was a soft palpable mass not attached to the underlying tissue with 2 mm of vertical displacement and 1.5 mm of relative enophthalmos.
Ocular movements were normal. (B, C) CT scan demonstrated a well-delineated low-density lesion anteriorly (C-large arrow). The lesion
appeared to be adjacent to orbital fat, which was of a higher density (C-small arrow). The lesion also appeared to cause bone excavation
anteriorly (B-arrow). It was thought to be a lipomatous tumor. At the time of excision, a well-defined lipomatous mass was removed en bloc from
the inferolateral orbit. (D) Histologically, it had the typical features of a lipoma. Reprinted with permission from Shah NB, Chang WY, White VA,
et al. Orbital lipoma:2 cases and review of the literature. Ophthalmic Plastic and Reconstructive Surgery. 2007;23:203

Liposarcoma is one the most common soft tissue
sarcomas of adult life,14 but is rare in the orbit.1,60
Thirty five cases have been reported in the literature,
but misclassification is likely since our definition and
pathological evaluation has improved over time.61,62
In orbital cases the average age was 64 years of age
with a female predilection. 55 The most common
presentation is painless proptosis with an 8 month
mean duration of symptoms.55 Three distinct entities
have been described, each validated with cytogenetic
evidence. In order of good to poor prognosis they
are: well-differentiated (adipocytic, sclerosing,
inflammatory, spindle cell, and dedifferentiated),
myxoid/round cell, and pleomorphic.14,63 In the orbit
the myxoid is the most common subtype.62 Unlike
lipomas which are primarily subcutaneous in location
liposarcomas are primarily deep tumors; if they occur
superficially they are referred to as atypical and have
an excellent prognosis since they are often amenable
to complete surgical resection.64-66 It is generally
believed that lipomas cannot transform into
liposarcomas; the distinction in location between
lipomas and liposarcomas is the primary argument
cited as proof of this.14 Clinical and histopathologic
diagnosis can be difficult. Treatment involves surgical
excision which typically involves exenteration,
although well circumscribed cases have been resected.
Liposarcomas of the orbit have a favorable prognosis
with no reports of a neoplasm, that meets todays
histopathological criteria for diagnosis, metastasizing
and only one recurring locally.61,62
When a lesion with imaging characteristics similar
to a lipoma with an adjacent non-fatty mass,
liposarcoma should be added to the differential
diagnosis. Liposarcomas vary from approximately a
25-75% fatty component depending on their level of
differentiation.68 Liposarcomas also show calcification
in approximately 12% of cases which is more common
than in lipomas.17 Few papers have described the
imaging in the orbit. Jakobiec et al. described central
lucency due to fat seen on CT imaging resembling a
cystic structure. 61 They also reported that these
lesions commonly involved the extraocular muscles
and one case was hyperintense on MRI T1 imaging.61
A single case report described unique characteristics
including an extraconal mass with MRI T2 imaging
showing hypointensity in comparison to cerebral
cortex and light peripheral enhancement with
gadolinium.54
Lesions shown to mimic fatty tumors on imaging

include myxoid tumors, lesions associated with a
subacute hematoma, muscle with fatty replacement,
and tumors invading surrounding fat.17
Fibrous Tissue Tumors

Despite the large number of subtypes of fibrous
tumors, only a few have been reported in the orbit.
Fibrosarcomas have been diagnosed much less
commonly over time as our definition and
pathological assessment has advanced.14 Recent series
have found that they are slightly more common in
men69 and usually present in the early 40s. 70, 71
Recurrence is very common occurring in
approximately 45% with surgical margins being the
best predictor. 69 Scott et al.s series found that
recurrence occurred in 79% of tumors with
inadequate margins and in 18% of those treated with
wide or radical excision.69 Five year metastasis rates
are around 63% and are typically to the lung, spine,
and skull.69 A 5 years survival rate of approximately
40% has been reported.69 These lesions are rare as a
primary tumor of the orbit but they can also invade
from the nasal cavity or face. Exenteration or excision
with wide local margins is recommended since they
are commonly incompletely removed. CT and MR
imaging typically show an aggressively infiltrating
orbital mass,72 however infantile lesions have been
reported to be well circumscribed.73
Solitary fibrous tumor classically occurs in the
pleural lining of the lung, but has been described
throughout the body including the orbit, adjacent
nasal cavity and sinuses.14 Approximately 55 cases
have been reported in the orbit since its first
description there in 1994 with most cases being
benign.74-76 It has been seen in all age groups, with a
median age of 50,77 and usually presents as a wellcircumscribed yet unencapsulated lesion causing
gradual proptosis.1 Pleural solitary fibrous tumor can
vary greatly in its clinical characteristics and
malignancy on pathological assessment. Even less is
known about non-pleural solitary fibrous tumors.
Approximately 10% of non-pleural lesions have been
shown to recur and metastases are very rare. 78
Pathological assessment for atypical features was
found to be a significant predictor of recurrence.78
Since these tumors are difficult to remove due to
invasion and they may undergo malignant transformation en bloc resection is recommended.79

On CT the lesions are well circumscribed and
take up contrast intensely in a homogeneous or ring
pattern,76 although one review suggests that contrast
uptake is mild to moderate.72 With MRI these tumors
also enhance intensely, commonly showing areas of
hemorrhage and T2 hypointensity.72
Histiocytic Tumors
Fibrous histiocytoma
Fibrous histiocytomas histologically and clinically can
vary from a slowly developing benign to locally
aggressive fast growing malignant lesion. Four
subtypes have been described in order of frequency:
storiform-pleomorphic, myxoid (myxoidfibrosarcoma), giant cell (malignant giant cell tumor of
soft parts), and inflammatory (xanthosarcoma,
malignant xanthogranuloma). 14 Benign fibrous
histiocytoma most commonly affects the skin but
approximately 0.3% can occur in deeper locations such
as muscle tissue. 80 Recurrence rates for benign
cutaneous fibrous histiocytomas following excision
range from 5-10%,14,81 with deeper tumors typically
presenting as a much larger lesion more often
resulting in incomplete resection and thus recurrence
rates have been reported closer to 50%.82,83
Malignant fibrous histiocytomas are the most
common adult malignant soft tissue tumor in the
body.84 Font et al.s orbital series found that 63%
were benign, 26% were locally aggressive and 11%
were malignant.82 They showed that if the tumor had
infiltrative margins, hypercellular zones or both,
recurrence rates were 57% compared to 31% in those
that did not have these features.82 Typically they are
slow-growing, firm, infiltrative, and present in the
upper nasal quadrant with a mean age of 43 years.82
Common presenting symptoms include proptosis
(60%), mass (46%), and decreased vision (25%) with
a mean duration of symptoms of 29 months. 82
Complete surgical excision is recommended. This is
typically accomplished by removing the tumor alone
although in some infiltrative cases this might not be
possible without exenteration. Recent large systemic
series have reported recurrence rates from 19-21%,
metastasis, mostly to lung and bone, in 31-35%, and
5 year survival rates from 65-70%.85-87 In contrast
ten year survival rates for orbital tumors is about

90%.1,82
It can be difficult to differentiate fibrous from
other lesions.17,72 (Figures 12.3A and B) No specific
ultrasonic and CT imaging characteristics have been
found (Figures 12.4A to E).88 Imaging is also highly
variable related to amounts of collagen, necrosis,
hemorrhage, and myxoid tissue but often displays
high signal intensity on T2. Benign tumors are
typically well-circumscribed and may remodel bone,
with malignant lesions typically having infiltrating
margins and bone destruction. 72 Benign histiocytomas are usually homogeneous on CT, and MRI
T1 and T2 imaging, whereas malignant lesions have
a more heterogeneous pattern. 72 In some cases
malignant lesions may have a homogeneous CT and
T1 image with a heterogeneous T2 image. 72
Histopathology and immunohistiochemistry help in
the diagnosis (Figures 12.5A and B)
Malignant Tumors of Uncertain Type

Rhabdoid tumor is a highly aggressive neoplasm
primarily seen in the kidney although it has been
reported in extrarenal sites including the central
nervous system and soft tissues including the
orbit.14,89-96 Extrarenal sites have approximately a 15%
survival, but there are too few reported cases in the
orbit to come to a conclusion as to whether they
behave differently than other extrarenal sites. Of the
7 described in the orbit 4 have died.
Figures 12.3A and B: (A) This 34-year-old woman presented with a

history of right upper lid swelling and ptosis over 1 year, which was
associated with tearing, light sensitivity and occasional sharp pain.
Physical examination findings were a palpable lacrimal gland on the
right, bilateral reduced Schirmer's function, 3 mm of ptosis and 3 mm
of proptosis. There was a soft nontender mass superolaterally, which
was causing a 3 mm downward and 4 mm inward displacement as
well as indentation of the globe, leading to elevation of macula and
disc (B)
Figures 12.4A to E: (A, B) The ultrasound for the patient depicted a well-defined solid lacrimal mass indenting the globe. (C to E) CT scan
demonstrated a well-defined, solid, enhancing, slightly bosselated mass that indents the globe and causes excavation of the adjacent lacrimal
fossa. The mass appeared distinct from the lacrimal gland and enhanced to a greater degree
Figures 12.5A and B: The patient underwent an excision biopsy of the lesion, which at the time of surgery appeared yellow and soft. It was
excised from the adjacent lacrimal gland. (A, B) Histologically, it was an encapsulated, somewhat myxomatous, vascular spindle cell lesion,
which was negative for S100, keratin and epithelial membrane antigen but positive for smooth muscle antigen, vimentin and CD34. The
histologic differential diagnosis included fibrous histiocytoma, myxoid tumor or leiomyoma
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84. Enzinger, FM, Malignant fibrous histiocytoma 20 years after

Stout. Am J Surg Pathol, 1986. 10 Suppl 1:43-53.
85. Salo, JC, et al., Malignant fibrous histiocytoma of the
extremity. Cancer, 1999;85(8):1765-72.
86. Le Doussal, V, et al., Prognostic factors for patients with
localized primary malignant fibrous histiocytoma: a
multicenter study of 216 patients with multivariate analysis.
Cancer, 1996;77(9):1823-30.
87. Zagars, GK, JR Mullen, and A. Pollack, Malignant fibrous
histiocytoma: outcome and prognostic factors following
conservation surgery and radiotherapy. Int J Radiat Oncol
Biol Phys, 1996;34(5):983-94.
88. Jacomb-Hood, J and IF Moseley, Orbital fibrous
histiocytoma: computed tomography in 10 cases and a
review of radiological findings. Clin Radiol, 1991;43(2):
117-20.
89. Haas, JE, et al., Ultrastructure of malignant rhabdoid tumor
of the kidney. A distinctive renal tumor of children. Hum
Pathol, 1981;12(7):646-57.
90. Sotelo-Avila, C, et al., Renal and extrarenal rhabdoid tumors
in children: a clinicopathologic study of 14 patients. Semin
Diagn Pathol, 1986;3(2):151-63.
91. Stidham, DB, et al., Congenital malignant rhabdoid tumor
of the orbit. J Aapos, 1999;3(5):318-20.
92. Walford, N, et al., Intraorbital rhabdoid tumour following
bilateral retinoblastoma. Histopathology, 1992;20(2):170-3.
93. Gunduz, K, et al., Malignant rhabdoid tumor of the orbit.
Arch Ophthalmol, 1998;116(2):243-6.
94. Rootman, J, KF Damji, and JE Dimmick, Malignant rhabdoid
tumor of the orbit. Ophthalmology, 1989;96(11): 1650-4.
95. Niffenegger, JH, et al., Adult extrarenal rhabdoid tumor of
the lacrimal gland. Ophthalmology, 1992;99(4):567-74.
96. Gottlieb, C, et al., Congenital orbital and disseminated
extrarenal malignant rhabdoid tumor. Ophthal Plast
Reconstr Surg, 2005;21(1):76-9.
13
Bone Tumors of Orbit
CHAPTER
Venkatesh C Prabhakaran, Dinesh Selva
Primary bone tumors of the orbit constitute 0.6 to

2.0% of all orbital tumors.1, 2 They can be classified
into 4 main sub-groups (Table 1).3 Clinically, the
commonest bone tumors encountered are osteomas,
fibrous dysplasia and cholesterol granuloma.
Clinical Presentation
Bony lesions of the orbit usually present in one of
three ways:3
1. Gradual proptosis and globe displacement
occurring over many years secondary to a slowly
progressive non-infiltrative mass effect. This is
commonly seen in benign fibro-osseous tumors,
such as the osteoma.
2. Sub-acute proptosis or globe displacement
occurring over weeks or months which may be
complicated by a sudden increase in the signs
and symptoms secondary to an intralesional
hemorrhage. This presentation is characteristic
of reactive bone lesions.
3. Malignant bone tumors can present with
infiltrative signs such as pain, restricted
movements and decreased vision occurring over

weeks or months.
On radiological examination, either destruction of
bone or hyperostosis is seen. It is important to note
that lesions other than primary bone tumors can also
cause these changes. Thus the differential diagnosis
of a destructive bony lesion includes epithelial
malignancies of the paranasal sinuses, bony
metastases, Wegeners granulomatosis, lymphomas,
fibrosarcoma and lytic meningioma. Hyperostotic
lesions may be seen in metastatic prostatic carcinoma,
meningioma and osteomyelitis.
Only the three commonest lesions (osteoma,
fibrous dysplasia and cholesterol granuloma) will be
discussed in detail. The other conditions are briefly
reviewed.
Osteoma
Osteomas are the commonest bony tumors affecting
the orbit. Orbital osteomas usually arise in the frontal
or ethmoidal sinus. They usually present in the fourth
and fifth decades and occur equally in males and
females.4
Table 1: Clinico-pathological classification of primary orbital bone disorders

Benign fibro-osseus and
cartilaginous lesions
Reactive bone lesions
Neoplasms
Vascular
Osteoma
Fibrous dysplasia
Ossifying fibroma
Chondroma
Cholesterol granuloma
Aneurysmal bone cyst
Giant cell granuloma
Brown tumor of
hyperparathyroidism
Langerhans cell histiocytosis

Myeloma
Osteosarcoma
Ewings sarcoma
Intraosseus hemangioma
Osteoblastoma
Mesenchymal chondrosarcoma
Giant cell tumor
Bone Tumors of Orbit 181

Clinically, osteomas cause gradual proptosis and
globe displacement occurring over many years. There
may be an associated headache and a bony mass is
usually palpable in the superior or superomedial
orbit. Obstruction of the sinus ostia may lead to
sinusitis or mucocoele formation. Uncommon
features include acquired Browns syndrome, gazeevoked amaurosis or pain, globe subluxation and
erosion leading to orbital emphysema or CSF
rhinorrhea. Osteomas of the sphenoid sinus, though
rare, are important as they can cause an orbital apex
syndrome and optic atrophy.3
An important systemic association is Gardners
syndrome which is a phenotypic variant of the familial
adenomatous polyposis syndrome. Gardners
syndrome is an autosomal dominant condition whose
features include colonic polyps, osteomas, and
congenital hypertrophy of the retinal pigment
epithelium (CHRPE). As the rate of malignant
transformation of the colonic polyps is very high,
screening for Gardners syndrome (dilated
funduscopic examination and gastroenterologist
referral) is recommended in patients with
osteoma.5
On X-ray and CT scans, osteomas are extremely
radiodense, well circumscribed lesions, usually
arising in the sinus and invading the orbit. Bone
windows on CT usually show a very dense periphery
with a more radiolucent center.
Histologically, osteomas are composed of lamellar
bone with fibrovascular stroma. They are divided into
three types: ivory, compact and fibrous osteomas
based on the relative proportions of lamellar bone
and fibrous stroma. 6 The ivory osteomas are
considered to be the most mature form of the lesion.
However, there does not seem to be a correlation
between clinical behavior and histological subtype.3
Asymptomatic
osteomas
are
treated
conservatively, except when involving the
sphenoidal sinus, as it is easier to remove a small
lesion before it encroaches on the optic canal.
When symptomatic, treatment is by surgical
excision. Anterior lesions are removed via an anterior
orbitotomy. A modified Lynch incision may be used
for superomedial osteomas. Endoscopic techniques
may also be used.7 Posterior lesions may require a
combined orbitocranial approach. Recurrence is rare,
even following partial removal.
CASE ILLUSTRATION (Figures 13.1A to D)

Fibrous Dysplasia
Fibrous dysplasia develops almost exclusively in
children in the first two decades of life, though the
disease may progress well into adulthood. It is a
deforming but not destructive disease of bone and
is caused by proliferation of fibrous tissue and
osteoid in medullary bone.
Three forms are described: monostotic fibrous
dysplasia accounts for most cases of orbital
involvement; in polyostotic disease, deformities of
long bones occur together with skull lesions; and
McCune-Albright syndrome which is a triad of
polyostotic fibrous dysplasia, sexual precocity and
cutaneous pigmentation (with coast of Maine
borders).
Most patients present with facial asymmetry,
proptosis and globe displacement progressing
over many years. Associated symptoms include
diplopia, anosmia, hearing defects, nasal obstruction
and epiphora.8 Increased intracranial pressure and
cranial nerve palsies can also occur. Extensive disease
results in a deformed facies known as leontiasis
ossea.
Progressive disease can result in optic nerve
compression. Acute compressive neuropathy may
occur secondary to intralesional hemorrhage,
sphenoidal sinus mucocoele or secondary aneurysmal
bone cyst.3
Imaging shows expansion of the bone with
thinning of the overlying cortex. A ground-glass
appearance is common on CT. The disease usually
affects multiple orbital bones, extending across suture
lines.
The main differential diagnosis is hyperostotic
meningioma, which occurs in an older age group,
and is differentiated on imaging by the presence of
a soft tissue component.
Histopathology shows a loose, moderately
cellular fibrous stroma containing spicules of
woven bone often with characteristic C or
Chinese character shapes. Osteoblastic activity is
inconspicuous.6
As the natural history of the lesion is usually one
of slow progression, a conservative approach to
management is recommended, unless functional
Figures 13.1A to D: A 19-year-old male presented with a dull headache and a swelling in the region of the left upper lid. On clinical examination
(A), fullness was noted above the left medial canthus with downward displacement of the globe. A bony-hard mass could be palpated in the
left superomedial orbit. Computed tomographic scan (B and C) revealed a highly circumscribed radio-opaque mass with a bosselated contour
occupying the frontal sinus and extending into the left superonasal orbit. The bone window settings (B and C) demonstrated the radiolucent
trabecular center surrounded by a dense periphery, characteristic of an osteoma. Surgical removal was performed using a stereotactic
assisted sino-orbital approach. The sinus component was removed by a nasal endoscopic approach following a modified Lothrop procedure.
The orbital component was drilled out through an external skin-crease approach. (D). The patient made an uneventful recovery following
surgery
deficits develop. Surgical treatment requires a

multidisciplinary craniofacial approach, with removal
of as much of the affected bone as possible and
reconstruction of the resulting defect in a single
operation.9

Ossifying Fibroma
This is a lesion peculiar to craniofacial bones. The
mandible is the site of predilection but the orbit
(usually the roof) may rarely be involved.6 It usually
presents in the first two decades of life with a very

slowly progressive displacement of the globe.
Imaging shows a well circumscribed lesion eroding
the bone with a sclerotic margin and foci of internal
calcification.
Histopathology reveals trabeculae of bone and
osteoid lined by osteoblasts in a cellular stroma.6 A
psammomatoid variant is described, in which
ossicles remniscent of psammoma bodies are seen.10
This variant shows more aggressive behavior and
has a greater risk of recurrence following incomplete
excision.
Figures 13.2A to D: This 22-year-old female presented with progressive decrease of vision in the left eye. Clinical examination revealed facial
bony asymmetry with prominent frontal ridges (A). On cutaneous examination (B), a large nevus with the so-called coast of Maine borders
was seen. Computed tomographic scan (C) showed extensive expansion of craniofacial bones extending across suture lines, with a groundglass appearance. These features are consistent with a diagnosis of polyostotic fibrous dysplasia (McCune-Albright syndrome). As the
disease was causing visual compromise, left optic nerve decompression was performed via a craniotomy. Histology shows the typical Cshaped trabeculae of woven bone (D, arrow) set in a cellular fibrous stroma
It is important to distinguish ossifying fibroma

from fibrous dysplasia as the former lesion is more
aggressive, and left alone, inexorably enlarges and
may enter into the cranium. As incomplete excision
frequently leads to recurrence6, complete surgical
excision is the treatment of choice.
Osteoblastoma
This is a benign tumor composed of osteoblasts and
is extremely rare in the orbit.11 It affects patients in
the second to third decades and presents with a
slowly progressive proptosis and globe displacement.
The reported cases have arisen from the roof and
ethmoid sinuses and imaging shows an osteolytic

lesion larger than 1 cm with a sclerotic margin.
Histologically, trabeculae of lamellar bone with
osteoblastic rimming are seen. The histological
appearance is indistinguishable from that of osteoid
osteoma (a lesion seen in long bones and measuring
less than 1 cm in diameter).6 Surgical excision is the
treatment of choice.
Chondroma
Chondromas are benign cartilaginous tumors that
may rarely be encountered in the orbit, usually near
the orbital rim or trochlea.12 Radiologically, they are

seen as dense, well circumscribed masses. On
histology, they are composed of lobules of mature
cartilage.13 Excision is curative.
Cholesterol Granuloma
Cholesterol granuloma is a foreign body response
to the presence of crystallized cholesterol. It
commonly involves the middle ear and temporal
bone, but the orbit may rarely be affected. In the
orbit, it occurs almost exclusively in the superolateral
frontal bone.14, 15
Males in the fourth to fifth decades of life are
predominantly affected. The usual presentation is that
of a slowly progressive superolateral mass resulting
in inferior globe displacement, proptosis and diplopia
in upgaze. There may be associated headache or pain
and a history of trauma may be elicited in some
patients.
CT imaging demonstrates an osteolytic lesion
expanding and eroding the frontal bone and
extending into the orbit and intracranially.14, 15 With
MRI, high signal intensities are seen on both T1 and
T2 weighted images (similar to dermoid cysts).
Histology is characterised by the presence of
numerous cholesterol clefts and an associated foreign
body giant cell reaction.
Curettage of the lesion via a percutaneous
approach is curative. An endoscope may be helpful
in visualizing areas behind the superior orbital rim.16
CASE ILLUSTRATION (Figures 13.3A to F)

Aneurysmal Bone Cyst
This cystic lesion of the bone is rare in the orbit and
usually presents with a painless proptosis in the
second decade of life.17 Sudden progression may
occur following an intralesional hemorrhage. Imaging
shows a destructive, expansile bony lesion usually
involving the roof of the orbit. Curettage of the lesion
is curative and histological examination shows blood
filled fibrous spaces that lack an endothelial lining.
Hemosiderin laden macrophages and bony trabeculae
are seen in the fibrous stroma surrounding the
spaces.3
Giant Cell Lesions

A histological picture dominated by giant cells is seen
in three different lesions: giant cell tumor, giant cell
granuloma and brown tumor of hyperparathyroidism. Giant cell tumor or osteoclastoma is
usually seen in epiphyses of long bones and affects
patients between the ages of 25-40 years. 6 It
uncommonly involves the paranasal sinuses and can
secondarily impinge upon the orbit.6 Histology shows
evenly scattered multinucleated giant cells containing
between 10-100 nuclei. The stromal cells contain
nuclei resembling those of the giant cells.6 En bloc
excision is usually curative.
Giant cell granuloma, also known as giant cell
reparative granuloma, is a rare destructive lesion of
bone that presents with proptosis and globe
displacement. 18 Headache and pain may also be
present. In contrast to giant cell tumor, giant cell
granuloma is seen in younger patients (average age
18.6 years) and on histology, the giant cells are sparse
and unevenly distributed and reactive new bone
formation may be seen.6 This lesion responds well
to curettage.
Brown tumor is histologically almost
indistinguishable from giant cell granuloma.
Clinically, however, it is associated with primary or
secondary hyperparathyroidism. The increased
osteoclastic activity leads to focal areas of bone
resorption and hemorrhage. As treatment of
hyperparathyroidism usually results in spontaneous
healing of the bony lesion, it is important that a careful
clinical evaluation is performed in patients with
histology suspicious for brown tumor.19
Osteogenic Sarcoma
Osteogenic sarcoma of the orbit is rare and is seen in
the 4th and 5th decades of life in patients who have
usually undergone previous radiotherapy for
retinoblastoma or fibrous dyplasia.6 Primary orbital
involvement is exceedingly rare.20 It develops rapidly
over a period of weeks to months and can present
with proptosis, pain, diplopia and visual impairment.
Imaging shows a mixed lytic and sclerotic mass with
indistinct margins. Histology shows sarcomatous

cells in a stroma with foci of osteoid formation.6
Treatment is by preoperative chemotherapy followed
by resection and postoperative chemotherapy.
The prognosis for craniofacial lesions, however, is
poor.
Figures 13.3A to F: This 50-year-old male presented with a 6-month history of right proptosis. Examination (A) showed 2 mm proptosis with
3 mm inferior displacement of the right eye. Computed tomographic scan (B) revealed an osteolytic lesion involving the right superolateral frontal
bone. On magnetic resonance imaging, the mass showed high signal intensity on the T1-weighted image (C). The T2-weighted image showed
a similar appearance. Based on the clinical and radiological findings, a diagnosis of cholesterol granuloma was made. Anterior orbitotomy was
performed through an upper lid skin crease incision (D). A friable mass was seen protruding from beneath the superior orbital rim (arrow) and
the lesion was curetted out. The portion of the lesion behind the superior orbital rim and abutting the dura was removed using endoscopic
visualization. Histology (E) showed the characteristic cholesterol clefts (arrow) surrounded by a granulomatous inflammation. The patient
made an excellent recovery (F)
Chondrosarcoma
Myeloma
These are slow-growing, non-metastasizing locally

aggressive tumors commonly arising in the paranasal
sinuses and invading the orbit.13 Imaging reveals a
well-defined osteolytic lesion with internal stippling.
Multiple lobules of hypercellular cartilage with
binucleate cells in lacunae and mitotic figures are seen
on microscopy. Treatment is by radical resection but
complete removal may not be possible in the
craniofacial region and multiple recurrences may be
seen.13
Multiple myeloma or solitary plasmacytoma may

involve the orbit, usually in patients older than 50
years of age.23 Presentation is with subacute onset of
pain and proptosis. An osteolytic area with a
contiguous soft tissue mass is seen on imaging.
Histology shows sheets of malignant plasma cells.
The orbital lesions are treated with radiotherapy and
chemotherapy is used for systemic disease.
Mesenchymal Chondrosarcoma
This variant of chondrosarcoma has a predilection
for the head and neck region. It may occur in the
soft tissues of the orbit, almost exclusively in females
in the second and third decades of life.21 The clinical
course is rapid and patients present with proptosis
and infiltrative effects of less than a years duration.
Imaging shows a non-specific, irregular, mottled soft
tissue mass. On histology areas of poorly differentiated mesenchymal cells intermixed with lobules
of mature cartilage are seen. As metastasis, especially
to the lungs, can occur, exenteration is the treatment
of choice.21
Ewings Sarcoma
This is a small round cell tumor of bone mainly
affecting patients in the first two decades. Orbital
involvement is usually by metastases or spread from
adjacent areas (Ewings sarcoma is responsible for
10% of pediatric orbital metastasis). Primary Ewings
sarcoma of the orbit is exceedingly rare.22 Clinically,
a rapidly developing non-axial proptosis is noted and
imaging reveals expansile mass with bone
destruction. Microscopy shows a featureless small
round cell proliferation. PAS positive glycogen
granules may be seen in the cytoplasm. Immunohistiochemistry (for CD99) is helpful in making the
diagnosis. Treatment is by chemotherapy followed
by resection or radiotherapy.
Langerhans Cell Histiocytosis (LCH)

LCH results from an abnormal proliferation of
Langerhans cells- specialized histiocytic cells
normally seen in the epidermis and characterised by
racquet-shaped cytoplasmic granules (Birbeck
granules) on electron microscopy. One form of LCH,
previously known as eosinophilic granuloma,
preferentially affects the skull and presents as a
localized lytic lesion of bone. The disease is usually
seen in young males and the children classically
present with proptosis secondary to a superolateral
orbital lesion.24 On CT, a central radiolucent area with
an enhancing rim is seen. Histologically, a
granulomatous infiltrate with Langerhans cells and
prominent eosinophils is observed. The treatment
of choice is curettage, though intralesional steroid
injections and low-dose radiotherapy have also been
used. An endoscopic aided curettage may achieve
complete removal of the lesion.

Intraosseous Hemangioma
This is a rare vascular tumor which presents as a
slowly developing orbital mass, often associated with
pain or tenderness. 25 The frontal bone is usually
affected and on CT, a well-defined, radiolucent mass
that expands the inner and outer tables of the bone
is seen. Histologically, the lesions are hemangiomas
composed of thin-walled vascular spaces lined by
endothelium. Treatment is by surgical excision of the
lesion with a rim of normal bone. Preoperative
angiography should be performed.
Figures 13.4A to D: This 5-year-old boy presented with a 3-week history of pre-septal cellulitis involving the left upper lid that had failed to
respond to antibiotics (A). A computed tomographic scan revealed an irregular, osteolytic lesion involving the left frontal bone and extending
through the roof of the orbit into the anterior cranial fossa (B). This lesion was approached via an upper lid skin crease incision. The periosteum
was reflected off the roof of the orbit and endoscopic visualization was used to effect safe curettage of the entire lesion, including from areas
abutting the dura (C); long arrow: superior orbital rim; short arrow: dura). Histology (D) showed a polymorphic inflammatory infiltrate with
predominance of eosinophils, characteristic of Langerhans cell histiocytosis. Staging showed this to be unifocal, unisystem disease and he had
no further treatment. There was no recurrence of the disease after a 3-year follow up (Figure 13.4D courtesy of Prof T.Y. Khong, Adelaide)
CASE ILLUSTRATION (Figures 13.5A to C)
Figures 13.5A to C: A 57-year-old female presented with a one-year history of a mass over the left forehead that was gradually increasing
in size. She was otherwise asymptomatic. Examination revealed a bony mass over the left frontal-orbital region. Ophthalmic examination
revealed a 2 mm inferior displacement of the left globe. A computed tomographic scan showed a mass lesion within the left frontal bone. Intense
contrast enhancement of the lesion was seen (A). Magnetic resonance demonstrated intermediate T1 and high T2 signal intensity (B). Biopsy
of the mass was performed by the neurosurgeon and revealed an intraosseus cavernous hemangioma (C). Postoperative angiography
showed that the lesion was supplied by the ethmoidal branches of the left ophthalmic artery and by the left middle meningeal artery. The supply
was too small for embolization and the patient has been under observation for the last 3 years, with no further symptoms
REFERENCES
1. Rootman J, Chang W, Jones D. Distribution and differential
diagnosis of orbital disease. In: Rootman J, ed. Diseases of
the orbit, 2nd ed. Philadelphia: Lippincott Williams and
Wilkins, 2003;53-84.
2. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC.
Classification and incidence of space-occupying lesions of
the orbit: a survey of 645 biopsies. Arch Ophthalmol
1984;102:1606-11.
3. Selva D, White VA, OConnell JX. Primary bone tumors of
the orbit. Surv Ophthalmol 2004;49:328-42.
4. Henderson JW. Fibro-osseus, osseus, and cartilaginous
tumors of orbital bone. In: Henderson JW, ed. Orbital
tumors, 3rd ed. Philadelphia: Raven Press, 1994.
5. McNab AA,. Orbital osteoma in Gardners syndrome. Aust
NZ J Ophthalmol 1998;26:169-70.
6. Fu YS, Perzin KH. Nonepithelial tumors of the nasal cavity,
paranasal sinuses and nasopharynx: a clinicopathologic
study. II. Osseus and fibro-osseus lesions, including
osteoma, fibrous dysplasia, ossifying fibroma,
osteoblastoma, giant cell tumor, and osteosarcoma. Cancer
1974;33:1289-305.
7. Chen C, Selva D, Wormald PJ. Endoscopic modified lothrop
procedure: an alternative for frontal osteoma excision.
Rhinology 2004;42:239-43.
8. Katz BJ, Nerad JA. Ophthalmic manifestations of fibrous
dysplasia. Ophthalmology 1998;105:2207-15.
9. Jackson IT, Hide TA, Gomuwka PK, et al. Treatment of

cranio-orbital fibrous dysplasia. J Maxillofac Surg
1982;10:138-41.
10. Margo CE, Weiss A, Habal MB. Psammomatoid ossifying
fibroma. Arch Ophthalmol 1986;104:1347-51.
11. Leone CR, Lawton AW, Leone RT. Benign osteoblastoma
of the orbit. Ophthalmology 1988;95:1554-8.
12. Jepson CM, Wetzig PC. Pure chondroma of the trochlea.
Surv Ophthalmol 1966;11:656-9.
13. Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity,
paranasal sinuses and nasopharynx: a clinicopathologic
study. III. Cartilaginous tumors (chondroma, chondrosarcoma). Cancer 1974;34:453-63.
14. Arat YO, Chaudhry IA, Boniuk M. Orbitofrontal cholesterol
granuloma: distinct diagnostic features and management.
Ophthal Plast Reconstr Surg 2003;19:382-7.
15. McNab AA, Wright JE. Orbitofrontal cholesterol
granuloma. Ophthalmology 1990;97:28-32.
16. Selva D, Chen C. Endoscopic approach to orbitofrontal
cholesterol granuloma. Orbit 2004;22:49-52.
17. Ronner HJ, Jones IS. Aneurysmal bone cyst of the orbit: a
review. Ann Ophthalmol 1983;15:626-9.
18. Spraul CW, Wojno TH, Grossniklaus HE, Lang GK.
Reparative giant cell granuloma with orbital involvement.
Klin MonatsblAugenheilkd 1997;211:133-4.
19. Parrish CM, ODay DM. Brown tumor of the orbit. Arch
Ophthalmol 1986;104:1199-202.

20. Parmar DN, Luthert PJ, Cree IA, et al. Two unusual osteogenic
orbital tumors: presumed parosteal osteosarcomas of the
orbit. Ophthalmology 2001;108:1452-6.
21. Guccion R, Font R, Enzinger F, Zimmerman L. Extraskeletal
mesenchymal chondrosarcoma. Arch Pathol 1973;95:336.
22. Guzowski M, Tumuluri K, Walker DM, Maloof A. Primary
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23. Rodman HI, Font RL. Orbital involvement in multiple

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24. Jordan DR, McDonald H, Noel L, Nizalik E. Eosinophilic
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25. Relf S`J, Bartley GB, Unni KK. Primary orbital intraosseus
hemangioma. Ophthalmology 1991;98:541-7.
14
CHAPTER
Tumors of
Lacrimal Gland
Raman Mittal
Mass lesions of the lacrimal gland fossa account for

a substantial proportion of orbital space-occupying
lesions. These are mainly inflammatory, structural
and neoplastic lesions.
These lesions can be broadly classified as:1
Inflammatory Lesions
1. Infective
i. Bacterial
ii. Viral
2. Non-infective
a. Idiopathic
b. Specific
i. Sjogren's syndrome
ii. Sarcoidosis
iii. Wegener's granulomatosis
Structural Lesions
1.
2.
3.
4.
Epithelial Cyst (Dacryops)

Dermoid
Mucocele
Implantation cyst
Neoplastic Lesions
1. Epithelial neoplasms
a. Benign Epithelial neoplasms
i. Pleomorphic adenoma
ii. Oncocytoma
iii. Warthin's tumor
iv. Myoepithelioma
b. Malignant Epithelial neoplasms

i. Adenoid cystic carcinoma
ii. Carcinoma in pleomorphic adenoma
iii. Mucoepidermoid carcinoma
iv. Adenocarcinoma and ductal carcinoma
v. Low grade carcinoma
vi. Other rare neoplasms
Acinic cell
Epithelial myoepithelial carcinoma
Sebaceous adenocarcinoma
The main focus of this section will be a discussion of
common lesions.
Epithelial Cyst (Dacryops)

The term Dacryops may be used to mean any simple
cyst of the lacrimal gland, whether it is in the
palpebral or orbital lobe.2
Clinical Features
Dacryops characteristically occurs in young adults
or middle aged patients, as a unilateral or bilateral,
painless, non-tender, fluctuant mass in the forniceal
conjunctiva supero-temporally. Most cysts either
remain relatively stable or demonstrate slow
progression.
The diagnosis can usually be made clinically. It
may be difficult to differentiate a dacryops clinically
from a simple epithelial cyst of conjunctival origin,
which are commoner in nasal portion. It can be
differentiated from a dermoid cyst by the fact that
the latter is usually attached to bone.
Tumors of Lacrimal Gland 191
Pathology and Pathogenesis

Grossly, the classic dacryops is a round cyst that
contains clear fluid (tears) and is lined by epithelium.
The epithelium may consist of one or two layers of
relatively flat cells similar to those found in a lacrimal
gland duct, or it may be composed of nonkeratinizing
stratified epithelium with goblet cells similar to those
in the conjunctiva. Normal lacrimal gland tissue is
usually identified in the histologic specimen adjacent
to the cyst.
It is believed that a dacryops results from
obstruction of one of the secretory ducts of the
lacrimal gland.3 The obstruction results in progressive
dilatation of the duct with formation of a thin walled
cyst.
Management
When a dacryops is small and asymptomatic no
treatment is necessary. If it is larger and symptomatic,
it can be managed by simple aspiration. Aspiration
can lead to recurrence, so it is advisable to remove
the lesion surgically using either a conjunctival
approach or a lateral orbitotomy.
Prognosis
The prognosis for vision and life is excellent.
Complications associated with a dry eye may occur
if an excessive amount of lacrimal gland tissue and
duct is removed.
CASE ILLUSTRATIONS
Case 1
Mr V, 47 years male, presented with complaints of
recurring redness and itching in both eyes. The patient
had been diagnosed earlier as a case of allergic
conjunctivitis and was treated accordingly. He also
had small, round, non-tender, cystic lesions in the
lacrimal gland area on both the sides. So the patient
was diagnosed to be a case of dacryops in both eyes
and was advised excision. (Figures 14.1A and B).
Pleomorphic Adenoma
Pleomorphic adenoma is the most common benign
epithelial tumor of the lacrimal gland. Typically they
occur at a younger age (2nd-5th decades) than
malignant tumors.
B
Figures 14.1A and B: Mr V, with clear, cystic lesions
(Dacryops) in both eyes
Clinical Features
The characteristic presentation is of a slowly
progressive (more than a year), painless proptosis,
downward globe displacement and swelling of
the upper lid, unassociated with inflammatory
signs or symptoms. Larger tumors may indent the
globe and cause blurring of vision and may
cause diplopia. The common signs consist of
proptosis, usually non-tender, palpable mass in
the superotemporal quadrant, downward and
inward globe displacement and sometimes restricted
upgaze. Fundus examination may show globe
indentation in larger tumors and also choroidal
folds sometimes.
Imaging
Pleomorphic adenoma is best seen on CT scan.
Usually a well circumscribed, homogenous or
heterogenous, moderate to markedly enhancing soft
tissue mass lesion is seen in the area of lacrimal gland.
The scans may show pressure indentation over globe
and expansion of the lacrimal fossa, suggesting
chronicity of the lesion, in most cases. The mass may
have few hyperdense areas suggestive of calcification.
Ultrasound may reflect the histologic pattern with a
highly reflective pseudocapsule, cystic spaces, and a
well demarcated mass.

The pleomorphic adenoma of the lacrimal gland is
characteristically firm, grayish-white, encapsulated
and bosselated mass on gross examination.
Histologically, the tumor is composed of both
epithelial and mesenchymal elements. The epithelial
elements take the form of ducts, cords and squamous
pearls. The mesenchymal elements usually include
myxoid and chondroid tissue and sometimes osseous
tissue. The diverse patterns of two components
account for the name, pleomorphic adenoma. An
important feature is the presence of microscopic
nodular extensions into the pseudocapsule. This may
account for the tendency of the tumor to recur when
appropriate margins are not taken. The pathogenesis
is not clear. It appears that both the cellular and
stromal elements are derived from epithelial cells
lining the acini and ducts.
Management
If there is a strong clinical suspicion of the lacrimal
gland tumor being pleomorphic adenoma, on the
basis of slow growing lesion, and absence of pain,
motility disturbance and bony expansion, then it is
best to excise the tumor completely without capsular
rupture and without a prior incisional biopsy.
Incomplete excision or capsular rupture may lead to
a recurrence, sometimes with malignant transformation. Therefore, an incisional biopsy is probably
contraindicated if the diagnosis is strongly suspected
clinically.
The most appropriate approach is by a modified
lateral orbitotomy. The important aspects are wide
surgical exposure, excision of the periorbita, careful
manipulation of the tumor to avoid rupture, removal
of a margin or adjacent tissue, and where possible,

preservation of the uninvolved palpebral lobe
(reducing the incidence of postoperative filamentary
keratopathy).
Prognosis
The prognosis of the patient with pleomorphic
adenoma of the lacrimal gland is generally very
good. It is likely that greater attention to a complete
en bloc excision will decrease the chance of
recurrence and malignant transformation.
Case 2
Ms P, 20 years female, presented to me with
protrusion of the right eyeball for 1 year, associated
with reduced visual acuity. She had gradually
increasing non-axial proptosis of the right eyeball.
As you see in the figure (Figure 14.2A) the eyeball
was displaced down and in. I could palpate a hard,
non-reducible mass in the right supero-temporal
orbit. There was neither tenderness nor any sign of
inflammation. The mass was not pulsatile. The
extraocular motility of right eye was restricted in
upgaze, dextroelevation and dextroversion. Anterior
segment of both the eyes were within normal limits,
but the fundus of the right eye had folds of the
internal limiting membrane, suggesting indentation
of the globe by tumor.
Her CT scan (Figures 14.3A and B) showed a
fairly well defined orbital mass in the area of lacrimal
gland. The mass was indenting over the globe. No
bony erosions could be seen. The moulding of the
orbital wall contours suggested chronic and benign
nature of the lesion.
My clinical diagnosis was right lacimal gland
tumor, most probably a pleomorphic.
Adenoma: I did lateral orbitotomy to excise off
the tumor completely. Cryoextraction of the tumor
was done. It was a well encapsulated grayish white
mass measuring 2.6 2 1.5 cm. (Figure 14.4A). Gross
examination of the cut section showed grayish and
chalky white areas and also cystic areas filled with
mucin material.
Histopathologic examination (Figure 14.4B)
showed a dimorphic picture with epithelial and
stromal cells in close proximation. There were
glandular and dilated cystic spaces lined by double
Figure 14.2A: Ms P, Non-axial proptosis right eye
Figure 14.2B: Well aligned eyes postoperatively
Figures 14.2A and B: Clinical picture of Ms P. Preoperative picture (A) showing non-axial proptosis of the right eyeball. The
globe has been displaced downwards and inwards. Postoperative picture (B) showing that both the eyes are now well aligned
Figures 14.3A and B: Axial and coronal sections of the CT scan showing fairly well defined orbital mass in the area of lacrimal gland
A
Figure 14.4A: Picture of the excised lacrimal gland tumor
B
Figure 14.4B: Photomicrograph of pleomorphic adenoma (H and E stain)

layered cuboidal cells with secretions in the lumen.
Stroma consisted of basaloid cells with spindle
appearance, indistinct cell borders with elongated
vesicular nucleus with stellate pattern. There
was abundant myxoid matrix in between the stromal
cells.
These all histopathologic features confirmed the
diagnosis of Pleomorphic Adenoma of Lacrimal
Gland, right orbit. Postoperatively, proptosis
resolved completely (Figure 14.2B).
Case 3
Mr K, 50 years old male presented to us with severe
pain, swelling and redness of right periocular area
for 15 days. He gave the history of gradually
increasing painful protrusion of the right eyeball for
about a month for which he underwent evisceration
2 weeks back. On examination, I could palpate a
moderate to hard mass filling the supero-temporal
and temporal part of right orbit. The lids and adnexa
were inflamed with conjunctival chemosis (Figure
14.5A). CT scan orbit was done which showed a well
circumscribed, soft tissue mass in the supero-lateral
quadrant of right orbit (Figures 14.6A and B). The
mass was mildly enhancing on contrast. There were
no bony erosions. A disfigured globe (S/P
evisceration) was seen. So, now clinically, the
sequence of events that I thought was; the patient
had gradually progressing lacrimal gland tumor
(most probably pleomorphic adenoma) which lead
to severe proptosis with exposure keratopathy and/
Figure 14.5A: Preoperative picture of Mr K. Note the

superior sulcus fullness
or perforated corneal ulcer. The previous surgeon

had addressed the corneal complication by
performing evisceration, without tackling the
primary cause, i.e. lacrimal gland tumor.
I did lateral orbitotomy to excise off the tumor
completely. Cryoextraction of the tumor was
performed (Figure 14.7). Histopathologic examination of this well encapsulated tumor showed cells
arranged in dimorphic pattern. The background
showed myxoid stroma in between. These features
were consistant with Pleomorphic Adenoma of
Lacrimal Gland, right orbit. He was provided with
a prosthesis after the postoperative edema subsided
and the patient was comfortable (Figure 14.5B).
Adenoid Cystic Carcinoma

Adenoid Cystic Carcinoma (ACC) is the most
common malignant epithelial tumor of the lacrimal
gland. It has a tendency for bimodal occurrence i.e.
more common in 2nd and 4th decades.
Clinical Features
The most important feature is a short duration of
onset, i.e. usually few months. Generally patients also
complain of having persisting pain, usually associated
with paresthesia. Other clinical features include those
which are seen in other lacrimal gland tumors as well.
To enumerate, these are; a mass lesion in superotemporal quadrant, proptosis, downward and nasal
displacement of the globe, ptosis and decreased
visual acuity.
Figure 14.5B: Postoperative appearance of patient with

readymade ocular prosthesis
B
Figures 14.6A and B: Axial and coronal sections of CT scan orbit show a well defined mass
located at the lacrimal gland area of right orbit
may show the tumor as isointense to brain and

extraocular muscles.
Figure 14.7: Gross appearance of the lacrimal gland tumor (Pleomorphic adenoma) along with a bone piece from lateral orbital wall
Imaging
CT scan is the imaging modality of choice. An
important feature (that differentiates many malignant
lesion from a benign one) is bony erosions or
osteolytic changes in the adjacent bone. Other
features that help me to say that the lacrimal gland
tumor is Adenoid Cystic Carcinoma are irregular
margins of the lesion and its extension towards the
orbital apex. But on CT scan, the lesion may actually
appear to be a well defined, solid and fairly
homogenous and hence can be easily confused with
a benign lesion. Presence or absence of calcification
doesn't help much. In MRI, the T2 weighted images
On gross examination, adenoid cystic carcinomas are

usually grayish white, firm and may give a false
impression of being circumscribed or pseudoencapsulated. Histopathologically, ACC has solid
areas or cords of malignant epithelial cells. It can be
divided into several histologic subtypes:
1. Cribriform (Glandular or Swiss cheese) pattern: Shows
multiple lobules with many small clear spaces,
giving it a cribriform appearance.
2. Sclerosing variant: Consists of hyalinized
cylinders of connective tissue and elongated
epithelial cords, surrounded by a dense
hyalinized stroma.
3. Basaloid variant: Shows solid epithelial lobules
with large basophilic nuclei and scanty
cytoplasm that resembles the lobules seen in
basal cell carcinoma.
4. Comedocarcinoma variant: Shows epithelial lobules
with large foci of central necrosis.
5. Tubular (Ductal) variant: Is composed of
elongated and comma shaped epithelial tubules
lined by two or three layers of cells.
In practical terms, cribriform is the most common
pattern. Basaloid pattern is the least common but is
most aggressive and therefore has the worst
prognosis.
Management
Case 4
The mainstay of managing ACC remains complete

surgical excision with adjuvant radiotherapy and
chemotherapy. For very well defined lesions, local
excision with the adjacent bone removal should be
done; for not so well defined lesions, orbital
exenteration and for tumors that have extended into
the bone or soft tissues of the orbit, radical en bloc
orbitectomy by a multidisciplinary team should be
done. As it is difficult to ensure complete tumor
excision, surgery should be followed by adjuvant
radical external beam radiotherapy. This EBRT may
prevent or delay local recurrence.
The role of local, regional or systemic
chemotherapy remains unclear in what is essentially
an indolent tumor. We used a combination of
carboplatin or cisplatin with 5-fluorouracil or
doxorubicin in our patients as postoperative adjuvant
therapy. 4 Among all the treatment options,
chemotherapy has the greatest potential to eradicate
occult metastatic disease.
This patient Mrs B, 18 years old female, presented

to me with left blepharoptosis and mild
enophthalmos. She gave the history of orbitotomy
done elsewhere on left side for orbital tumor (as is
evident from eyebrow scar in the picture) (Figure
14.8), about 5 years back. Old CT scan report (CT
scan films were not available) suggested inhomogenous soft tissue mass in supero-lateral quadrant
of left orbit. There was orbital fossa formation
without bony erosions or intracranial extension. She
was not aware of previous diagnosis nor was any
histopathologic report available. I did Tarsofrontal
Sling (Silicon rod sling) for her and advised her for
CT scan orbits and regular follow up to rule out any
tumor recurrence in future (Figure 14.9).
Prognosis
What so ever we may do, the prognosis of adenoid
cystic carcinoma remains dismal. The usual clinical
course of ACC is painful local and regional recurrence
followed by distant metastasis, usually to the lungs.
Tumor can recur even at a very late date (even 20
years later). Most patients die within 5 years of
recurrence.
Figure 14.8: Clinical picture showing left eye enophthalmos with

ptosis status post lateral orbitotomy (Note eyebrow scar)
But the patient was lost to follow up for about

18 months, and therefter reported with massive
proptosis and exposure keratopathy which had subacute onset (Figures 14.10A and B). CT scan done at
this stage revealed a large heterogenous mass in the
supero-lateral aspect of left orbit (Figures 14.11A and
B). The mass was large enough to end just short of
orbital apex. Roof of the left orbit appeared eroded
at places but no obvious bony breach or intracranial
extension was seen.
Clinical and radiological findings suggested that
I was dealing with a recurrence of Lacrimal Gland
Tumor, and that too a malignant one. I sent a
Figure 14.9: Clinical picture 4 weeks after ptosis correction
B
Figures 14.10A and B: Patient presenting with massive proptosis and exposure keratopathy of left eye caused by a large lesion in
lacrimal gland area
Figures 14.11A and B: Axial and Coronal sections of CT scan orbit show a large heterogenous mass located at the lacrimal gland
area of left orbit, ending just short of orbital apex. Note the bony erosions caused by the lesion
small tissue for cytology which strongly favored the

diagnosis of Adenoid Cystic Carcinoma. Finally, I did
lid sparing orbital exenteration (Figure 14.12A)
and sent the specimen for histopathologic examination.
Histopathologic examination (Figure 14.12B)
showed tumor cells arranged in cribriform pattern
with abundant mucoid matrix in extracellular spaces.

The features were consistant with Adenoid Cystic
Carcinoma of lacrimal gland. The patient was
referred to an oncologist to rule out systemic
metastasis and give adjuvant radiotherapy and
chemotherapy.
B
Figures 14.12A and B: Exenterated specimen (A) showing cribriform pattern of adenoid cystic carcinoma (H and E stain) (B)
REFERENCES
1. Diseases of Orbit, Textbook, 2nd Edition, Rootman J,
Lippincott Williams and Wilkins, Chapter - Neoplasia, Page
344-45.
2. Bullock JD, Fleishman JA, Rosset JS: Lacrimal ductal cysts.
Ophthalmol 1986;93:1355-60.
3. Smith S, Rootman J: Lacrimal ductal cysts. Presentation

and management. Surv Ophthalmol 1986;30:245-50.
4. Muthy R, et al. Adenoid Cystic Carcinoma of the
Lacrimal Gland: Management and Outcome. (Unpublished
data).
Cystic Lesions of Orbit 199
15
Cystic Lesions of Orbit
CHAPTER
Golam Haider, Subrahmanyam Mallajosyula, Mohd Javed Ali
Cystic lesions of the orbit are not uncommon. A true

orbital cyst is any closed cavity or sac within the
bony orbital confines that is lined with epithelium
and contains a liquid or semisolid materials.1 The
cysts may be developmental or acquired.
Developmental cysts
Dermoid / Epidermoid
Cytic teratomas
Encephalocele
Congenital cystic eye
Perioptic hygromas.
Acquired cysts
Dacryops
Mucocele
Dacryocele or amniontocele
Hematic cyst
Simple retention cysts
Epithelial implantation cysts
Chocolate cysts
Parasitic cysts like cysticercosis and hydatid
cyst
Cystic degeneration of certain tumors like
lymphangiomas, optic nerve gliomas and
schwannomas.
Another classification proposed by Shields JA and
Shields CL2 for pediatric cystic lesions of the orbit is
as follows:
1. Cysts of surface epithelium: These are further

divided into:
Simple epithelial cyst (Epidermal,
conjunctival and apocrine gland cysts)
Dermoid cysts (Epidermal and conjunctival
cysts).
2. Neural cysts: Further divided as:
Those associated with ocular maldevelopment like congenital cystic eye and
colobomatous cyst.
Those associated with brain and meningeal
tissues like cephalocele and optic nerve
meningocele.
3. Secondary cysts: The most important secondary
cyst is mucocele occurring in children secondary
to cystic fibrosis.
4. Inflammatory cysts: These include parasitic cysts
like cysticercosis and hydatid cyst.
5. Noncystic lesions with cystic component: These
include certain tumors like adenoid cystic
carcinoma, rhabdomyosarcomas and lymphangiomas.
Parasitic encystment is more often an
inflammatory granuloma. Hematic cysts are not lined
with epithelium but with a fibrous encapsulation of
blood or blood products. Malignant epithelial
neoplasm that secondarily invades the orbit may
develop central necrosis.
Parasitic cysts is being dealt as a separate chapter
in this book. Cystic degeneration of tumors is
discussed in their respective chapters.
DERMOID AND EPIDERMOID CYSTS

Dermoid cysts are among the most common
periorbital tumors presenting in childhood 3,4
(Figure 15.1). Its, incidence is 33% of all orbital cysts.
Dermoid and Epidermoid cysts are choriostomas
that arise from subcutaneous epidermal rests or
epidermal tissues trapped along bony suture lines
during embryonic development.
These cysts are present congenitally and enlarge
progressively. The superficial cysts are symptomatic
in childhood but deeper dermoid may not be
clinically symptomatic till in adulthood. The
epidermoid enlarges and develops into a cyst lined
with stratified squamous epithelium, which usually
is attached to the fronto-zygomatic suture superotemporally or to the naso-frontal suture superonasally. If the cyst wall contains skin appendages such
as hair follicles, sweat glands or sebaceous glands,
the cysts are termed as dermoid cyst.5
If skin appendages are absent, the cysts are
termed as epidermoid cyst. 6 Preseptal orbital
dermoid cyst occurs most commonly in the area of
lateral brow adjacent to fronto-zygomatic suture.
Duane's experience is that these cysts have occurred
with equal frequency both nasally and temporally.
Medial lesions in the infant should be distinguished
from congenital encephaloceles or amniontoceles.
The mass is painless, smooth, ovoid to round in
shape and firm to rubbery in consistency (Figure
15.4). It is immobile, being relatively attached to the
periostium of the underlying suture. But it is not
Figure 15.1: A 9 months old female child with cystic swelling in

lower part of orbit
attached to the overlying skin. In adulthood dermoid

cysts may have a more posterior location. 7 The
posterior located cysts may be difficult to palpate.
Proptosis and globe displacement are common. Long
standing dermoid in the superior orbit may
completely erode the roof and become adherent to
duramater. Rarely an orbital cyst may pass through
bony sutureline to extend intracranially or into the
temporal fossa. Pressure placed on the extracranial
portion of such a bilobed cyst may be transmitted
through the bony dehiscence into the orbit and is a
cause for the mastication proptosis reported by
Bullock.8
Less commonly, orbital inflammation may be the
presenting sign due to leakage of oil or keratin from
the cysts.
Expansion of the dermoid cyst and inflammatory
response to leakage may results in an orbital
cutaneous fistula, usually following incomplete
surgical removal.
Investigations
CT scan may show a heterogenous lesion with rim
enhancement, calcification, fossa formation in the
bone (Figure 15.2), bone erosion, bone sclerosis and
intracranial extension.
MRI
Particularly important to see the cystic nature of the
lesion when the cyst present as an inflammatory
orbitopathy with surrounding tissue reaction.
Figure 15.2: CT scan of left orbit showing cystic space in

lower part of orbit
Treatment
Surgical excision is the treatment of choice. Superficial
cyst in childhood can be excised through an incision
directly over the lesion or preferably through upper
lid crease. A posteriorly located cyst may require
more careful planning for an approach through an
anterior or lateral orbital route9,10 (Figure 15.3).
Cysts located along the superior orbital roof with
intracranial extension needs neurosurgical consultations and possible transcranial approach.11,12 It is
important to keep the cyst wall intact during surgery.
Intraoperative rupture with release of its contents
may incite granulomatous inflammation. When an
inadvertent rupture occurs operating area must be
flooded with irrigating solution. Complete removal
of the cyst wall is curative (Figures 15.4 and 15.5).
Incomplete removal may lead to recurrence.9
Figure 15.4: Dermoid cyst after removal
TERATOMAS
Like dermoids teratomas are congenital tumor and
choristomas.13,14 Dermoid and epidermiod cysts are
developed from one germ layer but teratomas arise
from two or more germ layers, including ectoderm
and endoderm or mesoderm or both.15 Tumors may
present at any age, many at or shortly after birth.
Elsewhere teratomas are common in the gonads,
mediastinum and pineal area. More than 50 cases have
been reported in the orbit.16,17
Histologically, the tissues are usually matured
and consist of ectoderm represented by keratinized
Figure 15.5: Case 5 days postoperatively
squamous epithelium and adnexal glandular

structures; mesoderm by fibrous tissue, cartilage, fat,
muscle and or bone; endoderm by gastrointestinal
mucosa and glandular tissue and neuroectoderm by
mature brain.16,17
Indication for surgery is unacceptable cosmetic
appearance to the patient. One should preserve the
globe whenever possible.18 Incomplete removal leads
to recurrence. Exenteration is sometimes preferred
because of fear of malignancy.15
CEPHALOCELE
Figure 15.3: Inferior orbitotomy for complete removal of cyst
A congenital dehiscence in the bony cranium may

enable the meningeal tissue to herniate into the orbit
forming cystic structure filled with CSF an orbital
meningocele. If brain protrudes inside the sac the

condition is termed as meningo-encephalocele. It may
be fronto-ethmoidal or sphenoidal.19,20
Presentation is usually during infancy and
childhood. It may present with pulsatile proptosis.
Common site is superomedial aspect of the orbit. The
mass is soft in consistency. The size of mass is
increased by straining, bending forward or weight
lifting. The direction of proptosis is inferotemporal.
It may present with enophthalmos due to
compression of orbital contents.21
Following conditions may be associated with
encephalocele:22
1. Neurofibromatosis
2. Hypertelorism
3. Malar or orbital bone depression
4. Optic atrophy
5. Optic nerve coloboma
6. Optic nerve glioma
7. Microphthalmos
8. Morning-glory syndrome.
Radiological Finding
CT scan may show defect in the anterior cranial fossa.
3-dimensional coronal views can detect encephalocele
easily. When associated with sphenoid bone
dysplasia, enlargement of superior orbital fissure in
plain film appear as "Bare orbit". CT scan may reveal
an enlarged middle cranial fossa. The temporal lobe
of brain may herniate through the whole posterior
orbit. Enlargement of pituitary fossa and optic canal
may be associated with these bony defects.22
Treatment
Surgical treatment is indicated in cases of exposure
and lagophthalmos. Excision of small encephalocele
through transfrontal craniotomy may be done. For a
larger encephalocele, dural patching and bone
grafting can be done.22
MICROPHTHALMOS WITH CYST

Microphthalmos with orbital cyst result from the
failure of the choroidal fissure to close in embryo.
This condition may be unilateral or bilateral. The
presence of an orbital cyst may be beneficial for
stimulating normal growth of the involved orbital
bone and eyelid. The cyst is lined internally by gliotic
retina and externally by fibrous envelop. The cysts

are usually located in inferior orbit and cause the
lower lid to bulge. In almost all cases resulting eye is
defective, smaller than normal and has an attached
cystic mass at birth.23,24
The cyst may be smaller or larger than the eye.
Cyst is bluish in color and translucent, and may
displace the globe. In contrast congenital cystic eye
results from failure of optic vesicle to involute.
The eye is filled with both solid and cystic form of
dysplastic neuroglial tissue.25 The cyst is connected
to the brain by an astrocytic filled stalk, but it does
not communicate to the anterior ventricle.
Both microphthalmos with cyst and congenital cystic eye should be distinguished from
cystic teratoma and encephalocele by imaging
studies.26
Management of microphthalmos with cyst and
congenital cystic eye involves excision of the cysts
and or globe along with abnormal neuroepithelial
tissue.27 There has been a lot a work done on the use
of sclerosing agents in such cysts. Ethanolamine oleate
sclerotherapy may be an effective minimal intervention treatment option for cosmetic rehabilitation
of patients with orbitopalpebral cyst associated with
congenital microphthalmos with no visual
potential.28,29
Each lesion is self centered and often an orbital
implantation be placed and a prosthesis fitted to orbit
in a satisfactory cosmetic effect. Sometimes removal
of bone and reconstruction of the socket may be done.
MUCOCELE
Destruction of sinus ostium due to recurrent
inflammation, trauma or intensive mucosal disease
result in a mucous filled sinus or mucocele which can
expand slowly to involve the orbital cavity. If the
sinus is inflamed and the cyst contain pus or mucous,
the term pyocele and mucopyocele respectively,
apply. It may occur at any age, most common in
between (40-70) years. Mucocele from frontalethmoidal sinus are most common. The enlargement
of the mucocele is insidious, with proptosis and
displacement of the globe being manifest in
association with a palpable, smooth wall mass in the
upper and inner quadrant of the orbit. Mucocele of
the posterior orbit may present more insidiously with

a complaint of headache and orbital discomfort. The
mucocele arises in the sphenoidal sinus and
postethmoidal air cells and may mimic retrobulbar
neuritis, or cause ophthalmoplegia and ptosis by
affecting branches of 3rd cranial nerve along with
axial proptosis.30,31
The differential diagnosis of cystic medial orbital
lesion must also include encephalocele with skull base
deformity.
Appearance of a typical mucocele on a CT scan
includes bowing of the sinus wall into the orbit, with
attenuation or even erosion of bone with a cystic
cavity. MRI shows highly variable signal intensities.
Treatment of mucocele is surgical for reestablishment of normal drainage, removal of the
lining of the cyst. Frontal and ethmoidal mucocele
are approached sub-periosteally with elevation of an
osteoplastic flap32; eradication and extirpation of all
diseased mucosa is done. Successful endoscopic sinus
obliteration and or re-establishing sinus drainage
protects against recurrence. 33 Sphenoidal sinus
mucocele is approached intranasally. Neurosurgical
consultation is indicated for those mucoceles with
intracranial extension.34,35
CYSTS OF THE OPTIC NERVE SHEATH

Dilatation and expansion of the optic nerve sheath
has been observed in variety of neoplastic and nonneoplastic condition. Such a cystic growth in a optic
nerve sheath filled with CSF has been perioptic
hygroma, arachnoid cyst of the optic nerve sheath,
optic hydrops and meningocele.36,37 Most perioptic
hygromas present in patients between the ages of
30-60yrs with complaints of headache and visual
disturbance. Optic nerve signs are defective vision,
RAPD, optic disc edema, optic atrophy and
visual field defects. The cyst can be well delineated
in MRI with fat suppression technique and
gadolinium contrast.
HEMATIC CYST
Hematic cyst refers to the accumulation of
hematogenous debris within a cavity lined with
fibrous tissue but not epithelial or endothelial
tissue.40,41
It can occur at any age. It should be distinguished
from endothelial-lined blood containing cyst such as
chocolate cyst associated with lymphangioma, venous
varices and hemangioma. Hematic cysts are
uncommon. Proptosis, globe displacement, motility
disturbance may occur due to chronic hematic cyst
(Figure 15.6). Spontaneous eyelid ecchymosis and
edema may suggest this diagnosis. In acute cases
there may be decrease in vision, RAPD and choroidal
folds.42, 43
Hematic cyst may be due to trauma and an
incompletely reabsorbed orbital hemorrhage, wherein
the presence of old blood and blood breakdown
product incite a granulomatous inflammation and
fibrous encapsulation. Others believe that a cyst arises
from bleeding within the bony diploes that breaks
out into the peripheral space.44
Investigations
Plain film may show bony erosion when cyst arises
in the superior orbit and involve the orbital portion
of the frontal bone. CT shows well-defined nonenlarging mass (Figure 15.7) having the same density
as the brain. On MRI T1 and T2 weighted signal hyper
intensity are constant with blood.45
Treatment
Surgical evacuation of the cyst content, removal of
the fibrous wall lining and establishment of
meticulous hemostasis to prevent recurrence.
Removal of one wall of the cyst often is all that is
necessary and all that is attached to the orbital tissue
can be left intact (Figures 15.8 and 15.9).
Figure 15.6: A 15 year girl with cystic swelling in

the superior orbital region
Figure 15.9: Postoperative appearance 3 weeks after the operation
Simple Cyst
Simple cyst may include serous, retention and
implantation cyst. These are rare in the orbit but are
slightly more common in the eyelid and conjunctiva.
This cyst is lined by simple epithelium.
Figure 15.7: CT scan shows well defined mass in the orbital apex
Retention Cyst
It originates in the glandular appendages of the
conjunctiva and adnexal structure. Obstruction of the
orifice of the lacrimal gland or accessory lacrimal
gland from trauma or cicatrix may result in a thin
walled epithelium lined cyst in the superior fornix
that can cause mass effect.
Lacrimal Ductal Cyst
Figure 15.8: Surgical approach for removal of hematic cyst
It arises often in the supero-lateral orbit and can be

easily seen by everting the upper lid. These are
frequently bilateral,46 they present as round lesions
originating from the palpebral portion of the lacrimal
gland which protrudes into the superior fornix.
Marsupialization may be preferable to complete
excision, because attempt to excise the cyst may
unnecessarily close the remaining lacrimal ductal
cyst.47,48 Ductal cysts can rarely arise from accessory
lacrimal glands.49
Implantation Cyst
It arises from misplacement of surface epithelium into
the orbit as a result of trauma or surgery. It is difficult
to eradicate due to variable depth. Removal of the
entire cyst or electrodessication of the epithelial lining
effects a cure.50
DACRYOCELE
Distension of the nasolacrimal sac by entrapped
mucoid material results in the formation of a
dacryocele due to obstruction of the nasolacrimal
duct. A congenital dacryocele manifests at birth as a
firm bluish swelling in the medial canthal area at or
below the medial canthal tendon. Lacrimal sac is tense
and filled with mucous and dacryocystitis with or
without cellulitis may be a frequent presentation.51
Surgical indications are dacryocystitis, cellulitis,
recurrent dacryocystitis, difficulties in breathing in
large nasal cyst, failure to respond to conservative
treatment. Difficulties in diagnosis of congenital
dacryocele include - encephalocele, mucocele,
dermoid cyst, and capillary hemangioma. Congenital
dacryocele should be conservatively managed
initially and if there is no response probing should
not be delayed.52,53 Adult dacryocele needs dacryocysto-rhinostomy (DCR) surgery.53, 54
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16
Parasitic Cysts of Orbit
C HAP T E R
Subrahmanyam Mallajosyula, Mohd Ather, Modini Pandarpurkar
The prevalence of parasitic cysts of orbit varies greatly

in different regions of the world. For example
cysticercosis which is endemic in Asia, Latin America
and Africa,1 is rare in North America and European
Countries. Nearly 15% of proptosis we treat is due to
cysticercosis! Hydatid is the next common parasitic
cyst. However, in view of the wide spread
international travel, it is essential to know about these
conditions, as revealed by the fact that many cases of
neurocysticercosis were reported in USA.2,3
Cysticercosis: Cysticercosis is the larval form of
Taenia Solium. Man is the definitive host for Taenia
Solium. The larval form of cysticercosis occurs in
infested pig, which is the intermediate host.
Cysticercosis occurs in humans when they take
contaminated food like improperly cooked or
uncooked vegetables or through water.
Cysticercosis can involve many organs and subcutaneous tissues. Neurocysticercosis is very
common. Extraocular cysticercosis is more frequent
than intraocular. We see 40-50 new cases of myocysticercosis per year while my vitreo-retinal
colleagues see 4-6 cases a year. Most often we see
solitary cysts. Multiple cysts or association with
neurocysticercosis is rare.4,5 Very few cases of optic
nerve involvement are reported.6,7,8
The clinical presentation of orbital cysticercosis
is varied. The onset can be acute/sub-acute or
intermittent. Pain and swelling can vary from severe
to minimal. Ocular motility restriction and diplopia
are very common. FDT is positive. The horizontal recti
and superior rectus/LPS complex are commonly
involved. Most of the patients are young with an

average age of 16.5 years. Myocysticercosis is the most
common cause of acquired ptosis in the age group of
10-20 years in our series. A high degree of suspicion
is needed to investigate and diagnose this condition.
Anticysticercosis antibodies are positive in about 60%
of cases. CT scan or Ultrasound B scan of orbit9,10 reveal
enlarged muscle with a cyst showing a hyper dense
spot within (which represents scolex). We prefer CT
scan to B scan for the diagnosis, since we want to know
if there is associated neurocysticercosis.
If neurocysticercosis also coexists, we refer the
patient to neurologist. If there is no neurocysticercosis,
we treat the patient with oral Albendazole 15 mg/
kg.body.wt/ day in 2 divided doses for 4 weeks along
with prednisolone. In those with severe inflammatory
signs, we prescribe Prednisolone at a dose of 2 mg/
kg.body wt/ day and in others at 1 mg /kg.body wt/
day, and will taper over a period of 4 weeks. We follow
the patients with B scan, since it is less expensive and
easily available.
Awareness of the disease, high index of suspicion
and imaging are necessary to diagnose this condition.
When the disease is for several months, and is not
diagnosed and treated properly, the cyst migrates
anteriorly . It can be seen in subtenons space, usually
in relation to an extraocular muscle. Such cysts are
excised, which on histopathology show a highly
convoluted membrane with scolex. These patients are
investigated for the presence of cysticercosis elsewhere
and treated acoordingly.Rarely spontaneous extrusion
may11 occur.
When to suspect Myocysticercosis?

Young patient (5-20 yrs)
Stay/visit to endemic region
Onset: Acute/Subacute/Intermittent
Pain: Severe/moderate
Proptosis: Mild to moderate and eccentric
Ocular motility: Restricted, FDT positive,
Diplopia common
Soft tissue changes: Severe/minimal.
Investigations
Serum Anticysticercosis: Positive in about 60% cases
only.
Imaging: CT/MRI preferred; Enlarged extraocular
muscle with a cyst and scolex.
in short time, symptoms not in proportion to the signs,

myocysticercosis was our clinical diagnosis. Plain CT
scan of Orbits revealed enlarged LPS-SR complex with
a cyst and scolex (Figures 16.3 and 16.4), confirming
our clinical diagnosis.
She was treated with Albendazole 400 mg bid for
4 weeks along with Prednisolone 90 mg/day (Her wt.
was 45 kg) Since the inflammation was fairly severe
as evidenced by severe edema of lids and markedly
enlarged. LPS-SR complex. We started her on
Prednisolone at 2 mg/kg.body wt./day and tapered
over a months time. The response was quite dramatic
as you can see in the Figure 16.5 which was taken 1
week after starting the medication.
Treatment
Albendazole: 15mg/kg body wt/day in 2 divided doses
4 weeks along with Prednisolone 1-2 mg/kg body
wt/ day, tapered over 4 weeks.
Figure 16.1: Miss S, F16 presenting with severe edema of lids and
swelling extending all round into periocular region of right eye
CASE ILLUSTRATIONS
Case 1
Miss S, an young girl of 16 years in age was referred
to us with the history of pain and swelling of right
eye since 2 days. She had a similar episode 2 weeks
back for which the referring doctor treated her with
systemic antibiotics and she responded.
As you could see, (Figure 16.1) she presented with
marked swelling of both the lids of her right eye, upper
lid being more severe. She complains of pain of
moderate intensity. There is a slight raise of local
temperature, mild tenderness, minimal chemosis, mild
proptosis and normal ocular motility (Figure 16.2).
We felt the chances of orbital infection are remote
since it is difficult to explain why an infection recurs
in such a short time in an otherwise healthy young
woman. In view of the age, intermittency/ recurrence
Figure 16.2: On elevating the eye lid, the eye looks normal
but for mild chemosis and minimal proptosis
Figure 16.3: Axial CT image shows enlarged

LPS-SR complex enclosing a cyst
Parasitic Cysts of Orbit 209
Figure 16.4: Coronal CT image showing a markedly enlarged LPSSR complex enclosing a cyst. The scolex( hyper-dense spot) within
the cyst can be easily made-out
muscle. It is not necessary to remove the cyst along

with the casing. We prefer to de-roof the surrounding
casing. Live cysticercus cyst usually shows motility.
In fact, most often, it tries to come-out after an incision
is made in the casing and tries to wriggle-out! These
movements can be very well appreciated with the
operating microscope.
Normally there is no need to use a cryo for the
extraction of the cysticercus, unlike in hydatid cyst,
wherein the use of cryo is mandatory. The excised cyst
showed a dense spot which was the scolex
(Figure 16.9).
Histopathology revealed convoluted membrane
(Figure 16.10)
Figure 16.5: One week post treatment, the edema has almost subsided, the chemosis disappeared and proptosis reduced. There is a
very marked clinical improvement
Case 2
Master V, a boy of 8 years presented with a swelling
of right lower lid of 4 months duration, associated with
mild pain. He gives history of occasional episodes of
dull retrobulbar pain for which he did not consult any
doctor.
On examination a cystic lesion, 15 mm 12 mm
in size and involving lower eyelid of right eye was
noticed (Figure 16.6). It is getting more prominent
when the child is looking up, and less prominent when
the child looks downwards, demonstrating its relation
to inferior rectus.
Transillumination was positive (Figure 16.7). The
eye was very quiet. Ocular motility was normal CT
scan of orbit revealed cysticercosis cyst with scolex
(Figure 16.8). Since the cyst is anteriorly located, and
is very easily accessed, it was excised through
conjunctival approach. We found that most often the
cysticercosis is encased in a wall which is about 2 mm
thick and is very closely related to the extraocular
Figure 16.6: Large cyst involving right lower lid
Figure 16.7: Transillumination of the cyst
Case 3
Figure 16.8: CT Orbits showing cyst with scolex
Baby H, a girl of 8 years presented with painful

eccentric proptosis of left eye of 2 weeks duration. On
examination fullness in supero-medial space with the
globe pushed down and out and severe tenderness
was present. Abduction was restricted (Figure 16.11).
In view of short duration, pain and inflammatory
signs, inflammatory /infective etiology was
suspected.
CT scan of the orbit revealed sub-periosteal
abscess with a cystic lesion within (Figure 16.12).
Careful examination revealed a white spot within
it representing scolex (Figure 16.13).
The subperiosteal abscess was drained
through superior Lynch incision. Along with pus,
a cysticercosis cyst also was removed (Figures 16.14
and 16.15). It is the first and the only case of
cysticercosis involving the subperiosteal space that we
encountered. It is one of the rarest presentations of
Figure 16.9: Excised cyst showing hyper-dense spot
Figure 16.11: Eccentric proptosis with fullness and

inflammatory signs
Figure 16.10: Microphotograph showing convoluted membrane
Figure 16.12: CT scan showing subperiosteal abscess with a cyst
Figure 16.16: Microphotograph showing

cysticercosis with suckers
Figure 16.13: Cyst with scolex
Figure 16.14: Incision mark on first postoperative day
Figure 16.17: One month postoperative picture showing normal

restoration of movement
cysticercosis and the only one to the best of our

knowledge and pub med search. The diagnosis was
confirmed by histopathology (Figure 16.16).
The postoperative recovery was uneventful. The
ocular motility was restored (Figure 16.17). Very faint
incisional scar can be made out.
Case 4
Figure 16.15: Excised cysticercosis
Mrs V, female 32 years, presented with sudden

diminution of vision of her right eye since 2 weeks,
associated with mild pain. Examination revealed mild
fullness of superior sulcus, RAPD, and normal ocular
motility. Optic disc was normal. Vision was 20/400
(Figures 16.18, 16.19 and 16.20).
The possibility of retro bulbar neuritis was thought
of. But to exclude any other pathology, CT imaging
of orbit was performed which surprisingly revealed

cysticercosis involving the lateral rectus, abetting optic
nerve at orbital apex (Figures 16.21 and 16.22). This is
an unexpected diagnosis, since cysticercosis is rare
after 20 years, and presenting like retro bulbar neuritis
is very unusual.
She was treated with oral Albendazole(15 mg/kg.
body wt. in 2 divided doses /day) for 4 weeks and
Prednisolone at a dose of 2 mg/kg.body wt./day
tapered over a period of 4 weeks. This high dose of
prednisolone was used because of associated optic
neuropathy.
The patient recovered very well. The patient's
periocular fullness disappeared. The pain subsided
(Figure 16.23). Her vision improved to 20/30. How
ever she had a inferior arcuate scotoma (Figure 16.24).
CT imaging after 4 months showed the presence
of a shrunken cyst and scolex could not be seen
Figure 16.18: Shows mild periocular fullness. Her vision was 20/
400. Pupil was dilated and sluggish with a significant RAPD
(Figure 16.25), which means that the cyst died. The

patient is doing well for the past 2 years.
This is a unique case scenario. Cysticercosis very
rarely causes a toxic optic neuropathy. It is rare after
25 years of age. In fact at the age of 32 years, this
patient is the oldest patient of cysticercosis we have
come across so far. We never suspected the possibility
of cysticercosis in this patient, but the hunch to get a
CT helped in making correct diagnosis and provide
correct treatment for this lady. We could restore her
vision.
This case clearly illustrates that even those who
regularly come across cysticercosis can also miss a
case, unless they are extra careful.
Figure 16.21: Cyst with hyper dense spot representing scolex,

involving lateral rectus at the orbital apex
Figure 16.19: Showing normal abduction
Figure 16.22: Cyst abetting the optic nerve
Figure 16.20: Showing normal adduction
Figure 16.23: Subsidence of periocular edema,

recovery of vision to 20/30
Figure 16.24: Visual fields showing inferior arcuate scotoma
Figure 16.25: CT after 4 months shows a cyst which is shrunken

and does not show the scolex
Case 5
Master R, a boy of 8 years presented with pain,
proptosis, drooping of left upper eye lid, since 4 days,
associated with headache and nausea and diplopia.
There was no history of convulsions, vomiting or
fever (Figure 16.26).
On examination, periocular fullness, more on
superior quadrant with eyeball pushed down,
minimal congestion of conjunctiva, and restricted
ocular motility in vertical gaze leading to vertical
diplopia were evident (Figure 16.27). The vision was
20/20, pupil was normal and optic disc showed mild
hyperemia with filling up of the cup. There were no
hemorrhages. Clinical diagnosis of myocysticercosis

involving LPS-SR complex was made because of
patients age, acute onset, with inflammatory signs,
acquired ptosis, mild proptosis, and restricted ocular
motility. In view of headache, nausea and early disc
edema, the coexistence of neurocysticercosis was
suspected.
On imaging, of the orbit, (Figures 16.28 and 16.29)
a markedly enlarged LPS-SR complex was noted.
A large cystic lesion was seen in relation to the
thickened muscles. Scolex was made out in the cyst.
Imaging of the brain confirmed our suspicion. It
revealed the presence of a calcified cyst in the brain
parenchyma (Figure 16.30) with normal architecture
of the surrounding brain. Typical " Coin Lesions" with
surrounding edema of brain was noted in other parts
(Figure 16.31). A total of 3 such active cysts were noted
in the serial sections of the brain. The surrounding
cerebral edema indicated that the cyst is live and
active. Anticysticercosis antibodies were positive at
1.68 od units, as against the values of > 0.5 od units
taken as positive. Most of the patients of
neurocysticercosis present with convulsions. The
unique features of this case are (1) absence of
convulsions, (2) multiple cysts in the brain, three are
active and one is inactive, association of orbital and
neurocysticercosis.
In view of associated neurocysticercosis the child
was referred to neurologist who treated him with
Albendazole, Prednisolone and Carbamazepam. The
child recovered well. Six weeks after initiating the
treatment, the ptosis disappeared, and normal ocular
motility was restored.The optic disc edema subsided
completely. There was no recurrence during the last
2 years of follow up (Figure 16.32).
Figure 16.26: Periocular swelling, ptosis, mild eccentric proptosis

with the globe pushed down in a child of 8 yrs. Onset was acute
Figure 16.27: Grossly restricted elevation of left eye, leading

to vertical diplopia
Figure 16.30: Coronal CT showing enlarged LPS-SR complex in the

orbit and a calcified mass in the brain parenchyma. Note that there is
no surrounding edema
Figure 16.28: CT showing a large cystic lesion in relation to

LPS-SR complex
Figure 16.29: Scolex in the cystic lesion
Figure 16.31: Axial imaging of brain shows the presence of typical

coin lesion with very severe edema surrounding the lesion. 2 more
similar active lesions were seen in other sections of imaging
Figure 16.32: 6 weeks post-treatment, the child recovered well from periocular swelling ptosis and proptosis
Case 6
We wish to share our experience with a unique clinical
experience.
Mr. S 18 years presented with a swelling on his
left eye of 4 days duration. He is a college student,
doing his graduation. He gives history of mild pain
and discomfort. There is no history of defective vision
or diplopia. He noticed mild drooping of his left upper
lid since 1 week, but did not pay attention due to his
approaching examinations. However he noticed this
morning that the swelling has enlarged and the
discomfort has increased in intensity. His food habits
include eating road-side junk food, raw vegetables and
green salads.
On examination (Figure 16.33) but for very
minimal fullness and 1 mm of ptosis of his left
upperlid,the rest looked normal. Ocular motility was
normal (Figures 16.34 and 16.35).
On elevating the upper lid, a cyst measuring 12
cm 10 cm, and in relation to superior rectus was
seen. Surrounding conjunctiva was congested. This is
the typical appearance of cysticercosis in the sub-
Figure 16.35: The ocular motility was normal. The elevation as you
can see here is full. He is orthophoric on cover test
Figure 16.36: Spontaneously extruded cysticercosis in a container

Figure 16.33: Anterior segment looks almost normal
tenon's space. The cyst is always in relation to an extraocular muscle. Most often the surrounding
conjunctiva shows congestion. Since the cyst was very
anteriorly placed, excision was planned and Mr. S was
advised to come after 2 days to the operation room
for excision of the cyst under local anesthesia. He
attended the operation room after 2 days, carrying a
small bottle in which he placed something that came
from his affected eye that morning (Figure 16.36).
To our surprise the bottle contained cysticercosis
cyst. This is an example of spontaneous extrusion.
Case 7
Figure 16.34: Note the cyst in relation to superior rectus muscle.

Surrounding conjunctiva was congested
We are to share with you a very rare clinical

presentation of cysticercosis, which we came across
only once so far. The patient, master D a young boy
of 12 years, was a student of class 7, and was from a

village, 80 miles away. He was brought with a history
of sudden loss of vision of his left eye since 2 weeks.
He complained of dull retro bulbar pain of 2 months
duration. There was no history of trauma, fever or
any other symptoms of systemic illness.
On examination, (Figure 16.37) there was mild
proptosis of his left eye, which was more evident on
Nafzeigers (Figure 16.38). The ocular motility was
normal. The pupil was dilated with a very significant
RAPD. The vision was reduced to PL only.
Retropulsion was mildly positive.
Fundus Examination revealed optic disc which
shows nasal hyperemia, and gross pallor on the
temporal side, with exudates, macular fan and
degenerative changes involving macula suggestive of
neuroretinitis (Figure 16.39).
This child was referred to us in view of proptosis
of his left eye. The association between proptosis and
neuroretinitis is very unusual and difficult to explain.
The fundus picture was more like a neuroretinitis
rather than disc edema.
We discussed about the possibilities of a fungal
infection extending from the sinuses with a vascular
involvement, which could explain proptosis and
sudden acute fall of vision (which is due to retinal
vascular obstruction) and could not explain

neuroretinitis.
The possibility of cysticercosis was also discussed,
but we were not for it as we did not come across such
a clinical presentation.
Imaging by CT scan of orbit showed a large cyst
in relation to the optic nerve and scolex could be very
clearly seen (Figures 16.40 and 16.41).
Figure 16.39: Severe macular changes including macular fan,

exudates and pallor of optic disc
Figure 16.37: Presents with sudden loss of vision of his left eye
Figure 16.40: Axial imaging of orbit shows a cyst in relation to optic

nerve. Scolex is seen very clearly
Figure 16.38: Proptosis of left eye is better appreciated

by Nafzeiger's test
Figure 16.41: Cyst involving optic nerve. Scolex could be seen in

the center of the cyst

Anticysticercosis antibodies were positive
which confirms the diagnosis of cysticercosis of optic
nerve. The associated neuroretinitis was due to the
severe inflammatory response produced by the toxins
released, which was common with cysticercosis.
The child was treated with oral Albendazole and
Prednisolone. Surgical excision of the cyst through the
antero-lateral orbitotomy was not considered in view
of the severe irreversible damage already occurred,
and the very poor visual prognosis. With treatment,
proptosis disappeared, but his vision was absent PL.
Hydatid Cyst of Orbit

Hydatid cyst is another parasitic infestation of the
orbit, less common than cysticercosis. It is caused by
echinococcus granulosus or the dog tapeworm. Dogs
are the primary hosts with sheep and cattle being
intermediary hosts. Humans are accidental hosts,
acquiring infection by ingesting ova along with raw
vegetables, contaminated water, or from direct contact
with dogs. Embryos pass across duodenal mucosa to
liver through the portal veins. Liver, lungs, and brain
are primarily affected. Orbital disease is seen in only
1% of hydatid disease.12 Hydatid cysts form 0.3-5% of
all orbital diseases.13,14
Hydatid infestation commonly presents in
children and young adults. Most of the patients are
below 16 years.13 Rarely elderly people are affected
by this disease.
Proptosis is the most common presentation of
orbital hydatidosis.14 Usually, the proptosis is of a few
months duration, associated with mild pain or
discomfort. Hydatid cysts are commonly located in
the intraconal space. In our experience it is the most
common cystic lesion of intraconal space. Since
intraconal space is the most common location, it can
present with defective vision associated with RAPD
and optic disc edema. Other causes of defective vision
in hydatid cyst we came across include refractive
errors and corneal perforation due to exposure
keratitis in one case. Other symptoms can be
periorbital pain, chemosis and headache.12 Diplopia
due to ocular motility restriction can be a rare
symptom. 15,16 In neglected and unattended long
standing cases, these cyst grow to a very large size,
deforming the globe (Figure 16.42).17 Diagnosis is by
a high index of suspicion especially in endemic areas,
and investigations like CT scan orbit. Sometimes
Figures 16.42A and B: CT Scan of orbit showing a well encapsulated, intraconal Hydatid cyst (A) Severe proptosis and distorted globe
with anterior staphyloma in a neglected case of Hydatid disease (B)
orbital hydatid may be a part of disseminated

disease,18 with lung, liver and brain being commonly
involved and hence in every case of hydatid cyst,
ultrasound of abdomen and CT scan of chest and brain
were advised.18,19
Investigations
CBP- usually normal
Stool examination does not show ova or cysts
Casoni's intradermal test is positive in 75% of the
people13
CIEP- Counter immunoelectrophoresis
Imaging techniques of importance are orbital
ultrasonography and CT scan.
On ultrasound, diagnostic double wall sign is
confirmatory, spoke wheel pattern and water lily sign
are seen with cyst calcification.
CT scan shows well encapsulated cystic mass with
cyst fluid showing attenuation values of 3-30 HU. The
mass indents and deforms the globe. Calcification of
the internal septa may be seen. We prefer CT scan of
the orbit.
Microscopic analysis of the cyst fluid shows
scolices and hooklets. The cyst wall is laminated and
has the characteristic "coats of an onion" appearance.
Management: We prefer surgical excision of the
cyst by performing orbitotomy . The cyst wall is very
thin and can rupture during surgery. Hence, to
prevent it, after exposing the cyst, we prefer to aspirate
the contents, so that the cyst shrinks in size. Then it
can be very safely pulled out with the help of a cryo.
Akon O. et al from Turkey 20 have advocated
percutaneous aspiration of the cyst under ultrasonic
guidance, followed by injection of 15% of hypertonic
saline and reaspiration (PAIR technique). They
showed a decrease in cyst size by three months and a
marked decrease to 0.5 ml. by 9 months.
Medical management of hydatid cysts and
recurrent cysts with Albendazole and Praziquantal
has also been tried.21
CASE ILLUSTRATION
Figure 16.43: Male 35 years presented with proptosis of right eye of 2

years duration with mild pain and discomfort. Note the eccentric
proptosis with the globe pushed down and out. Note also the fullness
of superior sulcus. His BCVA was 20/80
Figures 16.44A to C: CT scan of the orbit shows a very large cyst in the superior peripheral space, pushing the globe down and out
Figures 16.45A to C: The cyst was exposed through superior lid crease incision. Note the tense cyst, as it is protruding-out (A). About 10 ml of
the fluid was aspirated, and the collapsed cyst was removed with a cryo(B) The diagnosis of hydatid cyst was confirmed by the laminar / onion
peel appearance
Figure 16.46: First postoperative day picture, showing clinical

improvement
Note the position of the globe which has come back to normal. Also there is marked improvement in the fullness of superior sulcus. Patient's
vision improved to 20/30 in course of time
REFERENCES
1. Del Brutto OH. Neurocysticercosis: Updating in diagnosis
and Treatment Neurolgia, 2005;20(8):412-8.
2. Del la Garza Y, Graviss E A, Daver NG,Gambarin KJ,
Shandera WX, Schanz PM, White AC Jr : Epidemiology
of Neurocysticercosis in Houston, Texas Am J Trop. Med.
Hyg.2005;73(4):766-70.
3. Towas JM, Hoffmann CJ,: Neurocysticercosis in Oregon:
1995-2000 Emerg. Infect Dis 2004;10(3):508-10.
4. Pushker N, Bajaj MS, Balasubramanya R: Disseminated
Cysticercosis involving Orbit, brain and sub-cutaneous
tissueJ. Infect. 2005;51(5)e245-8.
5. Chadha V, Pandey PK, Chauhan D, Das s: Simultaneous
intraocular and bilateral extraocular Muscle involvement
in a case of disseminated Cysticercosis Int.Ophthalmol
2006;15.
6. Gulliani BP, Dadeya S, Malik KP, Jain DC : Bilateral
Cysticercosis of optic nerve J Neurophthalmol 2001;21
(3):217-8.
7. Bajaj MS, pushker N: Optic nerve cysticercosis Clinical
Experimental Ophthalmol; 2002;30(2);140-3.
8. Sudan R, Muralidhar R, Sharma P, Optic Nerve
Cysticercosis: case report and review of current
management Orbit, 2005;24(2);159-62.
9. Sekhar GC, Honavar SG, Myocysticercosis Experience
with imaging and therapy Ophthalmol, 1999;106(12)
2336-40.
10. Honavar SG, Sekhar CG, Ultrasonological Characterestics
of Extraocular Cysticercosis Orbit 1998;17(4),271-84
11. Bansal RK, Gupta A,Grewal SP, Mohan K, Spontaneous
extrusion of cysticercosis: Report of three cases, Indian J
ophthalmol,1992;40(2):59-60.
12. Turgut AT, Turgut M, Ko?ar U.: "Hydatidosis of the orbit

in Turkey: results from review of the literature 1963-2001"
Int Ophthalmol. 2004;25(4):193-200.
13. Xiao A, Xueyi C: "Hydatid cysts of the orbit in Xinjiang: a
review of 18 cases", Orbit. 1999;18(3):151-55.
14. Gomez Morales A, Croxatto JO, Crovetto L, Ebner R;
Hydatid cysts of the orbit. A review of 35 cases,
Ophthalmology. 1988;95(8):1027-32.
15. Kiratli H, Bilgi S, Oztrkmen C, Aydin O: Intramuscular
hydatid cyst of the medial rectus muscle, Am J Ophthalmol.
2003;135(1):98-9.
16. Jhn lCrompton, Prema V Iyer, David J Merry John Tomich,
Llance V Perrett "Hydatid cyst: an unusual cause of
diplopia" Australian and New Zealand Journal of
Ophthalmology 13 (2), 195-203.
17. Rastogi A, Arora R, Chaturvedi K. Orbital hydatid cyst: an
unusual presentation", Orbit. 1998;17(2):107-111.
18. Betharia SM, Pushker N, Sharma V, Avinash M, Kashyap
S: "Disseminated hydatid disease involving orbit, spleen,
lung and liver". Ophthalmologica. 2002;216(4):
300-4.
19. Andronikou S, Welman CJ, Kader E: "Classic and unusual
appearances of hydatid disease in children" Pediatr Radiol.
2002;32(11):817-28.
20. Akhan O, Bilgi S, Akata D, Kiratli H, Ozmen MN:"
Percutaneous treatment of an orbital hydatid cyst: a new
therapeutic approach" Am J Ophthalmol. 1998;125(6):
877-9.
21. Sihota R, Sharma T:Albendazole therapy for a
recurrent orbital hydatid cyst Indian J Ophthalmol.
2000;48(2):142-3.
17
Orbital Fractures
CHAPTER
Alon Kahana, Mark J Lucarelli, Cat N Burkat, Richard K Dortzbach
INTRODUCTION
The surgeon encounters orbital fractures in the acute,
subacute and chronic settings. In each situation, the
treatment goals are similar-restoration of orbital
integrity and volume. However, the nuances of
treatment can vary widely according to the specific
setting and injury. In this chapter, we will attempt
to provide an overview of how we approach orbital
fractures in a variety of settings, and provide enough
reference materials to satisfy the need for additional
study.
as part of La Forte II or III fractures. Fractures

involving the buttresses typically present with larger
displacements. Alternatively, when the buttresses are
intact, trapdoor-type fractures are more common.4
The anatomic landmarks of most fractures
correlate with the bony anatomy (Figure 17.2). Floor
ANATOMY
The adult human orbit has a volume of approximately
30 ml, of which the globe accounts for approximately
7 ml, or about 25%. 1 It traditionally is said to be
formed of 7 bones: maxillary, zygomatic, frontal,
lacrimal, ethmoid (lamina papyracea), palatine, and
the sphenoid, although the greater and lesser wings
of the sphenoid develop independently during
embryogenesis [the alisphenoid (greater wing) and
orbitosphenoid (lesser wing) bones].2 The optic canal
is part of the lesser wing of the sphenoid.
Orbital bony strength is dependent on a series
of dense bony buttresses that provide structural
integrity and create a protective frame around the
eye. Anteriorly are the frontomaxillary and
frontozygomatic buttresses. Posteriorly is the
pterygomaxillary buttress (Figure 17.1).3-9 Orbital
fractures can be classified as blow-out fractures (no
rim involvement), and fractures that involve the rim
Figure 17.1: Orbitomaxillary buttresses. Nasomaxillary (medial),

zygomaticomaxillary (lateral). Based on Gruss et al., 1986.
Diagrammatic representation of the maxillary buttresses showing the
two anterior buttresses (medial or nasomaxillary and lateral or
zygomaticomaxillary) and the posterior buttress (pterygomaxillary).
The relationship of these buttresses to the cranial base above, the
mandible below and the correct occlusion is seen
Orbital Fractures 221

fractures often extend up to the infraorbital groove
and/or canal, since the thin bone of the floor abuts
the stronger bone of the canal. Posteriorly, floor
fractures nearly always leave the most posterior
portion intact since it is part of the large and strong
palatine bone. Medially, floor fractures often leave
intact the inferomedial strut, a part of the
frontomaxillary buttress.10, 11 Medial wall fractures
often end at the frontoethmoidal suture line: the
lamina papyracea is very thin, whereas the frontal
bone is thick and supported by the cribriform plate.
Lateral wall fractures often occur as part of a
complex fracture involving the zygomatic arch and
the maxillary bone.
Orbital nerves and vascular bundles can often
be involved in orbital fractures, and serve as
important landmarks (Figure 17.2). Since the
infraorbital nerve often abuts the floor fracture edge,
it is commonly contused by the trauma but rarely
severed. Hence, hypoesthesia in the V2 distribution
of the trigeminal nerve is very common following
orbital trauma, but such numbness typically resolves,
at least partially, several weeks to months after injury
unless surgical repair causes further damage. Just
posterior to where the infraorbital groove and canal
meet, a perforating branch of the infraorbital artery
is often encountered, which can cause significant
bleeding if not isolated and cauterized in the course

of surgical repair.12
The zygomatic bone contains foramina for both
the zygomaticofacial and the zygomaticotemporal
nerves, branches of the V1 division of the trigeminal
nerve. Overall, the area innervated by these nerves
is small, and patients often tolerate hypoesthesia
associated with injury to these nerves, which may
occur from surgery as well as from the initial injury.
Injury to the infratrochlear, posterior ethmoidal
and/or anterior ethmoidal neurovascular bundles are
uncommon, but can be associated with significant
bleeding. Superomedial orbital injury may also be
associated with damage to the trochlea, causing
torsional diplopia.
Orbital fractures can often cause ocular dysmotility. There are several general etiologies in the
acute setting: direct muscle damage and/or edema,
nerve damage, or muscle entrapment. The restriction
caused by muscle entrapment often involves the
orbital fibrous connective tissue complex, of which
the extraocular muscle pulleys and septa are a part.13,14
Hence, herniation and entrapment of orbital
connective tissue that is associated with an
extraocular muscle can often cause a clinical picture
of entrapment even though the muscle itself is not
incarcerated in the fracture. Such findings can often
Figure 17.2: An anterior-posterior view into the right bony orbit

be subtle. So a high index of suspicion is important
(Figures 17.3A and B, and 17.4A to D).
Finally, the optic nerve enters the optic canal near
the apex of the orbit. Blunt trauma can result in optic
nerve injury through several mechanisms: collapse
of the optic canal with crush injury to the nerve, injury
of perforating vessels to the optic nerve, hemorrhage
with compressive optic neuropathy, severing through
avulsion of the nerve, and direct injury to the globe
with transmission of the impact posteriorly (Figures
17.5 and 17.6).
EXAMINATION
A patient with orbital trauma requires a complete
history and ophthalmic examination, including a
dilated fundus examination. Any loss of consciousness should be documented, and the possibility
of an intraocular or intraorbital foreign body must
be addressed. The possibility of an open globe should
be considered in every patient with orbital trauma,
and an open globe must be ruled out prior to any
orbital evaluation and management. Loss of vision,
dysmotility, hyphema, and 360 subconjunctival
hemorrhage are often associated with a ruptured
globe.
When the examination occurs in an intensive care
setting, as is often the case, exact history and a full
examination cannot be obtained. In such a setting,
early evaluation of the pupils, prior to sedation/
analgesia-related miosis, is critical to identifying optic
nerve trauma and a relative afferent pupillary defect
(RAPD). Intraocular pressure should be measured
with a handheld device, such as a Tonopen
(Medtronic Ophthalmics, Minneapolis, MN, USA),
and high intraocular pressure treated aggressively.

In an alert patient with loss of vision and elevated
intraocular pressure, the possibility of a retrobulbar
hemorrhage must be assessed, and when appropriate,
a lateral canthotomy with cantholysis performed
acutely. Particular attention must be given to
patients who are on blood-thinning medications,
such as warfarin, which can make an orbital
hemorrhage both more likely and more severe.
Canthotomy incision is a simple and fairly benign
technique for rapidly reducing vision-threatening
orbital pressure, and the addition of a cantholysis can
further improve the decompression.15 It is not rare
for patients with an orbital compartment syndrome
to report improvement in vision within minutes of a
canthotomy and cantholysis. Evacuation of an orbital
hematoma in the acute setting has been described,
including a minimally invasive technique.16
While extraocular motility cannot be evaluated
in the sedated patient, radiologic suggestion of
entrapment should be further investigated with
forced duction testing at the bedside, which the
sedation facilitates (Figures 17.7A to E). It should be
Figures 17.3A and B: Mild muscle entrapment. Patient is a 45 years

old man who suffered a left blow-out fracture and experienced
diplopia in upgaze. He presented several weeks after his initial trauma
with a CT scan taken shortly after the injury. Examination found mild
restriction of the left eye in upgaze. The CT scan showed left inferior
rectus rounding, consistent with muscle entrapment. He was only
minimally symptomatic in upgaze with no diplopia in primary or
downgaze, and did not require surgical repair
D
Figures 17.4A to D: Severe entrapment with muscle pinching. Patient is a 19-year-old college student involved in a nightclub alteration, who
attributed his pain to swelling and bruising. One week later, when the swelling subsided, he continued to have pain and nausea with eye
movement, with significant diplopia. Examination revealed right inferior rectus restriction (A and B). CT showed pinching of an entrapped
muscle in a small minimally-displaced floor fracture (C). Urgent exploratory surgery found a dusky IR. After muscle release and fracture repair,
he was instructed to patch his uninjured left eye and use his right eye to read and do homework. Over the next few weeks, motility recovered
to better than 80% of normal, with only minimal diplopia in extreme down and up-gaze (D)
Figures 17.5: Optic canal injury. Patient was a 12 years old involved
in an unhelmeted accident while riding an all terrain vehicle (ATV).
She developed a left relative afferent pupillary defect. Maxiface CT
scan revealed evidence of fractures (arrowhead) through the
sphenoid sinus extending into the left optic canal, with a small air
bubble located at the intracranial opening of the optic canal (arrow).
Figures 17.6: Axial section of CT scan showing small air bubble

(arrow) at the intracranial opening of optic canal. These subtle fracture
findings are a common occurrence in pediatric patients in whom the
bones are malleable
E
Figures 17.7A to E: Forced ductionsbefore (A to C) and after (D and E) repair. (A) Forced ductions before repair demonstrating vertical
restriction, (A to C) Forced ductions after floor fracture repair with release of entrapped inferior rectus muscle, demonstrating normal ductions
(D and E)

remembered that ductions may also be limited by
orbital edema, so the ductions should be compared
with one another. Often, the pupils cannot be dilated
because of the tenuous neurological state of these
patients in the immediate postinjury period. In these
cases a direct ophthalmoscope may be used to view
the vitreous and disc. A vitreous hemorrhage should
be noted and further investigated for a possible
rhegmatogenous retinal detachment using B-scan
ultrasound. Likewise, the presence of a corneal
abrasion or a hyphema should be assessed and treated.
The evaluation of facial fractures is often
performed by a multi-disciplinary team, and it is
important to communicate effectively with other
members of that team. The orbital evaluation should
include palpation of the rim for any step-offs, and of
the periorbital region for crepitus (Figures 17.8A
and B). If the patient is alert, sensation in the
trigeminal distribution can be assessed. Hypoesthesia
in the distribution of the V2 branch of the trigeminal
suggests an orbital floor fracture, but can also be
associated with nerve contusion and orbital edema.
Hertel exophthalmometry is a useful indicator of the
risk of enophthalmos, and in our experience,

the presence of 1.5 mm or more of enophthalmos
in the acute posttrauma period suggests that
further enophthalmos may develop once swelling is
reduced.
Additional structural and functional consequences of orbital fractures should be carefully
assessed. Attention should be given to integrity and
symmetry of the medial and lateral commissures.
Zygomatic fractures can often cause lateral canthal
dystopia (Figures 17.9A to D), whereas nasoethmoid
Figures 17.8A and B: Lateral canthal dystopia post-ZMC fracture

repair. Note the right lateral canthal dystopia. Patient had suffered a
motor vehicle accident with right orbital fractures. His zygoma was
reduced to achieve alignment in 2 dimensions (rather than 3
dimensions), with resultant enlargement of the orbital cavity. He
presented to our clinic with enophthalmos and diplopia. Subsequent
surgical repair resolved his enophthalmos and diplopia
Figures 17.9A to D: Traumatic telecanthus (A). Patient suffered significant facial trauma following a fall, with severe comminuted nasoethmoid
fractures along with maxillary and zygomatic fractures (B and C). Initial repair did not fully restore the anatomy of the medial canthus and she
was referred for a consultation. Intraoperatively, scar tissue was debulked and miniplate fixation was used to anchor the medial canthal
tendon. Postoperatively, she has medial scleral show, and only 1mm of telecanthus (D)

fractures can often cause telecanthus (Figures 17.10A
and B). Nasoethmoid fractures are also associated
with damage to the nasolacrimal duct, leading to an
increased risk of posttraumatic epiphora.
Often, surgical repair of orbital fractures can be
delayed until a better examination can be performed
and an informed consent can be obtained. In this
setting, useful adjuncts in the evaluation of diplopia
and ocular dysmotility are ocular alignment measurements of heterotropia, and the binocular visual field
(also known as diplopia field) which uses the
Goldmann perimeter to delineate the area of fusion.
IMAGING
Computed tomography (CT) scan without contrast
continues to be the workhorse of orbital fracture
imaging. A study including axial, coronal and sagittal
cuts through the orbit is prefered.9, 17 Displacement
should be noted using the bone-window, whereas
the presence of soft-tissue herniation, including fat
and muscle herniation, should be assessed using the
soft-tissue window. A careful and systematic review
of the orbital bones is necessary in order to avoid
missing a small fracture that may be clinically
relevant. Once a fracture has been identified, the
imaging study is carefully reviewed for other facial
fractures, especially nasal, frontal, zygomatic arch,
and mandibular fractures, as well as any fractures of
the orbital buttresses. The orbital rim, inferomedial
strut, and the bony platforms at the edges of fracture
are assessed. The repair of pan-facial fractures in most
centers is coordinated with the rest of the trauma
team in order to achieve the best possible outcome
for the patient. Of great import is the overall size of
the orbital fractures, as well as the extent of

displacement. We typically recommend surgical
repair for orbital wall fractures that total more than
50% of the size of the orbital floor, since such
fractures, when not repaired, can lead to significant
enophthalmos.18 (Figures 17.11A to C).
Next, the soft-tissue windows should be carefully
examined for herniation of fat and/or extraocular
muscle. However, it is important to emphasize that
muscle entrapment is a clinical diagnosis, not a
radiological diagnosis. The presence of retrobulbar
hemorrhage should be noted, and its size
approximated. The appearance of the extraocular
muscles should be carefully noted for a rounding
effect (which may signify entrapment) or for
enlargement that can be associated with a hematoma
(Figure 17.3). If muscle entrapment is noted clinically
but not supported radiologically, we recommend that
surgical exploration be carefully considered.
Figures 17.10A and B: Naso-ethmoid comminuted fracture repair

with miniplates, and fixation of the medial canthal ligament to the
miniplates. A nasal splint with silastic bolsters can facilitate
reconstruction of the medial canthal architecture
C
Figures 17.11A to C: Enophthalmos. Three examples of clinicallyapparent enophthalmos resulting from orbital trauma
IMPLANT MATERIALS
The choice of material for orbital fracture repair is
broad, and includes both autogenous tissues and
alloplastic materials. Each has advantages and
disadvantages.19
Alloplastic implants are easily available, requiring
no harvesting procedure with its associated
morbidity. Several, such as titanium plates and
porous polyethylene, have proven over time to be
strong and effective. 20-24 The disadvantage of
alloplasts is that they do not completely integrate
with the living orbital tissues, which can lead to early
and late complications, including infection, exposure
and/or extrusion. The bacterial load required to
infect an alloplastic implant can be lower by a factor
of 10,000 than for an autogenous graft.25 Implant
exposure can particularly pose a risk for infection. In
addition, because the integration of even the best
alloplastic implants is incomplete, migration and
exposure can occur, both in the early postoperative
period and as a late complication. Finally, when a
complication occurs with an alloplastic implant,
management will often require removal of the
implant, which can be challenging.
Alloplastic materials include porous polyethylene
(e.g. Medpor, Porex Surgical, Newnan, GA, USA),
hydroxyapatite (e.g. Biocoral, Wilmington, DE,
USA), titanium mesh (such as from KLS Martin,
Tuttlinger, Germany; Stryker Craniomaxillofacial,
Portage, MI, USA; Synthes Inc., West Chester, PA,
USA), and nylon (such as Supramid, S. Jackson,
Alexandria, VA).
Titanium is a strong, inert metal. It does not
integrate but can be easily fixated to the surrounding
bones and can provide excellent support for orbital
structures. 23 If bone resorption occurs, it may
infrequently require removal. Overall, titanium mesh
can be an excellent choice for orbital fracture repair
and has a significant track record of safety. 24
However, in our experience, reoperations following
implantation of titanium mesh are more difficult as
fibrous scar tissue insinuates itself into the holes of
the mesh (Figures 17.12A and B). Hence, titanium
mesh is not our first choice in the repair of orbital
floor fractures. In cases of severe obliteration of more
than one of the orbital walls, titanium mesh may be
an excellent choice, owing to its ability to be shaped
Figures 17.12A and B: Titanium mesh with scarring. Patient is a

young woman who was injured in a motor vehicle accident. She
suffered severe ocular injuries that eventually resulted in an
enucleation. In addition, she underwent floor fracture repair with
titanium mesh. Her implant motility was severely limited and she
developed significant enophthalmos with superior sulcus deformity.
She was referred for an orbital consultation and underwent orbital
volume augmentation. Intraoperatively, her inferior rectus and
surrounding orbital tissues were found to be tightly adherent to the
titanium mesh, with extensive scarring through the holes in the mesh.
These were dissected off to free the orbital tissues from incarceration
in the titanium mesh
and maintain the desired shape. The use of titanium

mini-plates in the stabilization of orbital rim and
buttress fractures is a mainstay of fracture fixation
techniques.
Nylon sheets come in a variety of sizes and are
easy to use.26 Fixation can be achieved with a fibrin
sealant (e.g. Tisseel, Baxter, Deerfield, IL, USA) or a
biological glue (BioGlue, CryoLife, Kennesaw, GA,
USA). However, late complications with nylon sheets
are not uncommon, and in particular, the capsule that
forms around the nylon sheet can spontaneously
hemorrhage.27
Our preferred alloplastic implant material is
porous polyethylene, which is strong, biocompatible
and can integrate well.20, 28-30 Medpor Barrier implants
(Porex Surgical, Newnan, GA, USA), are porous
polyethylene plates that are coated with a thin, nonporous, high density polyethylene barrier that is
heat-bonded to the porous material on one side. This
barrier is positioned toward the orbital tissues to
reduce scarring and attachment of orbital tissues to
the plate.31 The barrier also has the added benefit of
strengthening the sheet. Porous polyethylene
implants can also be easily secured to the rim with
screws if necessary, either using a channel implant
or directly through the porous polyethylene. They
are malleable and can be cut into various sizes and
shapes with a large Metzenbaum scissors. A newer
version of the porous polyethylene implant is the
TITAN implant (Porex surgical), which can hold
curved shapes particularly well. This implant is well

suited for repair of large floor fractures and
reconstructions that involve both the floor and the
medial wall (such as loss of the inferomedial strut)
(Figures 17.13A to D). The titanium mesh embedded
in the porous polyethylene contains holes through
which screws can be placed for improved fixation if
necessary.
Autogenous bone grafts have a proven track
record of reliability, but require a harvesting
procedure unless a cadaveric bone graft is used. Given
recent concerns with infectious agents and prion
diseases, cadaveric bone grafts may be seen as less
desirable to many patients. Fresh bone contains living
osteocytes and integrates well with the surrounding
bones. When cranial bone is used, early integration
is achieved and minimal resorption is observed.32-34

This is most likely the result of the neural crest origin
of calvarial bone, which is shared with orbital bones.
The neural crest-derived craniofacial bones form
through intramembranous ossification, whereas the
mesodermal bones of the ribs and pelvis form
through endochondral ossification.35, 36 Calvarial bone
can be harvested without the need for surgical
preparation of a different body site (Figures 17.14A
to D). In addition, calvarial access can be hidden
behind the hairline, and when a bicoronal approach
is used for orbital fracture repair, the same exposure
can be used for harvesting the graft. A major
disadvantage of calvarial bone grafts is that despite
the overall safety of the harvesting procedure, the
Figures 17.13A to D : Medpor TITAN in complex fractures and orbital reconstructions. A: Posterior large medial wall fracture and Medpor TITAN
implant prior to placement. The implant was cut to size, incorporating a notch for the inferior oblique. It was then bent and placed into position.
A=anterior. P=posterior. S=superior. B: Even a large Medpor TITAN implant can be bent and will hold its shape. This patient underwent orbital
reconstruction following excision of sino-orbital squamous cell carcinoma that involved the inferomedial orbital bones. Fixation was achieved
with glutaraldehyde-crosslinked albumin adhesive (BioGlue, Cryolife Inc)
D
Figure 17.14A to D: Calvarial bone grafts. After exposure of the skull, a partial thickness calvarial graft is harvested.
Next, it is used to repair an orbital floor fracture
inner table can be penetrated, and bleeding and brain

damage can occur.37-39 Particular care must be taken
to avoid harvesting within 2 cm of midline in order
to prevent injury to the sagittal sinus.40 Patients will
often feel a depression at the donor site, which must
be explained preoperatively. At our institution, we
rarely use calvarial bone grafts, and when we do, it
is in the context of extensive craniofacial reconstruction performed by a team that includes
craniofacial and neurological surgeons.
Iliac crest and rib bone grafts are commonly by
plastic surgeons. They offer a large supply of easily
accessible cortical bone. Ribs are malleable, which
can be both an advantage and a disadvantage. Iliac
bone is hard and can be difficult to contour but can
be used successfully.41 Donor site morbidity, in the
form of bleeding, pain, and gait disturbance, can be
significant. Both types of bone are of mesodermal
origins, and ossify through an endochondral process.
Their harvest into the neural crest-derived facial

skeleton can result in delayed integration and
significant resorption. We rarely use rib or iliac crest
bone grafts, which are more commonly considered
in facial reconstruction following craniofacial tumor
resection, and is beyond the scope of this chapter.
The interested reader is encouraged to refer to the
excellent reviews and textbooks that focus on this
subject (e.g. Holck and Ng, 200642).
GENERAL OPERATIVE
CONSIDERATIONS
In evaluating orbital fractures, several issues should
be addressed. These include any indications for
fracture repair (such as fracture size and diplopia
with entrapment), the timing of fracture repair,
managing patient expectations, working with multiple
surgical services, risk of infection and antibiotic

coverage, orbital edema and the use of steroids,
concomitant management of soft tissue injuries, the
use of blood thinning products, and elective versus
urgent repair. Patients should be advised to avoid
blowing their nose,43 and prescribed nasal saline spray
2-4 times daily. Patients are instructed to keep their
head elevated, and to use ice-cold compresses for 23 days. Any blood thinning medications, especially
aspirin, clopidogrel (Plavix, Bristol-Myers Squibb) or
warfarin, should be discontinued in co-ordination
with the prescribing internist. Other blood thinning
products include non-steroidal anti-inflammatory
drugs, vitamin E, garlic, ginseng, and ginkgo
biloba.44,45 With patients on warfarin, the prothrombin
time should be checked in the early preoperative
period. Since an orbital hemorrhage can have
catastrophic effects on vision, care must be taken in
operating on anticoagulated patients.
Antibiotics
Orbital cellulitis is a serious but uncommon
complication of orbital fractures.46 Practice patterns
vary widely regarding the use of antibiotics in the
context of orbital fractures. Multiple published studies
have shown that postoperative antibiotics do not alter
the rate of infection associated with orbital fractures,
although good studies have not been performed.22,47,48
A randomized trial with 181 patients who underwent
open reduction and fixation of mandibular fractures
also showed no advantage to postoperative
antibiotics.49 Risk factors for orbital infection in the
context of orbital fractures include open fractures,
sinusitis, and contaminated wounds. 46 In these
situations, we always give preoperative antibiotics,
since preoperative administration of antibiotics
has been shown to provide improved prophylaxis.50
In addition, in cases of frank wound contamination,
we carefully treat the wounds with 5% Betadine
solution and irrigate the wound intraoperatively
with bacitracin solution. Our antibiotics of choice are
Cefazolin, Ampicillin-Sulbactam (Unasyn, Pfizer,
NY, USA) or Clindamycin, with the latter two
providing improved coverage of anaerobic microorganisms.
The risk of antibiotic overuse cannot be overemphasized. The human body is continuously
colonized by bacteria that exist in steady-state
equilibrium in the context of the normal flora.
Treatment with antibiotics alters the equilibrium,
which can potentially lead to the proliferation of

antimicrobial-resistant pathogenic organisms. Hence,
overuse of antibiotics can cause an infection with
resistant bacteria. We usually limit antibiotic usage
to the preoperative setting, with administration prior
to surgical incision in order to achieve significant
tissue concentration at the surgical site intraoperatively. Postoperative antibiotics are given only
if there are active signs of infection or if systemic
steroids are prescribed in the context of a higher risk
of infection (as discussed below).
Steroids
Glucocorticoids can be extremely useful in the
perioperative management of orbital fractures.51-53
However, their use can lead to complications,
especially an increased risk of infection. Hence, any
steroid administration is done using a very rapid
taper regimen.
The most useful contexts of steroid usage are in
the preoperative assessment of extraocular muscle
function and in the prophylaxis of postoperative
emesis (which can cause implant movement and
bacterial spread). In the preoperative setting, it is
sometimes difficult to distinguish between extraocular muscle contusion or edema and frank muscle
entrapment. This distinction is important to make
since it can strongly influence the decision to
recommend surgical exploration repair. Therefore,
when there is significant dysmotility associated with
moderate to severe orbital edema, a small orbital
fracture and no obvious entrapment radiologically,
we document the dysmotility with a binocular visual
field (also known as a diplopia field, performed with
both eyes open on a Goldmann Perimeter), and
prescribe a rapid taper of Prednisone or
Methylprednisolone over the course of 5-7 days. A
typical Prednisone taper for an adult would be 40,
30, 20, 10, and 5 mg over the course of 5 days. We
re-evaluate motility toward the end of the treatment
course (in 5-7 days) and reassess the need for surgical
intervention. In numerous cases, these exams will
reveal significantly improved motility and near
resolution of diplopia, avoiding the need for surgical
exploration of a fracture that was otherwise too small
to warrant surgical repair.
Another context for prescribing a steroid taper
is in children, who tend to swell more and scar faster.
We try to operate on children earlier (within 1 week

and in cases of severe restriction, even sooner). A-5
day steroid taper can help control posttraumatic
edema and facilitate early and safe surgery.
Intraoperative IV steroids can be very useful in
two respects: control of nausea, and expedited
resolution of postoperative edema and discomfort.
In an adult, 8-10 mg of Dexamethasone are often
given by the anesthesiologist toward the end of
surgery. Intraoperative steroid irrigation of the orbit
is performed rarely, and usually in the context of
late repair of a scarred musculoseptal system.
Infrequently, a postoperative steroid taper is
prescribed if severe edema is anticipated (e.g. late
repair of severely scarred orbit), or if edema would
interfere with the postoperative evaluation and
patient management (e.g. with extraocular motility
evaluation). However, there are no good studies to
direct perioperative steroid use, and the potential
side effects must be fully considered.
Pediatric patients
Special attention must be given to nondisplaced
fractures and floor fractures in pediatric patients
since muscle entrapment with frank muscle pinching
may occur.54 Steroids will not only fail to resolve the
dysmotility, but will also delay needed surgical
intervention, which can lead to irreversible muscle
injury. Such fractures have been termed "greenstick"
fractures, and are the result of the fact that the facial
bones of children have a higher cancellous
composition and thinner cortices, resulting in
increased bone flexibility.55-58
At times, orbital floor fractures result in minimal
soft tissue injury, no orbital edema and no
enophthalmos but severe dysmotility with entrapment, pain, and nausea. CT scanning may only
demonstrate minimal tissue herniation and fracture
displacement. This presentation has been termed
"white eye syndrome."54, 59 Clinical signs of muscle
pinching and entrapment include restriction in
upgaze, severe pain worsened by upgaze, and
oculocardiac reflex induced by upgaze, which can
include nausea, vomiting, bradycardia and/or
syncope.58, 60, 61 Timely management of fractures with
muscle pinching is essential in order to reduce the
risk of irreversible muscle damage and strabismus,
both in the pediatric and adult population. In patients
with White Eye Syndrome, surgical exploration and

repair must be carried out urgently. The examination
of a child who is in pain and distressed following
orbital trauma can be quite challenging, and the
surgeon must maintain a high level of suspicion,
especially when the eye and orbit appear normal but
there may be a motility disturbance.
Timing of surgery
The ideal timing of surgical exploration and repair
depends on the clinical findings, the overall medical
condition of the patient, the radiographic evidence
and any concomitant injuries. Delayed repair has the
advantages of reduced swelling, a more thorough
preoperative evaluation, and an increased opportunity to establish a meaningful patient-doctor
relationship prior to any surgery. Early repair has
the advantage of reduced fibrosis and scarring. In
general, we prefer to repair orbital fractures within
2 weeks of injury once the majority of post-traumatic
edema has resolved.18, 60
There are several exceptions to this 2 week
guideline whereby early or late intervention would
be preferred. First, if the patient's overall medical
condition is unstable, then medical stabilization must
take precedence. Second, if entrapment with muscle
pinching is encountered (often in children as part of
a White Eye Syndrome), urgent repair is advisable
in order to reduce the risk of irreversible muscle
damage. Third, when the optic nerve may be
compromised, the status of the optic nerve should
be properly investigated prior to any orbital surgery.
Otherwise, surgery may further compromise the
already-traumatized nerve, or the patient may
perceive the surgery as the cause of any optic nerve
damage. The status of the globe and the possibility
of a retinal detachment must also be fully
addressed prior to any orbital fracture surgery.
Finally, if other surgical interventions are planned,
coordinating care can be an overall advantage to the
patient by minimizing multiple inductions of
anesthesia.
Another softer exception to the 2-week rule is
the pediatric population. Children swell more, but
their edema resolves faster. They heal and scar faster,
and are more prone to greenstick fractures and
muscle pinching. Hence, expedited repair is often
recommended for children.
Decision: repair or not repair?

Surgical repair of orbital fractures is a very safe
procedure when done appropriately. Nevertheless,
surgery can cause complication.62, 63 The decision of
whether to repair an orbital fracture is complex, and
must rely on the individual circumstances as well as
on the experience of the surgeon. However, several
guidelines have been published that can be of great
help in making a recommendation regarding surgical
repair. These guidelines address the risk of posttraumatic enophthalmos, 64 ocular dysmotility,
diplopia, and facial deformity.
Fracture size correlates well with the risk of posttraumatic enophthalmos.18, 65 Hence, radiographic
assessment of fracture size can be a critical part of
the decision tree. There are several methods that have
been advocated in the evaluation of post-traumatic
enlargement of orbital volume. When the zygoma is
not involved, it is simplest to assess whether the sum
of orbital floor and medial wall fractures constitutes
half of the floor or more. This algorithm is simple to
execute but does not take into account any lateral
wall/ZMC fractures. 65 Another method requires
measurement of the added volume caused by the
fracture. This is determined by comparing the
fractured orbit with the non-traumatized orbit.66-68
For example, using one algorithm, measurements that
reveal greater than 13% orbital volume enhancement
would lead to a recommendation for surgical repair.67
The weakness of this algorithm is that many patients
sustain injury to both orbits, which precludes useful
comparison.
Zygoma fractures can seriously complicate any
volumetric assessment, since floor fractures are often
present but get reduced once the ZMC fracture is
reduced. 65 Nevertheless, the rotation and/or
displacement of a fractured zygoma can lead to
severe orbital volume changes, and incomplete
reduction of ZMC fractures can be a common cause
for postoperative enophthalmos. ZMC reduction
requires special care since without three point
fixation, reduction may be incomplete although the
fracture may appear well aligned along the orbital
rim. Rotation and orbital volume expansion can still
occur and can often be hard to detect. Hence, threepoint reduction of ZMC fractures with proper
fixation should be the goal of these surgical
repairs.65,69,70
Ocular dysmotility and diplopia are debilitating

consequences of orbital trauma. Ocular dysmotility
can occur for several reasons, and it is the task of the
surgeon to distinguish between the etiologies in
order to make the appropriate recommendation.
Posttraumatic extraocular muscle dysfunction can
occur as a result of muscle contusion, hematoma,
fibrosis or avulsion, muscle entrapment with or
without pinching, cranial nerve injury and muscle
paresis, generalized orbital swelling, or contracture
of the antagonist muscle. Muscle fibrosis or
contractures are later complications that must be
prevented by proper management. However, at
times it can be very challenging to distinguish
between ocular dysmotility with or without
entrapment. Limitation in up-gaze with associated
pain and nausea can be telltale signs of muscle
entrapment. In addition, CT scans can be particularly
helpful in this task. Rounding of an extraocular muscle
is often associated with entrapment. The radiologist
and surgeon must be particularly cognizant of orbital
tissue herniation without frank muscle herniation:
the herniated tissues may cause entrapment by virtue
of their numerous fibrous attachments to a muscle.
Such a fracture should be repaired.
Some surgeons advocate exploration and repair
of orbital floor fractures for non-resolving infraorbital hypoesthesia. However, a meta-analysis of
the literature found little evidence to support
such a recommendation since the reported
surgical outcomes were poor. However, if infraorbital pain is worsening, exploration is probably
warranted.71-73
The decision to operate is complex and must be
individualized. Our preference is to operate within
2 weeks of injury except in children, in which case
surgical repair would preferably take place within a
week. In cases with pinched muscles, urgent repair
within 1-3 days of injury would be advocated. When
orbital edema makes the evaluation difficult, a steroid
taper can be very helpful. When the examination is
inconclusive, a re-examination is warranted and can
be critical to making the proper diagnosis and
surgical plan.
Irrespective of the type and location of the
fractures, the goals of surgery are fundamentally
similar: repositioning of herniated orbital tissues,

reconstructing the orbital bony support, and restoring
normal orbital volume and structure.
FLOOR FRACTURES
Orbital floor fractures are common, and result from
blunt orbital trauma in which force is delivered to
the thin bones of the orbital floor, typically along
the infraorbital canal. Often, an orbital floor fracture
will not involve the orbital rim, which is much thicker
and stronger. In such an instance, the term "blowout fracture" is often applied. The term was first used
by Smith and Regan in 1956 to describe orbital
fractures caused by striking the orbital rim with a
hurling ball.74-76
The orbital floor is the shortest of the walls. It
consists of the roof of the maxillary sinus with a small
contribution posteriorly from the palatine bone, and
contains the infraorbital groove and canal. The edge
of the canal forms a weak spot in the floor structure.
Hence, most floor fractures extend up to the canal,
but the canal is often left mostly intact.
Concomitant displaced zygomatic fractures
should be repaired first, since reduction and fixation
of the ZMC fracture will often reduce the floor
fracture as well.65 Floor fractures are often associated
with medial wall fractures. When making the
decision to proceed with or forgo surgery, the total
wall area involved by the fracture, including the floor
and medial wall, must be taken into account and
addressed.
The evaluation of a suspected floor fracture often
requires CT scanning with coronal sections, which
facilitates the assessment of bone displacement and
extraocular muscle findings. It must be emphasized
again that muscle entrapment is a clinical diagnosis,
which should be supported by the radiologic
evidence but need not be.
Extraocular muscle entrapment in the context of
orbital floor fractures is not uncommon.(Figure 17.4)
However, the finding of muscle entrapment can be
subtle, and a high level of suspicion must be
maintained (Figure 17.3). Tissue herniation is very
common in the context of orbital floor blow-out
fractures. All orbital tissues must be retrieved and
repositioned into the orbit at the time of repair;
otherwise, tissue incarceration can lead to necrosis
and permanent muscle dysfunction.
Our favored approach to the inferior orbit is

through a transconjunctival approach.77-80 We usually
avoid the lateral canthotomy and inferior cantholysis,
but at times, when better exposure is required for a
larger fracture, a tighter orbit, or more extensive
herniation, the canthotomy and cantholysis can
greatly aid in obtaining adequate exposure. Care must
be taken to perform the exposure correctly to avoid
postoperative complications.81 The inferior fornix and
lateral canthus are infiltrated with 2-3 ml of local
anesthetic containing 1% Lidocaine, 0.25%
Bupivicaine, and 1:100,000 dilution of epinephrine.
The patient's face and any wounds are carefully
prepped with 5% Betadine solution, and sterile
drapes are placed. We routinely place a lubricated
plastic corneal protective shield on the globe.
First, forced ductions are performed to assess
for muscle entrapment (Figure 17.7). Next, the
assistant retracts the lower eyelid down with a
Desmarres retractor. The surgeon uses a malleable
retractor to sweep the orbital fat posteriorly and
drape the conjunctiva and lower lid retractors over
the inferior orbital rim (Figure 17.15A). The surgeon
then uses a monopolar electrocautery device with a
microdissection needle (such as the Colorado needle,
Colorado biomedical, Evergreen, CO, USA) to cut
through conjunctiva, lid retractors and orbital rim
periosteum to reveal the bony rim. The incision is
made inferiorly in the fornix (at least half way
between the inferior edge of the tarsus and the
deepest part of the fornix) to help minimize cicatricial
changes (retraction or entropion) of the eyelid
margin (Figure 17.15B). We then use a Freer elevator
to lift the periorbita off of the orbital floor. Our
dissection is guided by the CT findings: the initial
sub-periosteal dissection is performed away from the
fracture and then brought to the fracture site. The
fracture edges are carefully defined and visualized.
A malleable retractor is used to gently retract the
globe superiorly while the herniated orbital contents
are lifted back into the orbit using a Freer elevator.
Often, the herniated tissues need to be bluntly
separated from any early scars that form at the edge
of the fracture and in the maxillary sinus; this must
be done slowly and carefully, taking care to avoid
injury to the infraorbital neurovascular bundle and
the perforating artery. The maxillary sinus is typically
well visualized through the fracture (Figure 17.15C).

When the sinus is full of blood, we typically evacuate
the blood using suction, which also improves
visibility.
Once the herniated tissue is released, a Teflon
sizer (DuPont, Wilmington, DE, USA) is used to assess
the size of the needed implant (Figures 17.15D and
E). In the absence of a sizer, aluminum foil from a
suture packing, sterilized X-ray film, or any other
similar strong and sterile material can be used.
Adequate overlap with the fracture edges must be
confirmed. Particular attention must be given to
avoidance of orbital tissue incarceration between the
implant and the fracture's bony ledges.
The implant is soaked in an antibiotic solution
(e.g. bacitracin solution), and cut to size with the
appropriate scissors (Figure 17.15F). It is then molded

into the proper curvature. It is slipped carefully into
the orbit to cover the fracture (Figures 17.15G to I).
If a porous polyethylene implant with high density
"barrier" surface is used, the barrier side is oriented
toward the orbital soft tissue and the porous surface
turned toward the maxillary sinus. Care must be
taken to avoid an implant that is too long which might
cause damage to the orbital apex. The support of the
implant by the bony ledges is confirmed, and any
tissue incarceration is reduced. The end point should
be a stable implant that can slide 1 mm with gentle
force but is otherwise immobile. When implant
stability is in question, the implant should be rigidly
fixated to the bony rim with a titanium microscrew.
Figure 17.15A: The lower lid is retracted and the rim palpated. A
malleable retractor is used to protect the globe, expose the rim, and
keep the fat pads pushed posteriorly
Figure 17.15B: The monopolar unit with a micro-dissection needle is

used to incise conjunctiva, eyelid retractors and orbital rim periosteum
deep in the inferior fornix
Figure 17.15C: After elevation of the periorbita with a freer elevator,

the orbital floor fracture is exposed. A combination of the freer elevator
and thin retractors is used to retrieve any prolapsing orbital contents
from the maxillary sinus
Figure 17.15D: The position, dimensions and posterior-most aspect

of the floor fracture are measured with the help of a probe
Figure 17.15F: Once the correct sizer is identified, the Medpor

implant is cut to size
Figure 17.15E: A Teflon sizer is used to assess
coverage of the fracture
I
Figures 17.15G to I: The implant is placed on top of the fracture. Care must be taken not to incarcerate any
orbital tissues between the implant and the orbital floor
MEDIAL WALL FRACTURES
Figure 17.15J: Once the implant is stable, the eyelid retractors are
reapproximated using buried interrupted sutures
A screw can be placed right through the Medpor

Barrier implant or through the TITAN MTB implant.
Some surgeons favor surgical glue for fixating the
implant temporarily. The Desmarres retractor is then
removed, and the eyelid retractors sutured with
buried, interrupted 7-0 polyglactin (Vicryl) suture
using 3-point fixation (Figure 17.15H). The
conjunctiva is left unsutured.
When no bony ledge is present to support the
fracture, cantilevering of the TITAN implant or a
Medpor Channel plate over the rim can provide rigid
fixation. Some surgeons favor titanium mesh in this
situation.
Other methods of fixation include the use of fibrin
sealant (such as Tisseel, Baxter, Deerfield, IL, USA)
and biological glue. BioGlue (CryoLife, Kennesaw,
GA, USA) is a two-component glue, containing
engineered bovine albumin in one tube and a
glutaraldehyde solution in the other. When the two
substances mix, the glutaraldehyde cross-links
albumin molecules to each other and to the
surrounding proteins. Glutaraldehyde cross-linking
resembles a peptide bond, and hence can easily form
covalent bonds among proteins in contact with the
chemical. When a porous implant is used, the albumin
infiltrates through the pores as it gets cross-linked,
resulting in strong biocompatible chemical bonding
of the implant to the surrounding tissues. The use of
biological glues and sealants is particularly useful
when implant stability is in question, yet the surgical
exposure is inadequate for complete rigid fixation
(often because the globe should only be retracted
gently and carefully).
Medial wall blowout fractures are common but

historically were under-diagnosed because they can
be asymptomatic in the acute post-traumatic
phase.4, 65 The nearly ubiquitous use of CT scanning
in the United States in the evaluation of facial fractures
has made the diagnosis of medial fractures much
easier. 82 However, when CT is unavailable, the
evaluating physician must maintain a high level of
suspicion. Epistaxis can be associated with medial
wall fractures because of tears in the sinus mucosa.
The epistaxis is typically self-limited. Visionthreatening orbital hemorrhage can occur if the
anterior or posterior ethmoidal vessels are injured.
It is important to remind patients to avoid blowing
their nose, since this can lead to orbital emphysema
and a compartment syndrome (Figures 17.16A and
B).43, 83
The thin lamina papyracea constitutes most of
the medial wall of the orbit, behind the posterior
lacrimal crest. The medial orbital wall is completed
posteriorly by the lesser wing of the sphenoid, and
anteriorly by the lacrimal bone. Both the sphenoid
and the lacrimal bones are thicker. Hence, medial
wall fractures occur because of the weakness of the
thin lamina papyracea, and are typically localized at
the boundary between the lamina papyracea and one
of the thicker bones. Medial wall fractures commonly
occur in the context of orbital floor blowout fractures,
but can also occur in isolation.4, 26 They can also be
associated with nasal fractures, which can lead to
Figures 17.16A and B: Orbital/periorbital emphysema. Monocular

patient was assaulted with a blow to the left orbit. He presented to
the emergency department after blowing his nose, leading to severe
pain and reduced vision. In the ER, his intraocular pressure was
measured at 60 mm Hg. Emergent lateral canthotomy and inferior
cantholysis were performed, resulting in decompression of the orbit,
restoration of vision and rapid decline in intraocular pressure to the
teens. A CT scan following the cantholysis revealed air in the orbit.
His lateral canthus was repaired 4 days later

telecanthus and possible damage to the nasolacrimal
drainage apparatus.84, 85
Described approaches to the medial wall fracture
include a Lynch incision in the medial canthus, a
bicoronal incision, a medial upper lid crease incision,
or a transconjunctival incision. However, our
preferred approach is the transcaruncular incision.8688
When the medial wall fracture is large, or associated
with a floor fracture, multiple incisions may be
necessary, such as a secondary lower lid fornix
incision.26, 89
The transcaruncular approach requires meticulous
hemostasis in order to properly expose and visualize
the fracture,. Pinpoint cautery with a micro-dissection
needle (e.g. Colorado needle, Colorado Biomedical,
Evergreen, CO, USA) is very helpful, as is the use of
thrombin solution, absorbable gelatin sponge
(Gelfoam, Pfizer), FloSeal (a combination of gelatin
foam and thrombin, Baxter) or 3% hydrogen peroxide
solution. Care must be taken with hydrogen
peroxide, since it can inhibit tissue healing, and its
use has been associated with air embolization.90-92
Good lighting is also important, and a head-mounted
light source is nearly always used in our cases.
Prior to initiating surgical repair, forced ductions
are performed to assess for muscle entrapment
Figure 17.17A: Transcaruncular exposure of the left medial wall

following disinsertion of the inferior oblique muscle
(Figure 17.7). The transcaruncular approach begins

with infiltration of the medial fornix with local
anesthetic containing 1:100,000 dilution of epinephrine. An incision is made through the caruncle
anterior to the plica semilunaris using Westcott
scissors.86, 87 The incision is extended superiorly and
inferiorly along the conjunctival fornices in order to
create sufficient exposure and prevent uncontrolled
tearing of the conjunctiva intraoperatively. A
sufficient distance away from the canaliculi is
maintained. Curved Stevens tenotomy scissors are
then used to bluntly dissect along Horner's muscle,
which inserts on the posterior lacrimal crest. Care
must be taken not to iatrogenically fracture the lamina
papyracea just posterior to the crest, since this will
make subsequent elevation of the periorbita more
challenging. Once the posterior crest is exposed, the
periorbita is incised with a monopolar unit and a
microdissection needle. Achieving excellent
hemostasis is critical at this point. Next, a Freer
elevator is used to lift the periorbita anteriorly in
order to fully expose the posterior lacrimal crest. The
periorbita is then lifted posteriorly to create a subperiosteal dissection plane along the lamina
papyracea (Figures 17.17A and B). The anterior
ethmoidal neurovascular bundle is typically found
approximately 24 mm posterior to the posterior
lacrimal crest, along the fronto-ethmoidal suture line.
This neurovascular bundle is carefully and thoroughly
cauterized with a bipolar cautery unit, and then
divided with the monopolar unit. The subperiosteal
dissection is then completed to fully expose the
fracture.
Figure 17.17B: Inferior oblique isolation. The IO muscle may be tagged

with 6-0 polyglactin sutures and the origin disinserted to provide good
exposure

If the fracture is very posterior, the posterior
ethmoidal bundle may be encountered. In such cases,
the posterior ethmoidal bundle should be carefully
cauterized with the bipolar cautery to avoid intraor postoperative hemorrhaging. The posterior
bundle is typically found approximately 12 mm from
the anterior bundle, and on average only 6 mm from
the optic canal.
After adequate hemostasis and exposure are
achieved, herniated orbital tissues are retrieved and
repositioned into the orbit. The fracture size is
measured, and implant properly sized. We have
found Teflon implant sizers to be very useful at this
step. The implant is then placed over the fracture,
ensuring that no orbital tissues are left incarcerated.
When using a Medpor Barrier plate, the barrier side
should be turned toward the orbit to reduce the risk
of tissue scarring to the implant.
If the fracture extends inferiorly to involve the
orbital floor, an inferior fornix transconjunctival
incision can be made to increase exposure. At times,
the inferior oblique muscle must be disinserted from
its origin. At the end of the case, the oblique is then
reapproximated to its origin with 6-0 polyglactin
suture. The floor and medial wall fractures can be
repaired with a single implant. For this, the Medpor
TITAN Barrier implant is ideal, since it can be molded
into the required semi-cylindrical shape and will
maintain this shape. Such a large implant is better
placed through the inferior fornix incision, and then
the positioning adjusted through the caruncular
incision. Some surgeons advocate multiple implants
that can overlap, using very thin implants.26
Closing the transcaruncular incision involves
placing one to three buried interrupted 6-0 fastabsorbing plain gut sutures to close the caruncular
conjunctiva.
Medial wall fractures are particularly challenging
to repair when they are part of a comminuted nasoethmoid fracture.93 In these circumstances, posttraumatic telecanthus is common, and can be quite
disfiguring (Figure 17.9). Repair of the fractured
posterior lacrimal crest and stabilization of the medial
canthal tendon should be attempted during primary
repair. Good exposure is critical, and when the nasoethmoid fractures are part of panfacial trauma,
a bicoronal approach has many merits.94 Transnasal
wiring has been described for the repair of post-
traumatic telecanthus,93, 95 and may be necessary in

combination with miniplate fixation when repairing
severely comminuted naso-ethmoid fractures.
However, whenever possible, we favor miniplate
fixation of the comminuted bones and the medial
canthal tendon (Figures 17.10A and B).96
LATERAL WALL AND

ZYGOMATICO MAXILLARY
FRACTURES
Fractures of the lateral wall of the orbit are common,
and typically result from direct blunt trauma to the
zygoma and lateral orbital rim. The zygoma
articulates with the sphenoid, maxillary, frontal and
temporal bones. Therefore, fractures of the zygoma
can often disrupt the architecture of the entire region,
and hence are often referred to as zygomaticomalar
complex fractures (ZMC fractures). Importantly, the
orbital floor is always fractured in the context of a
displaced zygomatic fracture, but will typically reduce
once the zygoma fracture is reduced.65
Numerous surgical approaches to the ZMC
fracture have been described for open reduction,
including transconjunctival,78, 97-99 intraoral,100 temporal
(Gillies), brow incision and bicoronal flap. In
addition, some surgeons advocate closed reduction
or observation for non-comminuted and uncomplicated zygoma fractures.101, 102 The approach to the
ZMC fracture is greatly aided by careful evaluation
of the CT scan, and different classification systems
have been proposed in an effort to provide guidance
to proper preoperative planning. Whichever
approach is chosen, a reduction of the zygoma should
attempt to replace the zygoma back into its normal
anatomic location. Because the zygoma can rotate
around an axis formed by the inferolateral orbital
rim, reduction of just the fronto-zygomatic and
zygomatico-maxillary sutures is not sufficient: a third
point of alignment should be confirmed in order to
ensure proper reduction and avoid late enophthalmos.65 Exploration of the lateral floor of the orbit
can greatly assist in the fracture reduction, as well
as help avoid orbital tissue incarceration between
the zygoma and the sphenoid bones.
When non-comminuted fracture displacement is
noted on examination and CT scans, we favor surgical
exploration and repair through a subconjunctival

inferior fornix and lateral canthotomy approach.99
First, forced ductions are performed to assess for
possible muscle entrapment. The lateral canthus and
inferior fornix are infiltrated with local anesthetic
containing 1:100,000 dilution of epinephrine, and a
lubricated plastic corneal protective shield is placed
on the eye. A lateral canthotomy and inferior
cantholysis (and sometimes superior cantholysis, too),
are performed to release the lateral canthal
attachments and provide good exposure of the lateral
rim. The lateral inferior fornix is then incised to reveal
the inferolateral rim. The periosteum is incised along
the entire exposed rim, and a freer elevator is used
to lift the periosteum and expose the rim from above
the fronto-zygomatic suture down to below the
maxillo-zygomatic suture and medially to the
infraorbital canal. When a depressed floor fracture
is present, the inferior fornix incision can be carried
out medially to provide full exposure of the floor.
Next, the zygoma must be properly reduced and
aligned along three points: the fronto-zygomatic
suture, the maxillo-zygomatic suture at the inferior
rim, and a third point that can be the zygomaticsphenoid suture at the lateral orbital floor, the
zygomatico-maxillary buttress, and/or the zygomatic
arch.65, 69, 70, 99 The first two points ensure anterior
alignment, whereas a third point ensures posterior
alignment. Failure to achieve posterior alignment can
predispose to enophthalmos by enlarging the
posterior orbital volume (Figure 17.8).
A useful tool in ZMC fracture alignment has been
the T-bar screw, also known as the Carroll-Girard
screw (Walter Lorenz Surgical, Jacksonville, FL,
USA).99, 103 This cork-screw-like instrument is screwed
onto the thick bone at the malar eminence and used
to manipulate the zygoma into proper position
(Figure 17.18A and B). This tool is particularly useful
in minimally-comminuted fractures, and allows for
exceptional control of the zygoma through a smallincision approach. However, when severe comminution exists without solid bone for placement of
the screw, multiple incisions would be required to
achieve complete reduction and rigid fixation.
Following reduction of the fracture, fixation is
accomplished using titanium miniplates. Forced
ductions are performed again to ensure that no
entrapment was caused by the fracture reduction.
The lateral floor is explored to assess the zygomatico-
Figures 17.18A and B: The Carroll-Girard (T-bar) screw is a powerful

tool for 3-dimensional control and manipulation of the zygomatic or
maxillary bones during orbital fracture repair. After drilling a small
guidance hole, the screw is positioned and attached to the T bar
(Photos courtesy of Benjamin Marcus, MD, University of Wisconsin)
sphenoid suture. Next, the periosteum is sutured and

the lower lid retractors are reapproximated using
buried interrupted 7-0 polyglactin suture. The lateral
canthal tendon is resuspended to the periosteum at
Whitnall's tubercle. If lower lid laxity is present,
horizontal tightening is performed to reduce the risk
of postoperative lower lid retraction. Placement of a
Frost suture can be done in the presence of
concomitant lower lid trauma and when the risk of
cicatricial ectropion is felt to be high.
LATE AND SECONDARY

FRACTURE REPAIR
As far back as 1957, Smith and Regan made the point
that "late cases are far more difficult to correct."74
Nevertheless, thirty years ago, many surgeons
advocated observation and delay of surgery for all
but the most severe orbital fractures.104, 105 However,
with the advent of CT scanning, an evolution in
implants and fixation devices, and greatly improved
surgical techniques, primary repair has become the
standard of care for symptomatic or large orbital
fractures. Still, there are occasions when late repair
of orbital fractures is still encountered. Some reasons
include lack of access to medical care following
trauma, misdiagnosis, multiple medical problems
preventing timely repair, or a suboptimal repair that
requires reoperation.
The goal of late fracture repair is the same as for
early repair, namely to restore orbital anatomy and
reduce or eliminate any symptoms. Late repair is
nearly always complicated by significant fibrosis and
abnormal anatomy that can distort the normal
anatomic landmarks. When dysmotility is a significant
symptom and its repair a main goal of the operation,

good motility measurements are critical, as is the
assistance of an expert in adult strabismus.
Dysmotility that results from entrapment can be
corrected when the muscle entrapment is reduced.
However, if the dysmotility is the result of
irreversible muscle or nerve damage, further orbital
surgery will not restore normal motility, and can
cause a significant increase in orbital fibrosis in
addition to the risk of an unnecessary orbital surgery.
Instead, strabismus surgery would be a better option.
Hence, careful diagnosis of the underlying cause of
dysmotility is crucial. In our practice, we will obtain
a CT scan to evaluate the bony anatomy, and
occasionally a dynamic MRI scan to evaluate the cause
of dysmotility. In addition, we request a consultation
from an expert in strabismus, and employ a teamoriented approach to caring for these patients.
A common reason for late surgical repair is severe
enophthalmos (Figure 17.11). In a paper by Koo et
al. (2006),64 clinically apparent enophthalmos was
found when enophthalmos was measured at 3-4 mm
or more. In such cases, patients must weigh their
symptoms against the risks of surgical complications.
It is important to emphasize that counseling and
managing expectations are very important for
achieving a result that is satisfactory to both the
surgeon and the patient.
Our approach to enophthalmos repair stresses
pre-operative counseling in order to clearly define
what the patient desires and what we believe can be
accomplished safely. Our surgical technique
emphasizes good exposure, optimized illumination,
and meticulous hemostasis. We employ the same
incisions that we utilize for early fracture repair,
namely the inferior fornix, lateral canthotomy/
cantholysis, and transcaruncular incisions. Often, the
main decision is whether to attempt reduction of a
healed fracture or to focus on the enophthalmos and
provide orbital volume augmentation through the
use of porous polyethylene implants. Given the
added risk of re-fracturing and reducing healed
fractures, we usually opt for orbital volume
enhancement and accept the mild deformities that
may be associated with poorly positioned but healed
ZMC fractures. For volume enhancement, we typically
utilize porous polyethylene wedge implants that are
shaped to reduce orbital volume or implants
with titanium that can be fixated at the orbital rim
(Figure 17.19).
Figure 17.19: Medpor wedge implant. Orbital volume enhancement

will often require placement of space-occupying implants. The Medpor
wedge implant, which comes in both right-sided and left-sided
configurations, is a useful tool. Alternatively, multiple flat implants can
be stacked to achieve the appropriate amount of volume augmentation
CONCLUSION
Orbital fractures are commonly encountered by the
ophathlmologist, otolaryngologist, and orbitofacial
plastic surgeon. Proper treatment requires a complete
ophthalmic evaluation, systematic review of the
radiographic evidence, and thoughtful surgical
planning with careful attention to anatomic principles.
The use of smaller and hidden incisions (particularly
conjunctival incisions) has made surgical treatment
of orbital fractures more esthetically satisfying while
reducing the risk of complications. The choice of
implant materials has never been greater, providing
the surgeon with options that can be tailored to the
needs of the patients. While surgical repair of orbital
fractures has advanced considerably over the past
50 years, there is no doubt that we will continue to
see innovations and further improvements in surgical
techniques, to the continued benefit of our patients.
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18
CHAPTER
Secondary and Metastatic

Orbital Tumors
Kasturi Bhattacharjee, Harsha Bhattacharjee, Ganesh Kuri, Shyamanga Borooah
Secondary orbital tumors are due to the extension

of a primary tumor into the orbit. The orbit may be
affected secondarily by tumors arising in the adjacent
structures. Such tumors commonly arise from the
globe, lids, conjunctiva, nasopharynx, paranasal sinus,
and lacrimal sac. Intracranial tumors can also have
intraorbital extension. Several factors contribute to
the extension of tumors into the orbit. These include
the site of the primary tumor, aggressiveness of the
tumor and adequacy of initial treatment.
Orbital Extension of Intraocular Tumors

This is more commonly seen in less developed
countries due to a delay in presentation for treatment
of a primary tumor. In more advanced countries
systems of ocular screening have reduced both the
morbidity and mortality from these neoplasms.
Though most intraocular tumors can invade the orbit
if not managed aggressively and adequately,
however higher frequency is encountered amongst
retinoblastoma, medulloepithelioma and uveal
melanoma.1 Retinoblastoma and medulloepithelioma
are usually found in pediatric population whilst
melanomas are more common amongst adults.
Retinoblastomas generally remain within the

globe for a considerable duration of time. However,
once they penetrate Bruch's membrane they become
highly aggressive. With time this leads onto
extraocular spread. This may remain localized to
the soft tissues surrounding the eye or the orbit
(Figure 18.1) or may extend via the optic nerve into
the brain (Figure 18.2) and meninges with subsequent
seeding of the spinal fluid. Distant metastasis may
occur through hematogenous spread involving the
bones, bone marrow, liver, pancreas, kidney, spleen,
lungs and gonads.
Extraocular extension of retinoblastoma into the
orbit most commonly occurs through the periemissarial blood vessels. Extension also occurs
directly into the choroid and through erosion of the
globe. Once the tumor enters the orbit, it enters a
state of neoplastic proliferation and rapidly develops
into an orbital mass. Spread of retinoblastoma into
Orbital Extension of Retinoblastoma

Retinoblastoma (RB) is the most common childhood
intraocular tumor. The incidence is approximately 1
in 15,000 to 1 in 20,000 live births. Diagnosis and
treatment can salvage life. However, early diagnosis
can salvage not only the eye, but also sight. In
developed countries the mortality rate from
extraocular retinoblastoma is as low as 5 %, but this
increases to over 90% in underdeveloped countries.2
Figure 18.1: Bilateral retinoblastoma with orbital extension
Secondary and Metastatic Orbital Tumors 245

the central nervous system usually occurs by direct
invasion of the optic nerve and the subarachnoid
space by the tumor cells. Very rarely retinoblastoma
of peripapillary choroid can egress into the central
nervous system via the posterior ciliary vessels.
Orbital invasion of retinoblastoma carries a poor
prognosis and is a predictive factor of metastasis
(Figures 18.3 and 18.4). Kapelman JE et al3 produced
a multivariate analysis suggesting that orbital
invasion and optic nerve invasion were the most
highly predictive risk factors of death from
retinoblastoma.
The main goal of treatment for extraocular

retinoblastoma is to extend survival and the disease
free interval. Rootman J et al4 reported that only 9.4
% of patients with orbital extension lived more than
2 years after diagnosis. It was noted that orbital
extension of retinoblastoma was frequently
associated with distant metastasis. Zyguiska
Manchowa H et al 5 had reported 14 cases of
extraocular retinoblastoma or with recurrence of
which 78.6% had died.
Though there is no proven effective therapy for
extraocular retinoblastoma, it should be managed
with aggressive chemotherapy and radiotherapy to
the orbit. When there is meningeal involvement
craniospinal irradiation and/or intrathecal chemotherapy is indicated. However, often palliative
treatment is required if therapy is ineffective. Highly
individualized aggressive therapy enhances longer
survival in patients with overt extraocular
retinoblastoma (Figure 18.5). Chantada G had
reported use of neoadjuvant combination chemotherapy followed by surgery in the form of
enucleation and/or resection of residual orbital tumor
mass and adjuvant chemotherapy and radiotherapy.6
Chemotherapy includes Vincristine, Etoposide,
Carboplatin, Cyclophosphamide, Doxorubicin,
Idorubicin, and Cisplatin.7 They reported that this
treatment regimen was highly efficacious for patients
with orbital extension of retinoblastoma.
Figure 18.2: CT brain of the child in figure 18.1 revealed a pinealoma

giving a trilateral presentation of RB .Classic of hereditary type
Figure 18.3: Bilateral advanced retinoblastoma with extraocular

extension involving the orbit and adnexae on the left side
Figure 18.4: Bilateral retinoblastoma. The child had undergone six

cycles of chemotherapy. The left eye was salvaged , however the
right eye had undergone enucleation

retinoblastoma cells. Medulloepithelioma forms a
stroma composed of loose, delicate fibrils with
abundant ground substance resembling embryonic
mesenchyme or myxoid tissue.
Figure 18.5: Fundus photograph illustrating regression

of retinoblastoma following SALT
Keralli H et al 8 reported that exenteration

followed by chemotherapy and radiotherapy does
not prolong the survival of patients with massive
orbital involvement of intraocular retinoblastoma.
Orbital Extension of Medulloepithelioma

Intraocular medulloepithelioma of the ciliary body
is a rare tumor occurring during the first decade of
life. This embryonal tumor usually arises from the
neuroepithelium of the ciliary body.9 Rarely, it may
arise from iris, retina and optic nerve.10-12
Though the tumor tends to locally invade the
surrounding ocular structures, it can also extend into
the orbit and rarely metastasize. It can be classified
as teratoid or nonteratoid medulloepithelioma.
Medulloepithelioma that contains heterogenous
tissues like skeletal muscle, cartilage and hair are
classified as teratoid.13,14
Histologically, medulloepithelioma consists of
two cellular components: epithelial cords and a
fibrillar matrix. Epithelial cells are arranged in
convoluted patterns or in a circular pattern around a
lumen or more commonly as elongated, interlacing
cords. Both pigmented and non-pigmented cells may
be present. More malignant tumors contain some
very poorly differentiated cells which may resemble
Clinically it presents with loss of vision, pain,

photophobia, ciliary body or anterior chamber
mass,leucocoria and as proptosis in advanced cases
(Figures 18.6 to18.9). Presence of a fleshy grey, pink,
yellow or brown color mass with cystic areas in the
ciliary body region is very characteristic. Very often
lens coloboma may be the only presenting sign
occurring due to absent zonules.The tumor may
present either as a solid or polycystic mass or as sheet
behind the lens, resembling a cyclitic membrane.The
cysts of the tumor may be free floating cysts or may
settle in anterior chamber or vitreous.The tumor may
contain heterogenous tisues like cartilage which
presents like chalk particles as grey-white opacities.
Complications are rubeosis, glaucoma (Buphthalmos), cataract and retinal detachment. Malignant
changes of medulloepithelioma are rare. These
changes are characterized by increased pleomorphic
and mitotic activity with areas of poorly
differentiated neuroblastic cells or sarcomatous
changes with invasion of surrounding ocular
structures.15 Charif CM et al. reported a case of a 4
years old child with malignant medulloepithelioma
involving the sclera with extension into the orbital
fat. 16 Though tumors predominantly occur in
children, they can rarely present in adults. J Michael
Jumper had reported a case of a 45 years old man
with invasive medulloepithelioma.17
Diagnosis is usually established by slit lamp
examination along with radiological investigations
like ultrasound biomicroscopy, CT scan and MRI.
Julian G Feijoo had reported characteristic
ultrasonographic features of medulloepithelioma
which included echogenic heterogenicity of the
tumor with presence of multiple cysts with stalk-like
prolongation of the surface.18 However diagnosis is
confirmed by histopathological examination and by
immunohistochemistry.
Treatment of choice is wide local excision. In
malignant tumors enucleation is preferred. However,
with extensive extraocular spread exenteration is
performed followed by chemotherapy.Role of
radiotherapy is palliative only.
Figure 18.6
Figure 18.7
Figure 18.8
Figure 18.9
Figures 18.6 to 18.9: Ciliary body medulloepithelioma with orbital extension. 5 years old boy presenting with buphthalmos and underwent
trabeculectomy initially elsewhere. 3 months following this presented to us with proptosis and orbital mass. On biopsy it was diagnosed as
medulloepithelioma which was confirmed by immunohistochemistry. The child underwent six cycles of chemotherapy and radiotherapy
followed by total exenteration
Orbital Extension of Uveal Melanoma

Orbital involvement of uveal melanomas occur after
extrascleral extension of massive intraocular lesions.
Although extraocular spread may present with its
own set of clinical problems, ultimately it is the distant
metastases which ultimately decide the long-term
prognosis of the patient.
Uveal melanomas are a diverse group of
pigmented tumors originating in the iris, ciliary body
and most commonly the choroid. They are the most

common primary intraocular tumors with an annual
incidence that varies worldwide. Melanomas tend
to be more common in those of caucasian descent
and tend to have increased incidence with decreasing
latitude suggesting that increased sun exposure may
be a factor in development. They predominate in the
older age group peaking in the sixth decade. 19
Predisposing factors include ocular melanosis and
melanocytoma.

The tumors can be subclassified by histological
cell type using the modified version of the prognostic
cell type introduced by Callendar. Spindle A, spindle
B, epithelioid and mixed types can be seen (Figures
18.10A to G) with epithelioid cells having the worst
prognosis.20 Other prognostic factors include size of

the primary and age of the patient at presentation.21
Recently, genetic mutations have also been noted
including monosomy 3 and duplication of
chromosome 8q confer worse prognosis.
G
Figures 18.10A to G: Case of oculodermal melanocytosis with choroidal melanoma. Patient had undergone enucleation and intraoperatively pigmentation was found in the orbital fat which was later confirmed by HPE to be melanocyte with mitotic epitheloid and spindle cells
Extrascleral extension has also been found to

portend a worse prognosis.21 This is linked to the
fact that there is increased risk of distant metastasis.22
Orbital extension of an intraocular primary occurs
via scleral emissary channels, vortex vein and
posterior ciliary artery (Figures 18.11A to C) . Rare
cases of extension through the optic nerve have also
been reported.23 Shammas et al found that upto 0.4%
of uveal melanomas showed orbital extension
however, it is thought that microscopic extension has
already occurred in between 10-40% of cases.24,25
Clinically, uveal melanomas may be difficult to
diagnose. Patients will rarely complain of any visual
disturbance unless central vision is affected.
Occasionally, pain may be noted due to impingement
on the posterior ciliary nerves. Nevertheless, the vast
majority remain asymptomatic. The advent of indirect
ophthalmoscopy has greatly assisted in peripheral
viewing and perception of depth. However, simple
examination may still provide some difficulty in
differentiating these lesions from eccentric disciform
lesions, naevi or other choroidal lesions such as
hemangiomas. This is where ultrasound B-scan may
be of some assistance in determining the size and
depth of the lesion. Although CT may have little place
in investigation, contrast enhanced MRI can assist.26
It is especially useful in determining early extra scleral
spread as well as assisting in finding the extent of
the tumor to assist management.
It is thought that by the time of presentation

40-45% of tumors will have metastasized. 27
Epithelioid and mixed cell types and larger tumors
are more likely to involve the orbit.24 It is this distant
spread which will ultimately decide the long-term
survival of the patient. Consequently, signs and
symptoms of spread should be sought and liver
function tests and chest-radiograph can be performed
at diagnosis to assist early screening. There is still
little effective treatment for metastatic disease. As a
result, it has been shown that aggressive surgical
treatment of these tumors has had little effect on
Figures 18.11A to C: Case of intraocular melanoma with extension to sclera and orbit. Patient had undergone exenteration and socket
was reconstructed with temporalis muscle transfer covered with split thickness skin graft
survival and consequently management has tended

to be more conservative. Treatment is partly tailored
to the size and type of extrascleral extension. Plaque
radiotherapy has been shown to be effective against
flat or small nodular extensions, whereas with larger
nodular extension and recurrence external beam
radiotherapy followed by exenteration can be used.
Vortex vein involvement requires resection of the
vortex vein followed by further enucleating or
radiotherapy. Recently, brachytherapy has also been
proposed for as a substitute for radiotherapy. Early
findings suggest that the technique requires less time
for completion of treatment whilst delivering a
higher dose of radiation to the tumor.
Despite improving diagnosis and management
of melanoma, survival has improved little. Thus the
aim is still to be able to diagnose these tumors prior
to extrascleral extension.28
Orbital Extension of Lacrimal Sac Tumors

Occurance of lacrimal sac tumor is very rare. In
majority of patients they are epithelial in origin These
epithelial tumors include squamous and transitional
cell papillomas, oncocytic adenomas and carcinomas
like transitional cell carcinoma, squamous cell
carcinoma, adenocarcinoma, adenoid cystic
carcinoma, mucoepidermoid carcinoma and poorly
differentiated carcinoma. 29,30 Amongst the nonepithelial tumor the most common is fibrous
histiocytoma followed by a variety of other tumors

including lymphoid tumor, malignant melanoma,
hemangiopericytoma, neurofibroma, granulocytic
sarcoma and lipoma. 30 It has been found that
approximately 55% of lacrimal sac tumors undergo
malignant transformation.30 Though these tumors are
locally invasive, however at times it can invade the
orbit and can also metastasize becoming life
threatening.
The age of presentation of lacrimal sac tumors
depend on the histologic pattern of the neoplasm.
Benign tumors like papillomas are found in younger
age group. However, the malignant tumors are seen
within 41-75 years of age.31 Ni C and co-worker had
reported that genetic and environmental factors may
play a role in clinical presentation of lacrimal sac
tumor.32
The signs and symptoms are usually insidious
with epiphora being the most common symptom and
lacrimal sac mass as the most common presenting
sign.
Histopathologically, lacrimal sac tumors are
classified as epithelial tumors and non-epithelial
tumors. These tumors are either benign or malignant.
The malignant tumors are found to arise either from
pre-existing tumors or arise de novo.
The papillomas are the most common lacrimal
sac tumors .According to their growth the papillomas

are subdivided as exophytic, inverted and mixed.
However according to the histologic patterns,
papillomas are classified as squamous cell papillomas
characterized by acanthotic, stratified squamous
epithelium transitional cell papilloma with stratified
columnar epithelium and mixed papilloma with a
mixture of squamous cell and transitional cell.
Malignant transformation in the form of carcinomas
like squamous cell, transitional cell or adeno
carcinoma seem to originate from the epithelial lining
of the lacrimal sac. Other epithelial tumors are
oncocytic adenomas and mucoepidermoid carcinoma.
Oncocytic tumors are composed of special types of
epithelial cell called oncocytes due to their abundant
eosinophilic cytoplasm. These tumors rarely undergo
metastasis. Mucoepidermoid carcinoma is a rare
epithelial tumor of the lacrimal sac. These tumors
are composed of a mixture of different cells like
mucus secreting cells, epidermoid cells and basal cells.
Seven types of non-epithelial tumors of lacrimal sac
are reported. The common amongst them are fibrous
histiocytoma, lymphoma, melanoma and hemangioopericytoma. 30 In general, lacrimal sac tumors
are rarely life threatening. Distant metastasis can be
fatal.
A thorough history directed to pertinent clinical
features is very important. A comprehensive ocular
and systemic examination is necessary to assess the
firmness and location of the mass in relation to the
medial canthal tendon. Careful examination to rule
out any punctal discharge and regional lymphadenopathy and a complete nasal and sinus
evaluation should be done.
Lacrimal function tests the rapidity of flow from
the lacrimal sac. Benign tumors demonstrate outflow
stenosis and carcinomas show complete obstruction.
Dacryocystogram of a lacrimal sac tumor shows a
discrete mass with a filling defect or a distended sac
shadow with uneven or mottled density of the
contrast media and some patency.
Computed tomography of orbit and sinuses is
essential to outline the tumor and assess erosion or
invasion into nearby structures. Located within the
lacrimal sac fossa, anterior to the posterior lacrimal
crest along the medial orbital wall, the nasolacrimal

sac is usually not prominently identified on
radiographic imaging (CT/MRI). Expansion of
lacrimal sac fossa, destruction of bone, a mass
extending beyond the lacrimal sac suggest a lacrimal
sac tumor. A chest X-ray, hematologic tests including
full blood count with differential, ESR, renal and
hepatic function tests, antineutrophil cytoplasmic
antibody and leutic serology may be considered.
Swabbing for microbiologic culture is essential in an
ulcerated lesion. Biopsy for histopathological
diagnosis is mandatory for treatment.
Traditionally, suspected lacrimal sac tumor is
managed by dacryocystectomy with tissue biopsy
and frozen section analysis (Figures 18.12A and B).
This was followed by further treatment as required
including radical resection, radiotherapy, or
chemotherapy.
Nowadays, incisional biopsy is done in large
ulcerative tumors to plan subsequent therapy.
Following definitive histological examination, if no
evidence of tumor is found, a DCR is performed. If
the only suggestion of a lacrimal sac tumor is a filling
defect on DCG, a DCR and biopsy should be
performed. If the biopsy reveals a locally invasive
tumor, nasal endoscopic monitoring is advised.
Aggressive tumors are treated with radical surgery
or radiotherapy.
Figures 18.12: A case of lacrimal sac tumor which was later

diagnosed as squamous cell carcinoma of lacrimal sac

Management varies as to the tumor origin. For
lacrimal sac neoplasms, surgery with adjuvant
radiotherapy is advised. In lymphomas, chemotherapy is the definitive treatment. For localized
epithelial and mesenchymal tumors, wide surgical
excision is performed. Postoperative radiotherapy
is recommended for malignant epithelial tumors.
Further surgery and adjuvant radiotherapy is
performed for recurrent lesions.
For malignant melanomas extensive surgical
resection, radiotherapy or chemotherapy may delay
recurrence but survival remains poor.
A partially or totally irreducible lacrimal sac
mucocele indicates an underlying neoplasm. This is
managed by DCR followed by definitive therapy
determined by histology with biopsy if bone erosion
is absent on CT. If radiology suggests malignancy,
treatment is as outlined above.
Orbital Extension of Eyelid Tumors

Malignant eyelid tumors have the propensity of
aggressive growth and for metastasis necessitating
aggressive management.Though there are different
types of eyelid tumors, however epithelial tumors
are most common malignant eyelid tumors. These
are usually slowly enlarging and destructive tumors
which can invade the adnexal tissues and the orbit.
The clinical features of malignant eyelid tumors
are variable.Very often they present as chronic
blepheritis or meibomianitis or as a lid mass.There
may be associated loss of eyelashes or pitting or
notching of lid lamella.Presence of superficial
telengiectatic blood vessels on the surface of the mass
is characteristic of malignant eyelid tumors.The
important and common epithelial malignancies of the
eyelid are basal cell carcinoma, squamous cell
carcinoma and sebaceous gland carcinoma.
Basal Cell Carcinoma (BCC)

Basal cell carcinoma is the most common malignant
eye lid tumor in Caucasians (80 to 90%), while it
shares equal incidence with sebaceous gland and
squamous cell carcinoma in Japan and Asia (20 to
40%).33,34 Its incidence is increasing worldwide. 35
Incidence of BCC in caucasians is generally higher in
males than females. It occurs rarely before 20 years
of age, but age specific incidence increases thereafter
peaking between 40 to 80 years with an average age
of onset of 48 years.
Independent risk factors for BCC include red hair

and light skin color.36 Dynamic tanning and burning
reaction to the skin is also a clear predisposing factor.37
Other risk factors include ionizing radiation, arsenic
exposure, smoking history and patients with AIDS.38,39
Rare syndromes associated with BCC include
albinism, xeroderma pigmentosum, Gorlin's
syndrome, milia, spina bifida and syndactyly.
The most frequent site of involvement is the
lower lid (50 to 60%) and medial canthus (25 to 30%).
Upper lid (15%) and lateral canthus (5%) are less
frequently involved.39 In the early stages BCC appears
as a pearly, raised area, through which dilated
vessels are seen. They ultimately undergo ulceration
and present as destructive lesions which distort the
eyelid anatomy. It has an indolent and painless
course. Histologically, BCC can be classified as
nodulo-ulcerative (rodent ulcer), pigmented,
morphea or sclerosing, superficial, keloidal and
fibroepithelial variety (pinkuus tumor). It may cause
loss of eyelash and mimic chronic infection of eyelid
margin. The nodular variety is the most common and
it appears as a raised pearly nodule with telangectasia
and central ulceration. Histology shows nests of basal
cells that originate from the basal cell layer of the
epithelium with peripheral palisading. Morpheoform
lesions are firm and slightly elevated with ill-defined
margins. Histology shows thin cords which radiate
peripherally. Morpheoform is more aggressive than
nodular tumor. BCC is a slow growing tumor and it
is locally invasive. It rarely spreads to distal parts of
the body.40 Reported incidence of metastasis is 0.0028
to 0.55%.3 Choroidal invasion occurs at advanced
stages with median survival of 3 years at
presentation. Orbital extension is usually found with
inner canthal lesions (Figure 18.13). This is due to
delayed presentation or treatment and multiple
recurrence following incomplete excision.
Surgical excision is the most common mode of
treatment, followed by curettage and cautery,
cryosurgery and radiotherapy.41 Mohs micrographic
surgery as practised in many centers allow 100%
freeing of excision margin. Micrographic surgery
allows 99% success rate with tumor removal by
smaller margin.42
Cryosurgery is suitable for superficial lid tumors
and gives 92% five years cure rate.43 Curettage is
usually combined with cautery and usually requires

the characteristic intraepithelial or pagetoid spread
of the tumor which involves the basal layers of skin
and mucous membrane in a radial fashion. It can also
present as thickening of the lid on the tarsoconjunctival border with areas of conjunctival
injection.
Figure 18.13: Basal cell carcinoma of eyelids with orbital

extension
multiple applications. A course of radiotherapy is

advocated for large tumors in older patients.
Photodynamic therapy and carbon dioxide lasers
have emerged recently. Percutaneous delta aminolaevulinic acid is used with exposure to light in the
620-670 nm range. The success rate of photodynamic
therapy in superficial BCC is 92 to 100%.44 Other
modalities of treatment include intra-lesional and
perilesional interferon 2 band 5-flurouracil.45,46
Management of basal cell carcinoma with orbital
invasion should be very aggressive. Radical surgery
includes wide excision under frozen section or Mohs
micrographic surgery technique. It may require
radical surgical procedures such as orbital
exenteration, bony removal and even craniotomy.
Sebaceous Carcinoma of the Eyelid

Sebaceous gland carcinoma has an incidence of 1-5%
of all eyelid malignancies.2 It has a much higher
prevalence amongst the Asians and especially those
of Indian origin. It occurs with increasing frequency
in advancing age. The upper lid is most commonly
involved in approximately 2/3rd of cases, followed
by lower lid involvement and rarely the caruncle.
Sebaceous gland carcinoma of eyelids is aggressive
with a tendency for widespread metastasis. Clinical
diagnosis at an early stage has a wide differential. It
may masquerade as chronic blepharitis, blepharoconjunctivitis, chalazion, keratoconjunctivitis or basal
cell carcinoma. 47 The blepharoconjunctivitis
associated with sebaceous gland carcinoma is due to
Histopathologic features of sebaceous gland

carcinoma include the presence of cells of sebaceous
origin confirmed by lipid stains (oil red O) on fresh
tissue specimen. The tissue shows varying degrees
of sebaceous differentiation and infiltration with loss
of normal architecture of the gland which is replaced
by pleomorphic cells with prominent vacuolated
cytoplasm with high mitotic activity. The degree of
differentiation starts from the periphery and
progresses towards the center. Differentiated cells
are vacuolated or foamy with slight basophilic
cytoplasm. However, less differentiated tumor cells
are more deeply basophilic and are anaplastic.
Minimally invasive tumors are composed of lobules
of varying sizes with minimal tumor extension.
Invasive tumors are composed of diffuse cords of
cells with minimum lobule formation that extends to
the stroma. Rao and coworkers reported a histologic
variant of sebaceous gland carcinoma called
comedocarcinoma in which the tumor lobules
undergo central areas of necrosis.48
Orbital extension of sebaceous gland carcinoma
has an incidence varying from 6 to 35% and is
associated with a 70 % mortality rate. There is 70 %
involvement of preauricular, cervical and submaxillary lymph nodes. Lymphatic spread of the
tumor may occur to lung, liver, skull and brain. The
overall 5 year mortality is approximately 15%.2
The management of sebaceous gland carcinoma
is surgical removal of the tumor under frozen section.
Rarely, surgical extirpation enhances the long-term
prognosis of the tumor (Figures 18.14A to C and
18.15A to E).
Patients with widespread lid involvement or
orbital extension require exenteration. These patients
require very close follow up with map biopsies
because of its multicentric presentation and pagetoid
spread. Involvement of the adjacent lymphatic gland
requires radical resection. Role of radiotherapy in
management of sebaceous gland carcinoma is very
limited. Though the tumor is radiosensitive and
Figure 18.15A: A case of Sebaceous gland carcinoma presenting

with thickening of lower lid on the right side for 6 months
Figure 18.14A: A case of sebaceous gland carcinoma
Figure 18.14B: Lid Reconstruction with Cutler Beard surgery
Figure 18.15B: Lid reconstruction with Hughes surgery
Figure 18.14C: 2 months postoperative
Figure 18.15C: 2 months postoperative
Figure 18.15D: 6 months later there was a mass in the orbit medially
and HPE revealed a sebaceous gland carcinoma. This must have
occurred due to incomplete removal of the primary tumor
papilloma virus55 exposure to ultraviolet radiation,56

immunodeficiency due to immunosuppressives
following organ transplantation57 and exposure to
arsenic.50 People with xeroderma pigmentosum, a
condition characterized by defective DNA repair
following UV radiation damage to DNA and
oculocutaneous albinism are also predisposed.58
Squamous cell carcinoma of the eyelid (Figure
18.16) can arise de novo in relatively normal skin or
from actinic keratosis. 59,60 The mean age at
presentation is around 60 years and is found to be
higher amongst the males.61 It shows a tendency
towards involving the lower lid and lid margin.50
Local spread of the tumor is more aggressive than
basal cell carcinoma and it may also metastasize to
regional lymph nodes (Figures 18.17A and B).50
The lesions present as firm hyperkeratotic
papules or plaques with erythema of the surrounding
skin and induration; ulceration may be present.
The tumor may spread perineurally and this is
associated with a higher incidence of recurrence and
metastasis.50,62 Orbital involvement from squamous
cell carcinoma may occur due to neglected, longstanding tumors 63,64 and occurs in 2.5 to 5.9% of
cases.60,64 Presentation may be with pain or restriction
of globe movement.
Poorly differentiated carcinomas, those arising
from scars, tumor size > 2 cm and regional node
involvement are associated with increased risk for
recurrence or death.65 Metastasis occurs in 1-21% of
eyelid SCC.66
Figure 18.15E: Secondary reconstruction was done with removal of

the orbital mass along with excision of the medial part of the eyelid
and medial canthus with reconstruction with forehead flap
responds to radiotherapy initially the recurrence rate

is high. Thus radiotherapy is indicated in patients in
whom surgery is not possible.
Squamous Cell Carcinoma of the Eyelid

Squamous cell carcinoma is the second most common
cancer among whites.49,50 The relative incidence of
BCC to SCC varies from 13:1 to 40:1.51
Risk factors for developing squamous cell
carcinoma include increasing age, 51 fair skin, 49,50
history of sunlight exposure during childhood with
a tendency to sunburns,52 PUV-A therapy,53,54 human
Figure 18.16: Squamous cell carcinoma of the eyelid
B
Figures 18.17A and B: Extensive squamous cell carcinoma of the eyelid with extension into the conjunctiva and the orbit
Biopsy is required for confirmation of diagnosis.

Usual treatment consists of the excision of the tumor
with monitoring of the margins by Moh's micrography or frozen section. Local control with Moh s'
surgery is 96.9% at 5 years.67
Radiotherapy may be preferred for patients who
are unable to tolerate surgery or who have inoperable
tumors. The five year tumor control rate with
radiotherapy was 93.3%. Irradiation was equally
successful in primary cases and in those recurrent
following surgery.68 Cryotherapy may be used for
tumors of large size to reduce their vascularity and
size so that surgical excision and repair can be carried
out with better surgical and functional results. It is
also useful as a primary mode of therapy for small
tumors with a wide base or those situated at the lid
margin. 69 Exenteration is required for tumors
extending to the orbit.70
Other eyelid tumors that can extend into the orbit
are malignant melanoma of eyelid and Merkels cell
tumor.
survival experience. 71 Based on the clinical and

histological features the cutaneous melanomas are
classified as superficial spreading, acral, nodular and
lentigo melanoma. Of all the melanomas involving
the eye and the adnexa, the melanomas involving
the eyelid margins and conjunctiva have worse
prognosis.72 Orbital extension of cutaneous or eyelid
melanomas constitute less than 1% of secondary
orbital tumors. Treatment is tailored to the size and
extension of the tumor. The management of
malignant melanoma of the eyelid is surgical removal
Malignant Melanoma of Eyelid

Malignant melanoma of the eyelid is very rare. It
may either arise denovo or from preexisting precursor
lesions like lentigo maligna or Hutchinson's melanotic
freckle, dysplastic naevus and giant naevus. In the
last 10 years we had come across only 4 cases of
melanoma of eyelid and only one case had orbital
extension (Figure 18.18). The relative risk of
malignant melanomas is extremely low for AfroCarrabian. Intra-orbital melanomas have the worst
Figure 18.18: Eyelid melanoma with orbital extension

of the tumor under frozen section control. Rarely,
surgical extirpation enhance the long-term prognosis
of the tumor. Patients with widespread lid
involvement or orbital extension requires exenteration. Our case of malignant melanoma of the
eyelid with orbital extension was managed by total
exenteration and socket reconstruction with
temporalis muscle transfer.
Orbital Extension of Intracranial Tumors

The orbit is very rarely invaded by tumors other
than meningiomas from the intracranial cavity. As a
result this section will mainly focus on the
management of meningiomas. Other tumors that do
invade the orbit include parasellar tumors such as
pituitary tumors and craniopharyngiomas.1,2,73,74 These
tend to involve the superior orbital fissure and optic
nerve foramen resulting in compression of structures
at these sites.
Gliomas are the most common intracerebral
malignancy. However, they rarely involve the orbit.
It is only the high grade and aggressive glioblastoma
multiforme that may invade the orbital contents
either directly or via the foramina. Unfortunately,
prognosis is poor for these tumors. External beam
radiotherapy may temporarily halt growth and
reduce short-term visual deterioration, but this
treatment is only palliative.3/75
Meningiomas account for approximately 18 % of
all intracranial neoplasms.4,76 Annual incidence is
approximately 2 per 10000 per year afflicting females
twice as often as males.5,77 Meningiomas originate
from the meningoepithelial cells of the arachnoid villi.
Consequently, they tend to originate at sites where
the arachnoid villi are present. These include
parasagittal regions, cavernous sinus and foramen
magnum. The sites of interest with regards to orbital
extension include the sphenoid wing, lamina cribrosa
and tuberculum sella.6,78 There is still some debate as
to the etiology of these tumors. Various theories have
been proposed including trauma or viruses.
However, as yet only ionizing radiation 7,79 and
deletions of chromosome 22 have been accepted.8,80
The alterations of chromosome 22 mean that there is
also a link to neurofibromatosis type 2. It has also
been noted that some meningiomas express
progesterone receptors and that there is a
preponderance for meningiomas in female breast
cancer patients.9,81 Thus there may also be a hormonal

driving force towards growth.
Meningiomas have been classified into 15
subtypes under the WHO classification system.10,82
94% are thought to be benign, 5% atypical and 1%
malignant.11,83 As the majority are benign they mostly
exert their effects locally by compression or more
distantly through mass effect or raised intracranial
pressure. Consequently, it is apparent that the
structures affected will be dependent on the site of
the tumor.
Orbital invasion most commonly presents with
early unilateral painless visual loss. The visual loss is
usually gradual, but in less than 5 % of cases may be
acute.12,84 Other symptoms may include headache,
nausea and diplopia. Examination may reveal optic
atrophy or papilloedema (Figure 18.19A).
Meningiomas also display bone involvement
showing hyperostosis and less frequently lysis. This
can be visualized well with CT. MRI can be used to
define the involvement of local structures (Figure
18.19B). With the increasing availability of 3
dimensional reconstruction planning of therapy is
now much easier.
The aim of surgery is to completely excise the
tumor (Figure 18.19C). If this is not possible then
aggressive debulking should be attempted with
Figure 18.19A: Intracranial meningioma with orbital extension
Figure 18.19B: CT scan showing a mass in the temporal fossa with

extension into the orbit and shows hyperostosis of the temporal
bone, sphenoidal bone and inferolateral orbital bones
postoperative radiotherapy. A combined surgical

approach can be performed with a neurosurgical
craniotomy and an ophthalmic orbitotomy. Ideally,
both neurosurgical and ophthalmic teams are
involved, although increasingly excision is performed
solely by neurosurgeons. Orbitotomy sites vary with
position of extension of the meningioma into the orbit.
Whilst craniotomies can be fronto-temporal,
pterional, supraorbital ridge or subfrontal. For cases
in which an operative approach may not be suitable
external beam radiotherapy(EBRT) or IMRT can be
applied. Newer treatments such as chemotherapy
with hydroxyurea seems to have achieved little
success in tumor debulking.13/85 Whilst hormonal
therapy with anti-progesterone receptor agents
seems to have had some success with selected
tumors.14/86
Final success depends on recurrence. The
recurrence rate of meningiomas varies between 10
and 20%. They may recur upto 20 years after initial
excision and yearly close follow up with CT scanning
is advocated. 15,87 Recurrence tends to yield large
bulky tumors that affect local structures and require
extensive debulking thus resulting in an increased
morbidity.
Figure 18.19C: 1 week postoperative following removal of the

meningioma by orbito-temporal approach
Orbital Extension of Conjunctival Tumors

Squamous cell carcinoma of the conjunctiva
The incidence of conjunctival squamous cell carcinoma
is 1-2.8 per100, 000.88 It affects the elderly with a mean
age of 60 year. Over 70% of the patients are male.88,89
Sunlight exposure due to UV-B radiation90 is a risk
factor.91 HPV has also been implicated especially in
types 16 and 18.92,93 Ultraviolet induced mutations in
TP53 have also been implicated. It occurs at a younger
age and is more aggressive in patients with immunosuppression.
The lesion occurs commonly in the interpalpebral
area, with the majority being perilimbal. The
presentation varies from a gelatinous mass or nodule
to a flat patch of leukoplakia or a diffuse invasive
lesion (Figure 18.20A).88,94 The commonest presenting
symptoms are red eye and ocular irritation. 95
Squamous cell carcinoma mimicking sclerokeratitis
has also been reported.
The tumor may involve the underlying sclera in
one third of the cases with intraocular involvement
in 11-13% and orbital invasion in 11-15% (Figure
18.20B).87,95 Risk of orbital involvement may be higher
with mucoepidermoid variants. 96 Impression

cytology may aid in the diagnosis by displaying
keratinized dysplastic cells, hyperkeratosis,
syncytial-like groupings and prominent and large
nucleoli.97 UBM can show intraocular extension of
conjunctival squamous cell carcinoma (Figure 18.20C).
Management of OSSN requires adequate excision
and careful follow up to monitor any recurrence.98
Recurrence following excision depends on histopathological grade of tumor and involvement
of surgical margins. 99,100 Cryosurgery, topical
5-fluorouracil or mitomycin C treatment following
surgical excision has been shown to decrease the

recurrence rate.101,102 Topical interferon 2b has been
used in the treatment of presumed recurrent corneal
and conjunctival intraepithelial neoplasia.103 Once the
orbit is involved exenteration may be required.
Malignant melanoma of the conjunctiva has an

incidence of 0.2 to 0.8 per 1000 in whites104-106 and
comprises 2-3% of ocular tumors 107,108 and is less
commonly seen in the pigmented races.106 It has
Figure 18.20A: Conjunctival squamous cell carcinoma (SCC) with

360 involvement of limbus
Figure 18.20B: Scleral necrosis and invasion by the

conjunctival SCC
Malignant Melanoma of the Conjunctiva
Figure 18.20C: UBM showing intraocular extension of conjunctival SCC in ciliary body region

increasing preponderance with age109 and arises de
novo or from a nevus. However, most commonly110
these develop from primary acquired melanosis
(PAM) with atypia (Figure 18.21). 111,112 The most
common site of origin is the limbus.104 Other sites
include the fornices, palpebral conjunctiva and
caruncle (Figures 18.21A and B).
Folberg R and Co-workers had reported that 50%
of PAM with atypia may progress to malignant
melanoma. 113-115 PAM appears as flat, patchy
pigmentation in the conjunctival epithelium and can
remain dormant for years or show slow progression
or may wax and wane. 116,117 Development of
thickening within areas of PAM may indicate invasive
growth. However, a junctional nevus may be
indistinguishable from PAM with atypia histologically. Junctional nevi are seen in childhood while
PAM occurs in middle-aged and elderly individuals.
Biopsy of lesions that are widespread, large,
thickened, dark, palpebral, unusually vascular or
progressive has been suggested.118
Conjuctival melanoma arising from pre-existing
nevus119,120 may be characterized by change in color,
nodularity or bleeding. The 10 year survival rate in
one series was 77.7%. Unfavorable tumor location
(palpebral conjunctiva, fornix, caruncle, corneal
stroma, eyelid), age greater than 55 years, higher
TNM category, tumor thickness more than 10 mm,
high mitotic index and more epitheloid cells were
associated with higher risk of tumor related death.
Tumors with unfavorable location, higher TNM
grade, and excision alone as initial therapy had a
Figure 18.21A: Conjunctival malignant melanoma
higher probability of local relapse than favorably

located (bulbar and limbal conjunctiva) tumors, lower
TNM grade, and excision plus adjuvant therapy.121,122
Orbital extension of malignant melanoma of
conjunctiva occurs far less frequently when compared
to intraocular melanomas in the ratio of 4:23.123
Treatment includes extensive tumor removal,
cryotherapy and topical mitomyin C with amniotic
membrane allograft for diffuse conjunctival and
corneal melanoma arising from primary acquired
melanosis (Figure 18.20B).124
As primary and adjuvant therapy, topical
mitomycin yielded an overall recurrence rate of
50%.125 Survival rates were found to be worst for
those with intraorbital spread.107
Orbital Extension of Tumors of the Nasal Cavity

and Paranasal Sinus
Malignant tumors of this region are a diverse group
of neoplasms. They are relatively rare and as a result
few definitive studies have been performed into
their management. The studies that have been
undertaken illustrate that prognosis and management
vary depending on the type and site of tumor
diagnosed.
Figure 18.21B: Postoperative total excision of conjunctival malignant

melanoma with application of cryotherapy to the conjunctival margins.
5 years postoperative without any recurrence

Growth is often large prior to symptoms from
these tumors, partly due to the nature of the cavities
from which they arise. Since only a thin bony wall
separates the nasal cavity and sinuses from important
structures, invasion of nerves, brain and orbit are
frequent.
These tumors are more commonly found in males
with 80% presenting between the ages of 45 and 85.
There is an increased risk in people living in
developing countries.126
Due to the non-specific nature of presentation
diagnosis is often difficult. Patients may present with
nasal obstructive symptoms, rhinorrhea, epistaxis
and less commonly pain. If the orbit is involved then
proptosis and ocular motility may be affected
depending on the site of the original tumor. Vision
is usually affected later unless the orbital apex is
involved whilst conjunctival congestion may result
from a larger mass in the orbit. Thus when nasal
symptoms are persistent cases should be investigated
further. Endoscopy and more detailed radiography
have indeed improved earlier pick up. The maxillary
sinus is the most common site of primary tumor
origin (55%) (Figure 18.22), followed by the nasal
cavity (35%) and then the ethmoids (9%). The
sphenoidal and frontal sinuses are rarely involved
but when they are they confer a poor prognosis.2,127
The majority of tumors are squamous cell
carcinomas (80%) 127,128 with adenocarcinoma and
adenoid cystic carcinoma making up the majority of
the rest. The orbit is thus also most frequently
invaded by squamous cell carcinoma. Routes include
the foramina, perineural spread or direct invasion
via bony erosion. Lymphatic spread is however rare.
Squamous cell carcinomas most commonly arise
from the turbinates. Consequently, they present more
commonly with signs of congestion and nasolacrimal
duct obstruction. Up to 80 % have bony erosion of
the orbit at the time of diagnosis.129 Nickel and
chrome workers have a higher incidence of squamous
carcinomas.130 It is also increasingly thought that
smoking is a risk factor for development of these
tumors.131 Biopsy is often important as the various
different forms of squamous carcinoma confer
different modes of spread and hence affect prognosis.
Adenocarcinoma is more common in woodworkers and is linked to other chemicals for instance
those found in the leather tanning industry. 132
The prognosis from these tumors varies with

histological grading. They commonly invade the base
of skull and the orbit affecting vital structures.
However, distant metastasis is rare. Undifferentiated
carcinomas have a very poor prognosis, usually being
picked up at a late stage. Unlike squamous carcinomas
they are commoner in women than men. Small cell
carcinomas are similar to their lung counterparts and
may present with early proptosis, epistaxis and
obstructive symptoms.
X-ray was the initial tool for diagnosis and is
still useful in determining location, size and presence
of bony erosion. 129 However, it has now been
surpassed by computed tomography which
highlights bony involvement. MRI T2 weighted helps
differentiate tumor from surrounding tissue and
edema. Bowing of tumor-fat interface in fat saturated
images gives a clue as to invasion. If this changes
from smooth to irregular, invasion is far more
likely.133
Treatment of nasal and sinus tumors usually falls
beyond the domain of ophthalmologists. Specialists
from the fields of head and neck surgery and maxillofacial surgery provide the best hope for patients.
Surgeons with skills in facial reconstruction may also
help the patients once treatment is completed. There
is still some debate over the best modality of
treatment partly due to the lack of adequate research.
However, in a recent meta-analysis, surgery alone
seemed to be better than combination with radiation.
Worst of all appeared to be radiation alone.134 Often
radiotherapy is given to help reduce the size of the
tumor prior to surgery, however, as yet there is little
evidence that this improves long-term survival. Thus,
surgery with wide local margins still seems to be
preferred for low stage cancers and surgery with
some adjunctive radiotherapy used for higher stage
cancers. Different approaches have been used to gain
access dependent on the size and position of the
tumor. Recently, endoscopic approaches have been
used to reduce postoperative morbidity with
complete transnasal resections, although this is still
a developing field.135 Another area of debate is the
role of exenteration with orbital involvement. Studies
have shown that preservation of the eye has not
affected survival rates. 136 Indeed if adequate
reconstruction is performed a good functional
outcome can be achieved in up to 90% of cases.137

However, we recommend orbital exenteration with
involvement of the orbital apex, extraocular muscles,
bulbar conjunctiva and sclera.Radiotherapy has been
used for some time in the treatment of head and
neck tumors. However, high doses of radiation are
required for successful treatment. Since the tumors
are found close to structures that are highly
radiosensitive, morbidity is often high following
treatment. In a review of 48 patients treated with
curative radiotherapy 33% developed unilateral
blindness whilst 4 developed bilateral blindness
secondary to optic nerve damage. 138 However,
irradiation can be used in rarer instances when
cervical lymph node spread has occurred.
Chemotherapy is usually reserved for certain
types of tumor. These include lymphoma, neuroendocrine, enthesioneuroblastoma and undifferentiated carcinomas. The trouble in the past has been
the systemic side effects. However, increasingly a
local approach has been attempted with some success.
Prognosis from a recent review suggests an
overall survival of around 40%. Prognosis varied with
site and type of lesion. Nasal fared better than
ethmoidal which in turn had better survival than
maxillary. There was also a worse prognosis for
frontal and sphenoidal involvement due to increased
invasion of the brain and dura. Glandular carcinomas
had a better prognosis than squamous carcinomas.134
Figure 18.22: Carcinoma of maxillary sinus with involvement of

paranasal sinus,nasal cavity, the orbit and surrounding adnaxae
leading to extreme lateral displacement of the right orbit
Other studies have shown that although orbital

involvement is not detrimental per se, and deep
orbital involvement leads to a poorer survival, hence
the advice for exenteration when these tissues do
become involved.139/140
Orbital Extension of Nasopharyngeal Tumor

Although nasopharnygeal tumors are found in close
proximity to those of the nasal cavity, they have an
altogether different pattern of presentation partly
because of the vast lymphatic network present in the
region. They are almost a distinct entity affecting a
generally younger age group, between 45-55. There
is a higher incidence amongst males with the highest
incidence amongst the southern Chinese populations.
Here 15-30 males per 100,000 are diagnosed with the
condition per year. However, in other parts of the
world the disease is relatively rare. There is thought
to be a genetic element as the risk continues to remain
high even in second generation Chinese emigrants
despite the change of environment factors. 141/142
Epstein-Barr virus has been implicated as the
environmental agent as carcinoma cells have been
found to contain viral DNA.143 Changes in the make
up of these cells are thought to result in squamous
cell carcinoma. Unlike nasal tumors they are far more
likely to incur systemic spread.
Patients tend most commonly to present with a
neck mass secondary to regional lymphnode
metastasis. This is often linked with dysphagia. Other
symptoms include nasal symptoms such as rhinorrhea
and nasal congestion, auditory problems such as
hearing loss, fullness and recurrent otitis media. If
the orbit is involved then symptoms may include
proptosis, reduced vision, ocular motility disorders
and pain. The orbit is rarely involved and is usually
affected later than other structures.144 Direct invasion
through bone is rare. However, common routes
include pterygopalatine fossa, inferior orbital fissure
and then secondarily through the paranasal sinuses.
Either CT or MRI can be used to help detect the
pathway of tumor entry into the orbit.145
Unlike nasal and paranasal sinus tumors surgery
plays only a minor role in the management of these
tumors, because local metastatic spread is already
likely to have occurred. Thus radiotherapy is the
primary treatment modality for orbital invasion.
Chemotherapy is used as an adjunctive treatment for

advanced regional metastasis or more distant spread.
Recent reviews of cases have shown that orbital
involvement confers a particularly bad prognosis. The
5 years survival of a recent case series of
nasopharyngeal cancer with orbital extension was
only 28%.146
Metastatic Orbital Tumors

Metastasis to the orbit occurs secondary to
hematological spread of a primary tumor. Of all the
orbital cases 1.5 to 3.3 % are metastatic of which 7 %
cases are bilateral. In the pediatric age group, the
common primaries include neuroblastoma, Wilm's
tumor and Ewing's sarcoma. In adults, the common
primary sites for metastatic tumors to the orbit are
from breast, prostate, lung, gastrointestinal tract,
kidney and cutaneous melanoma. Most commonly,
the patient has a known primary tumor. The most
common malignancies that metastasize to the orbit
are carcinoma of the breast in females and carcinoma
of the lung in males.147,148 Metastases to the orbit have
also been reported to occur from carcinoma of
the tongue, pancreas, gallbladder, cervix, penis,
urinary bladder, thyroid intestinal carcinoid, renal
cell carcinoma and carcinosarcoma of the parotid
gland.149-157
Orbital metastasis may also be the first
manifestation of a systemic malignancy. Metastases
to fat and bone are twice as common than muscle.
Usually the primary tumor is known except in about
one quarter of patients. Certain carcinomas like
melanoma and breast carcinoma are likely to have a
known primary.
Average survival is approximately 9 months from
the orbital presentation. The first manifestation of
symptoms due to orbital metastasis usually occurs
after a mean period of 31-64 months after diagnosis
of primary disease, but tumors such as breast cancer
and thyroid cancer have a longer delay (3-5 years).
Melanoma is intermediate (2 years) and those from
the lung and gastrointestinal tract are diagnosed
shortly before or after orbital presentation. The mean
age of the patients was 64 years in one study.147
These tumors present similarly to others
involving the orbit. Symptoms include lid swelling,
red eye, pain, proptosis and diplopia and signs
include incomitant strabismus with diplopia,
blepharoptosis, decreased vision, proptosis and a
palpable mass.158,159 However, motility disturbance

out of proportion to the proptosis is characteristic of
an orbital metastatic tumor. Unusually, enophthalmos
has been reported with schirrous carcinomas of the
breast whilst metastases from the prostate to the orbit
are usually osteoblastic in nature. 160,161 Renal cell
carcinoma has a tendency to induce hemorrhage and
consequently it may initially be difficult to
differentiate from orbital hematoma.156 Diagnosis of
metastatic origin follows simple lines. Site of the
original tumor may also be ascertained with
symptoms and signs suggestive of other systems
being involved. Investigations may include nonspecific tests such as carcinoembryonic antigen (CEA)
which is elevated in metastatic disease of the bowel.
Other specific tests may be refined with regards to
the primary tumor suspected. Both CT and MRI have
a place in management. Both hyperostotic and
spiculated CT appearances have been described.147,149
MRI may assist in soft tissue differentiation and to
assess spread. Ultimately, however, a fine needle
aspiration biopsy may be required to confirm the
diagnosis in situations where an orbitotomy is not
possible.162
The survival rate of these patients is poor and
consequently treatment is mainly palliative and is
aimed at providing symptomatic relief 163 with
radiotherapy, hormonal therapy, chemotherapy and
surgery depending on the type and stage of the
disease.
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Decision Making 271
19
Decision Making
CHAPTER
The etiology of proptosis is multifactorial. As we

know it can be congenital, or acquired due to various
causes like infections both nonspecific and specific
bacterial like tuberculosis,1-4 sarcoidosis,5-7 fungal
infections, inflammations, parasitic infestations,
vascular malformations, primary orbital tumors
arising from various tissues like nerves and their
sheaths, vascular tissues, mesenchymal, lymphoproliferative, bony lesions apart from secondary
involvement from the globe, paranasal sinuses and
the brain. Metastatic lesions from breast,8 thyroid,
prostate, GIT and other primary lesions
elsewhere9-12 were documented in the literature.
Involvement of Orbit in generalized lymphoproliferative lesions like systemic lymphomas,
leukemia is very well known. It is really surprising
to know the wide variety of pathology that can
involve such a small structure as orbit. It is not an
exaggeration that in the orbit one can come across
almost all the types of pathology that can involve
human body.
With such a wide spectrum of pathological lesions
causing proptosis, it is natural that the management
strategies also vary. The options include observation
and reassurance, medical management, surgery,
radiotherapy and chemotherapy. The surgical
procedures include biopsy, debulking, excision of
tumor/mass/cyst through various surgical
approaches, multi-speciality approaches along with
ENT/Neurosurgeon, and exenteration.The decision
depends upon accurate diagnosis of the nature of
the lesion (infective/cystic/tumor: benign/
malignant/Secondary/metastatic) and its location
and extent (intraconal/peripheral space/extension
to paranasal sinuses/intracranial extension).

A detailed clinical evaluation, and imaging with CT
scan/MRI of Orbit usually give us enough
information to plan proper management strategy.
Other investigations like serology, FNAC13,14, Squash
and IHC (Immuno-Histochemistry) are useful aids
in establishing the diagnosis and planning the
management. In this chapter I am outlining the
different management strategies and which to choose
from. The details of the management procedures are
described in the following chapters.
Medical management is indicated when proptosis
is due to orbital cellulitis, orbital myocysticercosis,
idiopathic orbital inflammation, most cases of thyroid
associated orbitopathy (the drug-trial for TAO is well
known), Wegener's granulomatosis, tuberculosis of
the orbit, fungal granulomas, in other words in all
the inflammatory and infective conditions. However,
drain an orbital or sub-periosteal abscess, along with
systemic antibiotics. The role of medical management
is discussed in a separate chapter.
Chemotherapy and radiotherapy have a great
role in the management of rhabdomyosarcoma,
lymphoma and other lymphoproliferative diseases,
retinoblastoma. They can also be used occasionally
in thyroid orbitopathy, idiopathic orbital inflammation. They are helpful in the treatment of other
malignancies like adenoid cystic carcinoma of
lacrimal gland, secondary and metastatic lesions.
These were discussed in a separate chapter.
For me, it is always very important to get at an
accurate diagnosis as early as possible, so that I am
not missing any serious condition. That is why, even
in a case of clinically suspected orbital cellulitis, I get

a CT scan as early as possible to make sure that I am
not missing other conditions like rhabdomyosarcoma,
retinoblastoma which can occasionally present as
orbital cellulitis. The CT scan also informs me if there
is an abscess which I have to drain, or it is only orbital
cellulitis which I can manage medically.
When the diagnosis is not definite, I prefer to do
a biopsy and get a histopathological diagnosis. The
tissue diagnosis can be by way of FNAC (Figures
19.1A and B and 19.2A and B), biopsy or intraoperative Squash smear examination. However I
personally don't perform FNAC on a lacrimal gland
tumor. Instead I wish to excise the tumor in toto and
plan further treatment depending on the
histopathological diagnosis.
Biopsy is indicated for histopathological
confirmation of clinical diagnosis (Figures 19.3A to
C) as in lymphoma, meningioma of optic nerve
sheath, rhabdomyosarcoma, so that the patient can
be referred to an oncologist. Biopsy is also indicated
when the clinical diagnosis is not definite (Figures
19.4A to E).
Figures 19.1A and B: FNAC showing malignant cells in whorl pattern,

suggestive of neuroblastoma. The details are better seen in higher
magnification. This patient presented with painful proptosis and
restricted ocular motility. CT scan revealed a mass lesion with bony
erosion. Diagnosis of metastatic neuroblastoma was made from FNAC
When I am planning excision of the tumor, the

approach depends on its location. If it is involving
surrounding structures like PNS, or brain, it will be
a multispeciality approach. However, FESS
(Functional endoscopic sinus surgery) is the best way
to manage fronto-ethmoid mucocele. If the tumor is
confined to the orbit, it will be dealt with by me
alone, the only exception being a small apical lesion,
for which I prefer a transcranial approach.
The orbital approaches to proptosis are lateral
orbitotomy approaches and anterior orbitotomy
approaches. The anterior orbitotomy approaches are
further divided into cutaneous approaches and
conjunctival approaches. When choosing a procedure
I consider the surgical exposure and also the cosmetic
result. Though I wish to hide the scar and give the
best cosmetic result, adequate surgical exposure of
the lesion is of paramount importance, which can not
be over-emphasized. I advise the beginners and the
occasional orbital surgeons to choose simpler
procedures first, and as they gain experience, they
can perform other complicated procedures. I wish
Figures 19.2A and B: FNAC showing cells with large, peripheral

nucleus and vacuolated cytoplasm arranged in clusters, from
metastatic ductal carcinoma of breast leading to proptosis
Figures 19.3A to C: Typical Salmon's patch of lymphoma arising from the upper fornix (A) and lower fornix (B).
Biopsy is indicated for histopathological confirmation (C)
Decision Making 273
Figures 19.4A and B: This patent presented with painful proptosis

of 1 month duration.He had exposure Keratitis and his vision was 20/
400. Note the severe swelling, tarsorrhaphy for exposure keratitis,
and chemosed conjunctiva. The CT scan shows a heterogenous
lesion surrounding the optic nerve. Radiologist reported it as
meningioma. The past history was significant that he had discontinued
treatment for tuberculosis.Since this type of acute/subacute
presentation is unusual for meningioma, I thought of infective pathology
( tuberculosis) and wanted to confirm the diagnosis by a biopsy
Figures 19.4C to E: When lateral orbitotomy was performed (C) a pinkish mass was found around the optic nerve, which was biopsied. The
histopathology showed it to be non-Hodgkins B-cell lymphoma (D) He was referred to an oncologist and he responded very well to radiotherapy (E)
to emphasize that usually there are more than one

option, and the procedure chosen varies from one
surgeon to other, depending upon the individual's
preference.
Intraconal Lesion: Lateral orbitotomy is
indicated for intraconal lesions and lacrimal gland
lesions. There are different approaches; the most
commonly performed are Stallard-Wright's
procedure, Reese-Berke's procedure and superior lidcrease incision (Figures 19.5A and B). Each has its
own merits. I will outline these approaches, before I
discuss my preferences.
Stallard-Wright's incision starts at the lateral third
to half, beneath the eyebrow, up to the lateral end
of the brow, and then descends vertically along the
lateral border of the orbit, and extends horizontally
along the crow's feet .Thus it has two horizontal
incisions, one at the level of sub-brow, and the other
Figures 19.5A and B: Lateral Orbitotomy incisions in frontal (A) and

lateral (B) views. Stallard-Wright (yellow), Reese- Berke (White) and
superior lid crease incision (pink)'
at crow's feet, which are well hidden. But the vertical

component of the incision leaves a visible scar. The
advantages of the incision include a very large and
adequate area of surgical exposure to deal with huge
tumors. The other advantage is that the lateral
canthus is not disturbed.
Reese-Berkes incision (Figures 19.6A to 19.9B)
is a horizontal incision that starts from the lateral
canthus and extends 4-5 cm horizontally along the
crow's feet. This incision gives a very good exposure
to deal with most of the tumors. The scar is very
well hidden in the crow's feet and surgical scar is
never an issue. The only disadvantage is that the
lateral canthus is disturbed and needs to be
reconstructed at the end of the surgery. For those of
you who are routinely doing oculoplastic procedures,
this is neither difficult, nor time consuming. In those
situations where the patient had a long standing and
prominent proptosis, you may even do lateral tarsal
strip at the time of reconstruction of the lateral
canthus and correct horizontal laxicity of the lid.
Superior lid crease incision (Figures 19.10A to
19.11C) is another excellent approach, where in the
incision is along the lid crease, and then extends along
the crow's feet. The entire incision is very well
hidden, so that the scar is not visible. It also gives a
very adequate exposure. The lateral canthus is not
disturbed. However, damage to Levator Palpebrae
Superioris leading to ptosis is a known complication.
Steps of Reese-Berke Approach
Figures 19.6A and B: Steps of lateral orbitotomy Reese-Berke's incision. Traction suture was applied to
lateral rectus (A). Horizontal incision made from the lateral canthus (B)
Figures 19.7A and B: Zygoma was exposed (A), cuts were made above and below and the zygoma was
being removed with a rounger (B)
Figures 19.8A and B: Periosteum was incised, and reflected (A). Lateral Rectus was identified. Traction
suture applied earlier helps in its identification. Lateral rectus was retracted away from the tumor (B)
Decision Making 275
Figures 19.9A and B: The tumor was removed with the help of cryo (A). After securing hemostasis, the Zygoma was replaced in its place
and secured. Drain is placed in a separate stab incision and not in the original incision. This gives a better scar. The wound is closed in layers
Steps of Superior Lidcrease Incision
Figures 19.10A to C: The incision is along the lid crease and extends horizontally along the crow's foot (A and B).
Carefully dissect up to the septum, and approach the lateral wall (C)
Figures 19.11A to C: The lateral wall is exposed (A), which has been removed. Periosteum was incised.
The tumor was exposed (B), dissected, and removed with the help of a cryo (C)

I believe that a scar on the face is a cosmetic
blemish. I wish to perform the orbitotomy leaving
as minimal scar as possible. The scar induced by the
vertical component of Stallard-Wright's incision is
of concern, and I wish to avoid it. Hence it was more
than 2 decades since I performed Stallard-Wrights
incision. My routine is Reese-Berke's approach.
It gives adequate exposure to deal with most of the
tumors (Figures 19.12A to 19.13C). If the tumor is
too big, then I perform superior lid crease
incision. The scar induced in these two procedures
is very well hidden and patient's satisfaction is very
high.
However it is very important to remember that
intraconal tumor can be excised by lateral orbitotomy
approach, only if it is lateral to optic nerve. If the

tumor is medial to optic nerve, it cannot be excised
through lateral orbitotomy as the optic nerve can
get damaged. In these situations, antero-lateral
orbitotomy is preferred (Figures 19.14A to 19.21B).
Hence the importance of assessing the relationship
of the tumor with optic nerve in coronal sections of
CT scan cannot be over-emphasized.
In antero-lateral orbitotomy, lateral orbitotomy
is performed to get space and better surgical
exposure. 180 peritomy is performed, the medial
rectus is disinserted, the globe is retracted laterally
and the tumor is removed between the globe and
the retracted medial rectus. Then the medial rectus
is reattached.
Figures 19.12A to D: Note the gross proptosis of right eye in this female, due to a very large cavernous hemangioma occupying
most of the orbit, as shown in the CT scan imaging. Such a large tumor could be excised through Reese-Berke's incision excised tumor (C) and
postoperative recovery (D)
Figures 19.13A to C: Another patient with a prominent proptosis of right eye due to a large neurofibroma, excised through Reese-Berke's
incision. Note that the incisional scar is very well hidden in the crow's feet
Decision Making 277
Figures 19.14A and B: Diagrammatic representation of anterolateral

orbitotomy for a case of axial proptosis (A) due to intraconal tumor
located medial to optic nerve (B)
Figures 19.15A and B: Lateral orbitotomy was performed with the

lateral wall removed. The medial rectus muscle was disinserted, with
double armed 6-0 vicryl sutures attached to the tendon
Figures 19.16A and B: The eyeball and the medial rectus were retracted to get adequate space for dissection of the tumor. The tumor was
excised through this space with the help of a cryo (A). The medial rectus was reinserted, conjunctiva was sutured. The lateral orbitotomy
incision was closed
Figures 19.17A to C: Female 45 years presented with painless, progressive proptosis of left eye of 3 years duration and progressive blurring
of vision since 6 months. She had RAPD with optic disc edema (A). Her BCVA was 20/200. CT scan (B and C) revealed a homogenous,
hyperdense tumor with very well defined borders in the intraconal space and medial to the optic nerve (red arrow B). There was no
enhancement on contrast. Since the tumor was intraconal and medial to optic nerve, anterolateral orbitotomy was planned
Figures 19.18A to C: Lateral orbitotomy was performed with Reese-Berke's incision. The Zygoma was removed, and periorbita incised to
facilitate moving the globe laterally (A). 180 peritomy was performed medially from12 to 6 o'clock position. Medial Rectus muscle was identified
(B), and disinserted after applying 6-O vicryl sutures (C)
A
Figures 19.19A and B: The globe is retracted laterally, and the disinserted medial rectus medially to get
adequate surgical space (SA). By careful dissection in this space, the tumor was identified (TB)
B
Figures 19.20A and B: The tumor was dissected carefully from the surrounding structures and
was removed carefully with the help of a cryo (A and B)
Decision Making 279
Figures 19.21A and B: The medial rectus muscle was anchored to its original incision (A). The conjunctiva was
sutured into its place (B). The lateral orbitotomy wound was closed as usual
Lesions of Superior Peripheral Space: Anterior

orbitotomy approaches are normally used for these
lesions, the exception being lateral orbitotomy for
lacrimal gland tumors. However I am excising
lacrimal gland tumors, up to moderate size through
anterior orbitotomy approaches.
For tumors of the superior peripheral and
subperiosteal spaces, both subciliary and superior
lidcrease incisions give very good surgical exposure
(Figures 19.22A and B). However the surgical scar is
better camouflaged in the lid crease and hence is my
preferred procedure (Figures 19.23A to 19.31C).
Apical conal lesions are better approached through

transcranial approach with the help of a neurosurgeon.
This was discussed in the concerned chapter.
Imaging studies tell us if we are dealing with
optic nerve glioma or meningioma of optic nerve
sheath. I prefer to follow closely a case of optic nerve
glioma with imaging every 6 months and perform
surgery only if the eye has become blind or the tumor
is nearing the orbital apex. For meningioma, I do
lateral orbitotomy and take a biopsy for histopathological confirmation (which is mandatory here)
before referring for radiotherapy.
B
Figures 19.22A and B: Superior Anterior orbitotomy incisions, subciliary (white) and
Lid-crease (yellow) incisions
Figures 19.23A to C: Female 38 years, presented with proptosis of left eye of 2 years duration. Note that the globe is pushed down and in,
with fullness of superior sulcus (A). CT scan of the orbit revealed lacrimal gland tumor pushing the globe down and in (B) Excavation of the
lateral wall of orbit could be seen (C)
B
Figures 19.24A and B: Lid-crease incision was made (A), the septum was identified and
reflected from the orbital rim (B)
B
Figures 19.25A and B: The lacrimal gland mass was removed with the help of cryo (A). The
excised tumor is shown in B
Decision Making 281
Figures 19.26A and B: Female 18 years, presented with progressive, painless proptosis of right eye since 8 years. Note the severe
proptosis of right eye with the eyeball pushed down and out. Note also the bluish mass lesion in the right upper lid (A and B)
Figures 19.27A and B: CT scan of the orbit shows multilobulated lesion, occupying most of the intraconal
space, pushing the optic nerve infero-laterally, and extending from the apex to the upper eyelid
Figures 19.28A and B: Superior lid-crease incision was chosen, since if needed, it could be converted to lateral
orbitotomy also. Note the tumor (T) on either side of the superior oblique (S.O) tendon. The tendon of superior
oblique was outlined yellow to facilitate recognition
Figures 19.29A and B: The tumor was excised carefully, without

damaging the superior oblique, with the help of cryo (A). Note that the
superior oblique tendon is intact (B), and also the cavity formed after
the excision of the tumor
Figure 19.30: The excised tumor (Cavernous hemangioma)
Figures 19.31A to C: A patient with cavernous hemangioma located in the anterior part of superior peripheral space being excised in toto
through superior lid crease incision. The incision in this situation can be smaller (A) The tumor is seen in B. Look how well the incision is
camouflaged in the lid crease (C) in the figure taken immediately after completion of surgery. The surgery was performed under local
anesthesia
For lesions of medial peripheral space,

the approaches can be percutaneous Lynch
incision or transconjunctival incisions (Figures 19.32A
and B).
Subcaruncular incision is popular for thyroid

decompression of medial wall, since it gives access
to ethmoid sinus. The scar is never an issue with
transconjunctival approaches.
B
Figures 19.32A and B: Lynch Incision (white line) and transconjunctival approaches
(subcaruncular incision blue line and subconjunctival peritomy incision redline)
Decision Making 283

large, Lynch incision is my choice (Figures 19.33A to
19.34B).
However, the beginner finds it a little difficult

with these approaches. Lynch incision is simpler, and
a better procedure for the beginner.If the lesion is
Figures 19.33A and B: Eccentric proptosis of right eye (A) progressing for the past 3 years. He has RAPD and optic disc edema. CT scan,
revealed a huge osteoma of ethmoid (B) Since the tumor is very large, the only practical approach is transcutaneous, modified Lynch incision
Figures 19.34A and B: The osteoma being removed through a modified Lynch incision (A) The excised osteoma is seen in (B)
Lesions of inferior peripheral space, can be

approached either through the skin or conjunctiva
Subciliary is more popular of the skin approaches.
It still leaves a scar, which is mostly cosmetically
acceptable. However, trans-conjunctival approaches

are without a visible scar (Figures 19.36A to 19.38B).
Swinging lower eyelid approach causes a scar which
is hidden in the crow's feet.
Figures 19.35A and B: The approaches for inferior peripheral space can be cutaneous like Subciliary incision (yellow line), or conjunctival
approach. The swinging lower lid approach (Pink line) is a combine of both skin and conjunctival approaches and gives adequate room for
surgery
Figures 19.36A to C: Note a large cystic lesion involving left orbit, pushing the globe up (A) Also note how brilliantly it is transilluminating (B).
The cyst was excised through transconjunctival approach. The result on first postoperative day (C) is satisfactory. There is no visible scar, and
the normal contour of the lid was restored
Figures 19.37A and B: This male, 24 years of age, presented with a painless swelling of 1 year duration. Examination revealed, a firm, nontender lesion in the inferior space, with orbital extension, pushing the eyeball up (A). The lid was everted, the conjunctiva and inferior retractors
were separated from the lower border of the tarsus (B)
Figures 19.37C and D: Dissection was carried in this plane, and the tumor was identified (C), and carefully dissected-out
and removed (D) I find Westcott Scissors and Hoskins forceps very useful in these dissections
Decision Making 285
Figures 19.38A and B: The conjunctiva and the inferior retractors are carefully reattached to the lower border of
tarsus with interrupted, 6-0 Vicryl buried sutures (A) Note that the normal contour of the lid was restored on the
first postoperative day. Note also that the position of the globe is back to normal, and there was no scar. This
surgery can be performed comfortably under local anesthesia
Swinging lower eye lid approach is one of my

favorite procedures for large lesions in peripheral
surgical space, for optic nerve decompression in
thyroid associated orbitopathy, and orbital
floor fractures. The advantages include an
excellent exposure of field of surgery, possibility of
3-wall decompression and very minimal scar
(Figures 19.39A to 19.43B).
Thyroid associated orbitopathy: In India, I find the
clinical presentation of thyroid orbitopathy less
aggressive than in western reports. This is the same
observation of almost all my colleagues in India.
However, I came across many Indians at Vancouver
whose presentation of thyroid associated orbitopathy
was as severe as in the Caucasians. The weather

conditions or the life style may be the reason for this
difference and requires a systematic evaluation.
In thyroid orbitopathy, steroids are indicated
when the patient has inflammatory symptoms or
diplopia. Optic nerve compression is another
indication, before surgical decompression is
performed. Radiotherapy with linear accelerators in
non-diabetics patients is an option when the
inflammatory signs and symptoms are controlled
with steroids. Surgery is usually in stages,
decompression followed next by muscle surgery for
squint/diplopia, and then lid surgery for
blepharochalasis/lid retraction.
Figures 19.39A and B: Female 55 years presented with bilateral proptosis of 6 months and defective vision of 3
months. She is hypothyroid and diabetic and has typical features of thyroid associated orbitopathy. Her BCVA was
20/200 20/400, and has severe optic disc edema
Figures 19.40A and B: CT scan of the orbits showed gross enlargement of the inferior, medial and
superior recti, sparing the tendons. Note the bilateral apical compression, which is evident in both axial
and coronal sections
Figures 19.41A and B: She underwent bilateral 3 wall decompression along with excision of fat
(about 6 cc) from each orbit by swinging lower eyelid approach
Figures 19.42A and B: Note the gross difference between the preoperative (A) and postoperative
(B) conditions. Note that the postoperative scar after swinging lower eyelid approach is practically
not visible. The patient's vision improved to 20/20 and 20/30
Decision Making 287
B
Figures 19.43A and B: The visual fields of right eye before(A) and after(B) orbital decompression showing marked improvement
REFERENCES
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2. Shome D, Honavar SG, Vemuganti GK, Joseph J. "Orbital
tuberculosis manifesting with enophthalmos and causing
a diagnostic dilemma" Ophthal Plast Reconstr Surg. 2006;
22(3):219-21.
3. Aversa do Souto A, Fonseca AL, Gadelha M, Donangelo I,
Chimelli L, Domingues FS, "Optic pathways tuberculoma
mimicking glioma: case report" Surg Neurol. 2003;
60(4):349-53.
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208-10.
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769-77.
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swelling and diplopia as presenting features of orbital
sarcoid"Indian J Ophthalmol. 2000;48(3):231-3.
7. Segal EI, Tang RA, Lee AG, Roberts DL, Campbell GA:
"Orbital apex lesion as the presenting manifestation of
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8. Ahmad SM, Esmaeli B. "Metastatic tumors of the orbit

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9. Sivagnanavel V, Riordan-Eva P, Jarosz J, Portmann B,
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11. McCulley TJ, Yip CC, Bullock JD, Warwar RE, Hood DL:
"Cervical carcinoma metastatic to the orbit", Ophthal Plast
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pelvic telangiectatic osteosarcoma metastatic to both orbits",
Ophthal Plast Reconstr Surg. 2004;20(1):77-9.
13. Heerema A, Sudilovsky D: "Mucinous adenocarcinoma of
the ovary metastatic to the eye: report of a case with
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14. Saikia B, Dey P, Saikia UN, Das A : "Fine needle aspiration
cytology of metastatic scalp nodules", Acta Cytol.
2001;45(4):537-41.
20
Orbitotomies
CHAPTER
Ramesh Murthy, Anirban Bhaduri, Vikas Menon, Santosh G Honavar
General Principles
Before undertaking any surgery in the orbit, a
thorough knowledge of the normal eyelid and orbital
anatomy is essential. In addition we always evaluate
every case starting with a thorough history,
meticulous examination, imaging and laboratory
investigations. This helps us to arrive at a differential
diagnosis. As a general rule, an excisional biopsy is
indicated for well circumscribed lesions or those
which are benign, while an incisional biopsy is
performed for a lesion which is suggestive of
malignancy or inflammation.
Thorough understanding of CT and MRI is a must
and we must emphasize that familiarity with surgical
approaches is essential. One should observe and assist
orbital surgeries before one embarks into this
independently.
One should also understand the instrumentation
needed for this surgery. When using electric saws
one needs to ensure safety of the patient's eyeball as
well as of the surgeon and the team. Protective
glasses should be worn along with face masks to
prevent inadvertent spillage of blood and bone
fragments. Use of retractors is required to provide
adequate exposure. One also needs to be careful not
to exert pressure on the globe when using the
retractors. Wright's and malleable ribbon retractors
are used not only to expose the tissues but also to
protect the surrounding tissues. Proper illumination
and adequate magnification is essential to visualize
the orbital structures.1
The key to safe surgery is good surgical exposure.
The surgical incision should be of adequate length.
This can be supplemented by traction sutures at
appropriate locations. While cosmesis is desirable,

safe and adequate surgical access is the aim. Patience
is required when dissecting lesions in the orbit.
Gentle blunt dissection is performed using a Freer
elevator or lens spatulas to separate the orbital mass
from the surrounding tissues. Repositioning of the
retractors is essential as the dissection proceeds. In
order to perform dissection, the blunt tipped Westcott
tenotomy scissors is used. The little finger is a useful
instrument to palpate the lesion and perform blunt
dissection. Gentle counter traction is needed when
dissecting an orbital mass. A cryoprobe can be used
to hold the mass and pull it gently while dissection
is going on.2
Adequate hemostasis needs to be ensured during
orbital surgery. Hypotensive anesthesia is advantageous. However the anesthetist should bring back
the blood pressure to normal following surgery, to
ensure adequate intraoperative hemostasis. Bipolar
cautery should be used in the orbit. When the source
of bleed cannot be identified, simple packing with
gauze should be performed. Indirect pressure over
the closed lids is also useful. Bone wax or gel foam
soaked in thrombin can also be used to stem bleeding
during orbital surgery.
For an accurate pathological diagnosis, it is
necessary to obtain a sample of adequate size,
representing the lesion and undamaged by cautery
or surgical instrumentation. Routine samples are
usually sent in formalin. If a fresh sample needs to
be sent or frozen section analysis is required, it is
best to inform the pathologist beforehand. The
requisition form sent to the pathologist should have
detailed clinical findings.
Orbitotomies 289
Approaches
Many approaches can be used to gain access to the
orbit.3 The various types of incisions are demonstrated in Figure 20.1.
The approaches can be
1. Anterior orbitotomy
a. Approach to the superior orbit
Benedict incision4
Upper lid crease incision
Byron Smith lid split incision5
b. Approach to the inferior orbit
Mc Cord swinging lower lid incision6
Subciliary incision
c. Approach to the medial orbit
Lynch incision
Gull wing incision
Transcaruncular incision
2. Lateral orbitotomy
3. Transnasal endoscopic approach
4. Transantral or CalwellLuc approach
5. Transfrontal orbitotomy.
Anterior Orbitotomy
This approach is useful for anterior orbital lesions,
for the drainage of hematomas and abscesses and
incision biopsy of posteriorly placed orbital lesions.
For superior lesions a transcutaneous approach

through the upper lid skin crease leaves a less visible
scar. However approach thorough the lower lid skin
can leave an unsightly scar. The Byron Smith lid split
incision which can be used to access large
superomedial lesions is not very popular.
A Lynch incision can be used to approach the
medial subperiosteal space. A transcaruncular
approach is also useful and may be combined with a
lateral orbitotomy.
For inferior orbital lesions a lower eyelid
transconjunctival approach is the least disfiguring.
Swinging Lower Lid Flap

The first step is to make a mark on the skin
horizontally at the lateral canthus. A bridle suture is
placed through the inferior rectus muscle and then a
mosquito artery forceps is applied to the lateral
canthus along the skin mark to provide hemostasis
when the incision is made. The skin is then incised
with a Bard parker knife or a Colorado needle. After
cutting the lateral canthus, inferior cantholysis is
performed. Two 4/0 silk traction sutures are passed
through the lower lid margin through the grey line
of the lower lid. The traction sutures are secured to
provide exposure. A conjunctival incision is then
made at the inferior border of the tarsus or slightly
lower starting laterally and then extending it
medially. The plane between the orbicularis muscle
Figure 20.1: Various surgical approaches for orbit

and the orbital septum is dissected to the inferior
orbital margin. Further traction sutures can be placed
through the conjunctiva and inferior retractors to
improve visualization. A Desmarre's retractor can
be used to give traction and expose the tissues. The
periosteum or septum is then opened. With the help
of retractors the lesion is exposed and blunt
dissection performed to separate the lesion and
perform a biopsy. Adequate hemostasis is ensured.
The edges of the periosteum are sutured with 6.0
vicryl interrupted sutures. The conjunctiva is closed
with interrupted 6/0 vicryl sutures and the lateral
canthotomy is repaired by using 6/0 prolene sutures
to secure the tarsus to the lateral orbital periosteum.
Closure is performed in 2 layers with 6/0 vicryl for
the soft tissues and 6/0 prolene for the skin.
Antibiotic ointment is instilled and a pressure
dressing is applied. The steps of this technique have
been demonstrated in Figures 20.2A to Q.
Lateral Orbitotomy
This is a useful technique for lesions in the intraconal
space and lesions lateral to the optic nerve. In
addition this is useful for large lesions anywhere as
this can be combined with other approaches to allow
the globe to be moved laterally for increasing surgical
exposure. The Berke-Reese incision disturbs the
lateral canthus and leaves a less satisfactory scar.7
The modified Stallard Wright approach is a good
approach. We are presently using a lid crease
approach which gives aesthetically pleasing results.
Stallard-Wright Lateral Orbitotomy8

The first step is to pass 4/0 silk sutures below the
insertions of the lateral and superior rectus muscles
and form a loop. This is tied to get a hold on the
muscle and for identifying the muscle as surgery
progresses to avoid any inadvertent damage to the
muscles. An incision is made on the skin. The incision
starts from just below the lateral aspect of the brow
and ends in a rhytid over the anterior zygomatic
arch. The skin incision is made with a no. 11 BardParker blade. Under stretch and lifting the tissues,
the subcutaneous tissues are dissected down to the
periosteum using a radiofrequency monopolar probe.
As this dissection proceeds, one must ensure that
adequate hemostasis is achieved by using a bipolar
cautery. Multiple 4/0 silk traction sutures are placed
to gain exposure. The periosteum is exposed over

the whole lateral orbital margin. The periosteum is
cut with a monopolar probe about 4 mm behind the
orbital rim starting superiorly and ending inferiorly
just above the zygomatic arch. Relaxing incisions need
to be given to the periosteum. The periosteum is then
reflected. The periorbita is also lifted away from the
orbital bone upto the anterior one third of the orbit.
This has to be performed with care to avoid any
breach. The zygomaticotemporal and zygomaticofacial vessels may bleed and may need to be
cauterized. The temporalis muscle also needs to be
separated laterally and reflected. This muscle is very
vascular and adequate hemostasis needs to be
ensured. Incision lines are made on the bone about 3
mm above the frontozygomatic suture superiorly and
just above the zygomatic arch inferiorly. A
Desmarre's retractor is placed to pull the skin and
subcutaneous tissue laterally and a lid guard is placed
inside the orbit to protect the contents of the orbit.
Using an oscillating saw, cuts are made along the
incision lines on the bone. Irrigation is performed as
the saw is being used. Drill holes may be made on
both sides adjacent to the bone cut. Once the cuts
have been made, the bone fragment is held with a
bone rongeur and moved back and forth until it
fractures posteriorly. It is then removed and wrapped
in wet saline gauze. The bone can be further nibbled
with a bone punch or removed with a burr for further
exposure. Hemostasis is essential especially in the
region of the temporal fossa. Bone wax may be used
to cover any bleeding points in the bone. A T shaped
incision is made in the periorbita. This is done with
a no 15 Bard Parker blade or a blunt tipped Westcott
tenotomy scissors. The incision is then extended
circumferentially. A nick is made posteriorly and the
periorbita at the cut edges is grasped and gently
spread apart to extend the cut posteriorly. Dissection
of the orbital mass is performed by blunt dissection.
Location of the orbital mass can be confirmed by
gentle palpation. Wrights retractors and malleable
retractors are used to gently retract the globe and
keep the orbital fat away from the area of dissection.
Hemostasis is achieved by bipolar forceps. The pupil
is checked at regular intervals.9 A cryoprobe can be
used to aid delivery of the lesion. If it is an
encapsulated lesion, dissection is performed close to
the capsule using a Freer elevator. Once the orbital
Orbitotomies 291
surgery has been completed, the periorbita can be
closed with 6/0 interrupted vicryl sutures. The bone
fragment may be replaced and either a wiring
through the holes is performed or the cut edges are
stuck with cyanoacrylate glue. The periosteum is then
closed with 6/0 vicryl sutures. The subcutaneous
tissues are apposed with 6/0 vicryl sutures and the
skin is closed with 6/0 prolene continuous sutures.
Intravenous steroids are preferably given at the end
of the procedure. A suction drain may or may not be
placed. A pressure dressing is placed after applying
antibiotic ointment. The steps of this technique have
been demonstrated in Figures 20.3A to S.
Transcarcuncular Approach
This approach through the conjunction for medial
orbital lesions is technically difficult but can give
better cosmesis than a skin approach. This can be
combined with a lateral orbitotomy for greater
exposure.
Transnasal Endoscopic Approach and Transantral Approach

This is best performed by or with the assistance of
an ENT surgeon and is especially useful for biopsy
of lesions near the orbital apex or arising from the
sinuses. This is also a useful approach to remove any
bone fragments that may be impinging on the optic
nerve following an orbital fracture. This is useful for
inferior lesions especially those arising predominantly
from the maxillary or ethmoidal sinuses for
performing a biopsy of these lesions.10
Transfrontal Orbitotomy11-13
This approach is performed to access lesions at the
orbital apex. A team approach including a
neurosurgeon is needed. There are potential
complications in this procedure especially ptosis and
extraocular muscle palsy.
A bicoronal flap is created. The frontal bone flap
is hinged laterally, still attached to the temporalis
muscle and pericranium. This provides good exposure
of the medial, superior and lateral orbital apex. If the
lesion is confined to the orbit, an extradural approach
is undertaken. For orbital lesions extending into the
cranium, an intradural approach is needed. The orbital
roof is removed, keeping the periorbita intact. The
frontal nerve is an important landmark, which runs
anteroposteriorly over the levator muscle. Entry into

the orbit is made medially avoiding the area of the
superior orbital fissure. The orbital roof is reconstructed by using an alloplastic material or using the
inner table of the frontal bone flap.
Complications
This is a major surgery and there can be
complications.14
1. Vascular
a. Bleeding
b. CRVO, BRVO
c. Vitreous hemorrhage
d. Short posterior ciliary artery occlusion
2. Neural/Muscular
a. Corneal anesthesia
b. Internal ophthalmoplegia
c. Extraocular muscle paresis
d. Lateral rectus adhesion
e. Ptosis
f. Optic neuropathy
g. CSF leak
h. hypoesthesia
Postoperative Management
The patient is advised bed rest with the head elevated
and advised not to strain to prevent increase in the
venous pressure. Steroids and anti-inflammatory
medication is prescribed. Systemic antibiotics may
be prescribed. The vision, pupil, ocular motility and
fundus is assessed.
CASE ILLUSTRATIONS
Case 1
(Swinging lower lid incision of McCord)
A 7-year-old girl presented with complaints of a
palpable mass below right eye, gradually
increasing in size over 3 months (Figure 20.2A).
Clinically a firm to hard mass was palpable in the
anterior inferior orbit (arrow indicates the
inferomedial orbital mass) (Figure 20.2B). The mass
was immobile and non-tender. The mass was causing
superior displacement of the globe. Anterior segment
and fundus examination were normal except for
indentation effect of the mass on the globe, seen
inferiorly. There was no limitation of ocular
movements.

CT scans showed an ill defined, hyperdense,
extraconal mass in the inferomedial quadrant of the
orbit with indentation of the globe and distortion of
the medial wall of the orbit (Figure 20.2B).
Excision biopsy of the mass was planned and was
done through an anterior orbitotomy approach using
a swinging eyelid incision and the mass was removed
completely (Figures 20.2C to Q).
Steps of Surgery
Figure 20.2C: 4/0 silk sutures are passed through the lower lid
margin, one near the lateral canthus and one centrally
Figure 20.2A: A 7-year-old girl presented with a mass below the

right eye (arrow) with history of gradual increase in size over 3
months
Figure 20.2D: Mark is made on the skin horizontally at the lateral

canthus and a lid speculum is placed. An artery forceps is used to
crush the tissues along the mark
Figure 20.2B: CT scan (coronal section) revealed an ill defined

hyperdense, inferior orbital mass in the right orbit (arrow)
Figure 20.2E: A lateral canthotomy is performed with scissors
Orbitotomies 293
Figure 20.2F: Inferior cantholysis is performed
Figure 20.2G: A conjunctival incision is made a few millimeters

below the inferior tarsal margin
Figure 20.2H: The plane between the orbicularis muscle and the
orbital septum is dissected to the inferior orbital margin. Desmarre's
retractors are used to give traction and expose the tissues
Figure 20.2I: The periosteum is cut 4 mm from the orbital margin

with a radiofrequency monopolar electrode
Figure 20.2J: Using a Freer elevator the periosteum is lifted up and

access to the inferior orbit is achieved
Figure 20.2K: The orbital mass is isolated and separated from the
adjoining structures
Figure 20.2L: A pink firm well defined mass was removed

from the inferior orbit
Figure 20.2M: The cut edges of the periosteum are then isolated
Figure 20.2N: The edges of the periosteum are sutured with 6/0
vicryl interrupted sutures
Figure 20.2O: The conjunctiva is closed with interrupted 6/0 vicryl

sutures and the lateral canthotomy is repaired by using 6/0 prolene
sutures to secure the tarsus to the lateral orbital periosteum
Figure 20.2P: Closure is performed in 2 layers with 6/0 vicryl for

the soft tissues and 6/0 prolene for the skin
Figure 20.2Q: One week post surgery, there is minimal lid edema and
the wound is well apposed. Histopathological examination revealed it
was a degenerated parasitic cyst with chronic inflammation and
calcification. The child had an uneventful recovery thereafter
Orbitotomies 295
Case 2
(Modified Stallard Wright orbitotomy)
A 30-year-old female presented with gradually
increasing protrusion of the left eye for one year
associated with diplopia followed by progressive loss
of vision, watering, redness and pain in the eye. On
clinical examination, she had severe non-axial
proptosis measuring 12 mm with lateral displacement
of the globe (Figure 20.3A). There was protrusion of
orbital fat through the orbital septum. The mass was
palpable and seemed to encompass the globe. It was
firm in consistency, with mild tenderness. There was
severe restriction of eye movements, lagophthalmos
and exposure keratopathy. Fundus examination
showed a pale optic disc with blurred margins and
Figure 20.3A: A 30-year-old lady presented with progressive

proptosis of the left eye with downward and lateral displacement of
the globe
Figure 20.3C: CT scan (axial section) showed the ill defined

mass occupying most of the left orbit (arrow)
collateral vessels on the disc. CT scan showed an

irregular intraconal mass; optic nerve could not be
seen separately (Figures 20.3B and C). The mass
showed specks of intralesional calcification and was
causing distortion of the globe and medial orbital
wall indicative of a large optic nerve sheath
meningioma. Incision biopsy was done through a
subbrow incision which confirmed the diagnosis of
meningioma. As there was no optic canal or
intracranial extension, it was decided that debulking
of the mass would restore the eye to its natural
position although the eye was blind. Debulking was
subsequently done through a lateral orbitotomy
approach (modified Stallard-wright incision).
Steps of Surgery
Figure 20.3B: CT scan (coronal section) showed a large, irregular,

hyperdense mass behind the left globe
Figure 20.3D: A mark is made on the skin for the S shaped incision
Figure 20.3E: The skin incision is made with a no 11 Bard Parker

blade. The incision starts from just below the lateral aspect of the
brow and ends in a rhytid over the anterior zygomatic arch
Figure 20.3F: Under stretch and lifting the tissues, the

subcutaneous tissue is dissected down to the periosteum
Figure 20.3G: The periosteum is cut with a monopolar probe about 4

mm behind the orbital rim starting superiorly and ending inferiorly just
above the zygomatic arch
Figure 20.3H: The periosteum is then reflected. The periorbita is also

lifted away from the orbital bone upto the anterior one third of the orbit
Figure 20.3I: The temporalis muscle is separated from the underlying bone using a bipolar cautery
Orbitotomies 297
Figure 20.3J: Incision lines are made on the bone about 3 mm above
the frontozygomatic suture superiorly and just above the zygomatic
arch inferiorly. A Desmarre's retractor is placed to pull the skin and
subcutaneous tissue laterally and a lid guard is placed inside the orbit
to protect the contents of the orbit. Using an oscillating saw, cuts are
made along the incision lines on the bone. Irrigation is performed as
the saw is being used
Figure 20.3K: Once the cut have been made, the bone fragment is
held with a bone rongeur and moved back and forth until it fractures
posteriorly
M
Figures 20.3L and M: T shaped incision is made in the periorbita with a blunt tipped Westcott scissors
Figure 20.3N: The cut edges of the periorbita are grasped and
gently spread apart to extend the cut posteriorly
Figure 20.3O: Retractors are used to keep the orbital fat away and
dissection of the mass is performed by blunt dissection
Figure 20.3Q: The periorbita is closed with

interrupted 6/0 vicryl sutures
Figure 20.3P: A reddish friable firm mass was

removed from the orbit
Figure 20.3R: The subcutaneous tissue is apposed with 6/0 vicryl

sutures
REFERENCES
1. Wright JE et al. Continuous monitoring of the visual evoked
response during intraorbital surgery. Trans Ophthalmol
Soc UK. 1973;93:311.
2. Putterman A, Goldberg MF Retinal cryoprobe in orbital
tumour management. Am J Ophthalmol 1975;80:88.
3. Leone CR Surgical approaches to the orbit. Ophthalmology
1979;86:930.
4. Benedict WL Surgical treatment of tumours and cysts of
the orbit. Eleventh de Schweinitz lecture. Am J Ophthalmol
1949;32:763-73.
5. Smith B The anterior surgical approach to orbital tumours.
Trans Am Acad Ophthalmol Otolaryngol 1966;70:607-11.
6. Mc Cord CD Jr Orbital decompression for Graves' disease.
Exposure through lateral canthal and inferior fornix
incision. Ophthalmology 1981;88:533-41.
Figure 20.3S: The skin is closed with continuous 6/0 prolene

suture. The patient was doing well at last follow-up
7. Berke RN Modified Kronlein operation. AMA Arch

Ophthalmol 1954;51:609-32.
8. Wright JE Surgical exploration of the orbit. Trans
Ophthalmol Soc UK 1979;99:238-40.
9. Simonton JT, Garber PF, Ahl N Margins of safety in lateral
orbitotomy. Arch Ophthalmol 1977;95:1229-31.
10. Maroon JC, Kennerdell JS. Microsurgical approach to orbital
tumours. Clin Neurosurg 1979;26:479-89.
11. Shucart W Transfrontal approach to the orbit. In Hornblass
A(ed): Tumours of the Ocular Adnexa and Orbit St Louis,
CV Mosby, 1979.
12. Love JG, Benedict WL Transcranial removal of intraorbital
tumours. JAMA 1945;121:777-84.
13. Schurmann K, Oppel O. Transfrontal orbitotomy as a
method of operation in retrobulbar tumours. Klin Monatsbl
Augenheikld 1961;139:130-59.
14. Long JC, Ellis PP Total unilateral visual loss following orbital
surgery. Am J Ophthalmol 1971;71:218-20.
21
CHAPTER
Multidisciplinary Approach
to Proptosis
Subrahmanyam Mallajosyula, B Ranganadha Reddy, M Chandrasekhar Reddy
Introduction
The orbit is located between the facial structures,
paranasal sinuses and the skull base. Some of the
bony walls that separate the orbit and the paranasal
sinuses are very thin. Orbit is in direct communication
with brain through the optic canal. Various
pathological lesions extend from these surrounding
structures in to the orbit and vice versa. Rarely the
lesion can involve the sinuses, orbit and the brain,
like sino-orbito-cranial mucormycosis. Thus orbit is
an area of interest for several other surgical
specialties like ENT specialist and neurosurgeon.
Ophthalmic surgeons can deal with orbit by a number
of direct orbital approaches. ENT surgeons can gain
access to pathological conditions arising with in the
air sinuses through percutaneous approaches, oral
cavity or endonasal endoscopic approaches.
Neurosurgeons can access to those tumors that
invade both the intracranial and orbital space. A
detailed discussion of these lesions is beyond the
scope of this book. However we review the various
ENT and neurological lesions that may present with
proptosis and outline various surgical approaches
towards managing these lesions.
Surgical Anatomy
The orbital cavity is a 30 ml pear shaped; four walled
structure. The central axis of the orbit and the visual
axis of the globe are separated by 23 degrees.
The medial wall is formed by the lacrimal and
ethmoidal bones along with body of the sphenoid
bone.
Lateral wall of the orbit is formed by zygomatic

bone and greater wing of sphenoid, floor is formed
zygomatic, maxillary and palatine bones and roof is
formed by horizontal portion of the frontal bone and
by the lesser wing of the sphenoid bone.
The optic canal: A tubular cavity lying in the
deepest portion of the orbit enters the cranial cavity
medial to the anterior clinoid process. The optic canal
measures an average 5 to 10 mm long, 4.5 mm wide
and 5 mm in height.
The thickness of the medial wall of the optic canal
is an important surgical consideration in the
transethmoidal and transsphenoidal approaches to
the canal. In about 12% of cases the medial wall of
the optic canal is bordered not by the sphenoid sinus
but by a posterior or superior ethmoidal air cell. The
inclination angle of the optic canal relative to its
surroundings is practically important concern.
For the superior orbital fissure, through which
the intracranial duramater joins the periorbita, medial
margin is formed by the lesser wing of the sphenoid
and greater wing of sphenoid forms the lateral
margin.
Optic nerve has a flattened longitudinal shape,
measure approximately 4 6 mm. As it enters
the cranial end of the optic canal, it is circular and
5 mm in diameter, and continues to the globe as a
6 4 mm vertically oval structure.
The intracranial pia and arachnoid accompanies
the nerve from the chiasm and both fuse at the globe.
There are loose trabeculations in the subarachnoid
space.

The annulus of Zinn forms the tendinous insertion
of the extraocular muscle cone at the apex. It spans
the superior orbital fissure creating an intraconal and
extraconal compartment of the fissure, dissecting the
structures that run through it.
ENT APPROACH TO PROPTOSIS

The paraorbital region represents the paranasal air
sinuses surrounding the orbit. Tumors of the orbit
and paraorbital region sometimes distort the natural
architecture of the eye. Although proptosis may seem
to be primarily the concern of the ophthalmologist,
because of the close proximity of the orbit and para
nasal sinuses and various connecting fissures and
foramina between the two, many ENT lesions may
present with proptosis.
Various Etiological Factors of Proptosis in ENT

There are various etiological factors of interest to
the otolaryngologists, which can cause proptosis.
Diseases can be classified as follows:
a. Diseases of the orbit caused by the inflammation
in Paranasal sinuses.
b. Tumors of the orbito-sinual-region.
c. Diseases of the lacrimal apparatus secondary to
sinonasal diseases.
a. Infection and inflammation

A variety of inflammatory and infective sinonasal
conditions may impinge on the orbit; the commonest
of which are as follows:
1. Acute purulent sinusitis
2. Gross polyposis-particularly when it begins at
an early age
3. Fungal infections
4. Mucocele-commonest is frontoethmoidal
mucocele.
b. Tumors of the orbitosinual disease

The Paranasal sinuses tumors are classified into benign
and malignant.
Benign Paranasal sinus tumor

A. Epithelial tumors:
1. Papilloma
2. Inverting papilloma
3. Tumors of minor salivary glands
B. Angiomatous tumors:
1. Juvenile nasopharyngeal angiofibroma
2. Hemangiopericytoma
3. Lymphangioma
C. Mesenchymal tumors:
1. Fibrous dysplasia
2. Osteomas
D. Odontogenic tumors: Ameloblastoma
E. Neurogenic tumors
1. Schwannoma
2. Neurofibroma
Malignant paranasal sinus tumors

A. Epithelial tumors
1. Squamous cell carcinoma
2. Adenoid cystic carcinoma
3. Esthesioneuroblastoma
4. Malignant melanoma of sinonasal tract
B. Lymphoreticular tumors:
1. LymphomaNon-Hodgkin's
2. Extramedullary plasmacytoma
C. Mesenchymal tumors:
1. Osteogenicsarcoma
2. Rhabdomyosarcoma
3. Fibrosarcoma
4. Chondrosarcoma
c. Diseases of the lacrimal apparatus

1. Chronic dacryocystitis
2. Tumors of the lacrimal apparatus
Clinical Manifestations and Evaluation

The most common problems the patients complained
are
a. Proptosis
b. Nasal obstruction
c. Epistaxis
d. Reduced vision
e. Facial swelling
f. Nasal discharge
g. Redness of eye
h. Diplopia
After thorough ENT clinical examination the patients
presented with following manifestations.
Multidisciplinary Approach to Proptosis 301
anosmia, epistaxis and eccentric proptosis. Diagnosis

is by CT scan of paranasal sinuses. Medical treatment
includes nasal steroidal sprays. Surgical treatment
includes intranasal polpectomy, intranasal ethmoidectomy, external ethmoidectomy and FESS.
Proptosis
Nasal mass
Restricted eye movements
Septal deviations
Facial swelling
Reduced vision
Naso-pharyngeal mass
Congestion of the eye
Nasal discharge
Mucormycosis
A. Sinus Diseases Causing Proptosis

Purulent infections
Most of the bacterial infections in the orbit are caused
by the spread through infections of the paranasal
sinuses (Figures 21.1A and C).
Spread of infection to the orbit is through valve
less veins, and direct spread through lamina
papyracea.
The most common organisms involved in this
disease are Streptococcus pneumoniae, H. influenza,
Beta-hemolytic Streptococci, Staph. Aureus. Ethmoid
sinus is most commonly involved.
Treatment
High dose, intravenous broad spectrum antibiotics
for 2 weeks along with nasal decongestants.
Surgical decompression and drainage if
necessary.
Extensive nasal polyposis

Ethmoidal polyps can be seen in individuals having
history of allergy. This condition is most common in
Indians especially males. Patient presents with
bilateral nasal obstruction, nasal discharge, hypo/
This is a fulminant opportunistic infection caused by

saprophytic fungi of the order mucorales, commonly
seen in immunocompromised patients (Figures 21.2A
and B).
It occurs as rhinocerebral, pulmonary, ocular,
superficial and disseminated forms.Rhinocerebral is
again subdivided into rhinomaxillary and rhinoorbitocerebral form. It is characterized by bloody
nasal discharge, facial swelling, proptosis, altered
mental status, palatal or gingival necrosis, facial
nerve palsy. Dry black crust is seen in the inferior
turbinate, septum and palate. In advanced cases brain
and major vascular structures in the head can be
involved. This condition is diagnosed by CT scan
and frozen sections. This is treated by controlling
the underlying predisposing factors, immediate
debridement of all devitalized tissues and IV
amphotericin.
Allergic fungal sinusitis

This condition occurs when an atopic individual is
exposed to inhaled fungi. The fungi deposited in the
sinus cavity initiate the immunological reactions,
causing mucosal edema, stasis of the secretions, and
inflammatory exudates blocking the sinus ostia. This
process may expand to involve adjacent sinuses and
may produce sinus expansion and bony erosion.
Secondary bacterial infection can supervene.
Figures 21.1A to C: Male 42 years, presented with acute, painful proptosis of left eye of 4 days duration. Note the severe edema, of lids and
periorbital swelling with purulent discharge (A) CT scan of orbit axial views (B and C) show severe inflammation of orbit associated with
infected ethmoidal and sphenoidal) sinusitis

The patient presents with nasal congestion,
rhinorrhea, headache, epistaxis, and eccentric
proptosis. This condition can be diagnosed by raised
serum IgE levels, PAS stain, KOH mounting, CT
scans of PNS. Treatment includes steroid therapy,
surgical debridement and creating permanent
drainage.
Frontoethmoidal mucoceles
It is an epithelial lined, mucus containing sac filling
the sinus and capable of expansion. Frontal and
ethmoidal sinuses are most commonly affected.
Secondary bacterial infection may change these to
pyoceles. These are formed due to obstruction of the
affected sinus and inflammation. Patient presents
with proptosis, headache, and facial pain. This

condition is diagnosed by CT scans. This condition
can be treated surgically by external ethmoidectomy/
FESS.
B. Tumors of Paranasal Sinuses Causing

Proptosis
Benign tumors
Inverted papilloma
Lateral wall of the nose is the most common site.
There is proliferation of the covering epithelium and
extensive finger like inversions into the underlying
stroma of the epithelium. Patient presents with nasal
obstruction, epistaxis, and proptosis. This condition
is premalignant. This condition can be diagnosed by
CT scan. Treatment of choice is surgery either by
lateral rhinotomy and medial maxillectomy or mid
facial degloving.
Fibrous dysplasia
Figures 21.2A and B: A case of mucormycosis with proptosis and

severe ptosis of right eye. MRI (B) shows the involvement of maxillary,
ethmoid and frontal sinuses. Note intracranial and orbital extensions
Figures 21.3A and B: A case of allergic fungal sinusitis with

involvement of the anterior and posterior ethmoid sinuses and a huge
orbital extension (B) which is causing a severe eccentric proptosis
of the right eye (A)
Fibrous dysplasia is a skeletal developmental

anomaly of the bone-forming mesenchyme that
manifests as a defect in osteoblastic differentiation
and maturation.
The following 4 disease patterns are recognized:
Monostotic form
Polyostotic form
Craniofacial form
Cherubism
Monostotic form is the most common type(70-80%).
Sites of involvement most commonly include the
frontal, sphenoid, maxillary, and ethmoidal bones.
Hypertelorism, cranial asymmetry, facial deformity,
and proptosis may occur because of involvement of
orbital and periorbital bones. However visual
impairment, leading to blindness is rare. Involvement
of the sphenoid wing and temporal bones may result
in vestibular dysfunction, tinnitus, and hearing loss.
When the cribriform plate is involved, hyposmia or
anosmia may result. Diagnosis is by X-rays and CT
scans. Treatment is by local excision.
Hemangiopericytoma
A
Figures 21.4A and B: Eccentric proptosis of left eye (A) with the
globe pushed down and out. Note the infected ethmoid sinus (B)
It is of vascular origin arising from Zimmermann

pericyte cell. Patient presents with nasal obstruction,
epistaxis, and proptosis. Distant metastasis is
common to lungs, liver and bone. Diagnosis is

confirmed by histopathological examination.
Treatment is by radiotherapy followed by wide local
excision.
Juvenile nasopharyngeal angiofibroma

It is a highly vascular, benign but biologically
aggressive tumor originating in the nasopharynx
exclusively seen in adolescent males. This tumor has
both vascular and fibrous elements. Patient presents
with nasal obstruction, epistaxis, facial deformity,
proptosis, and headache. Biopsy is contraindicated.
Investigations required are CT scan with contrast,
and MRI angiogram. Preoperative embolization is
useful. Treatment includes lateral rhinotomy with
medial maxillectomy, transpalatine with or without
transantral approach, mid facial degloving.
Malignant Tumors
Squamous cell carcinoma
Maxillary sinus is the most commonly involved. It is
seen in elderly males. Patients present with nasal
obstruction, mass in the nasal cavity, epistaxis, facial
swelling, altered facial sensation. If mass involves
ethmoids then patient presents with proptosis,
Figures 21.5A and B: Eccentric proptosis of left eye (A) due to

angiofibroma. CT scan shows the tumor involving the sinus and the
nasal cavity (B)
swelling in the bridge of nose, CSF rhinorrhea, signs

of raised intracranial involvement. Diagnosis is by
biopsy, and imaging with CT scan and MRI.
Treatment includes surgery, radiotherapy and chemoradiotherapy. Surgery includes lateral rhinotomy and
medial maxillectomy, partial maxillectomy, total and
extended maxillectomy and craniofacial dislocation.
Adenoid cystic carcinoma

These arise from minor salivary glands of the nose.
These grow very aggressively and have perivascular
and perineural spread. Patients die of distant
metastasis. Diagnosis is by biopsy. Treatment includes
surgical resection with postoperative radiation.
Rhabdomyosarcoma
Most common soft tissue tumor of childhood. Patient
presents with proptosis, visual impairment, painless
facial swelling, nasal obstruction, and epistaxis.
Diagnosis is by biopsy, CT scan, bone marrow
examination. Treatment includes radical surgery,
radiotherapy and chemotherapy.
Non-Hodgkin's lymphoma
These are commonly seen in elderly males. B-cell
lymphomas are much more common than T-cell
lymphomas. The maxillary and ethmoid sinuses are
most commonly involved. Patient presents with nasal
obstruction, epistaxis, proptosis, infraorbital
anesthesia, facial swelling. Histopathological
evaluation, including immunohistochemistry of
biopsy specimen is diagnostic. If it is plasmacytoma
then serum electrophoresis should be done.
Treatment includes surgical resection, radiation and
chemotherapy.
Figures 21.6A to C: Note the pinkish tumor in the right nostril. CT scan shows a hyperdense lesion filling the entire
ethmoidal sinuses and extending into the right orbit (B and C)
Esthesioneuroblastoma
Also known as olfactory neuroblastoma or
neuroendocrine tumor, this arises in the upper part
of nasal cavity from stem cells of neural crest origin.
Patient presents with mass in the nasal cavity,
epistaxis, proptosis. It is a slow growing tumor which
may become large and destructive. Biopsy and
using specific tumor markers is the diagnostic
tool. Treatment includes surgical resection and
radiation.
Various approaches for tumor removal

Caldwell-luc operation
Lateral rhinotomy
Trans palatal approach to remove postnasal
tumors
Intranasal ethmoidectomy
External ethmoidectomy: Lynch-Howarth
procedure
Patterson's operation
Maxillectomy:
Medial
Partial
Total
Extended
Craniofacial approach
Functional endoscopic sinus surgery
Caldwell-Luc operation
Incision extends from canine ridge, runs laterally for
3.5-4 cm parallel to the teeth.
The soft tissues are incised down to bone.
Using a periosteum elevator, the bony anterior
wall of the antrum is exposed.
Using a 5 mm Jenkin's gouge, a sliver of anterior
wall is elevated and removed with forceps. The bony
opening is enlarged with Hajek's sphenoidal punch
forceps.
The antral mucosa is incised and the contents are

examined.
After surgery the cavity is packed with BIPP.
Jansen-Horgan operation
After performing the Caldwell-Luc operation the
posterior ethmoidal cells are opened through the
antrum. This is achieved by pushing a closed
Tilley-Henckle forceps in an upward, medial and
posterior direction at the upper and inner angle of
the antrum.
The opening is enlarged with suitable punch
forceps to allow adequate exenteration of the
posterior and middle ethmoid cells. Great care is
necessary in enlarging the opening as it may damage
cribriform plate or optic nerve.
Intranasal ethmoidectomy
Under general anesthesia the polypus bearing area
of the nose is exposed and the polypoid masses are
removed with Henckel's forceps. The remaining
pieces of polypoid mucosa are removed with Citelli's
upturned forceps, which is also used in uncapping
the ethmoidal bulla and for removing every visible
trace of edematous mucosa from the air cells.
External Ethmoidectomy
Lynch-Howarth's operation
Under general anesthesia a slightly curved incision,
medial to, and concave towards the medial canthus
of the eye is made. The periosteum is elevated to
B
Figures 21.7A and B: Caldwell-Luc operation
Figure 21.8: Jansen-Horgan procedure
Figure 21.9: Intranasal ethmoidectomy
reveal the nasal process of both the maxilla and

frontal bone and medial orbital wall. The lacrimal
sac is elevated from its groove and displaced laterally.
The thin ethmoidal wall medial to the orbit is
penetrated thus exposing the ethmoidal cells.The cells
are progressively exenterated.
Figure 21.10: Incisions for ENT approaches. Lynch-Howarth

approach (Green), Patterson's approach (Blue) and Lateral Rhinotomy
(Brown) incisions
Retraction of the cheek laterally enables the

anterior face of the maxilla to be removed as far as
the level of the infraorbital foramen.
Patterson's operation
An incision of size 1-2 cm is made in the natural crease
line about a finger's breadth below the infraobital
margin. The orbicularis muscle is exposed and split
in the line of the fibers. The periosteum is elevated
from the bone. The medial wall of the orbit defines
the lateral limit of the surgery. All cells medial to
this line are exenterated using the Tilley- Henkel
forceps.
Lateral rhinotomy/ medial maxillectomy

Under general anesthesia an incision is made from a
point halfway between the medial canthus and the
dorsum of the nose running medially to the
nasomaxillary groove upto the nasal ala.
Elevation and retraction of the skin and
periosteum over the nasal bone and frontonasal
process of the maxilla allow removal of these bones.
Bone removal can be extended to include both
lacrimal bone and the lamina papyracea of the
ethmoid. By this means the ethmoidal labyrinth can
be removed.
Figures 21.11A to D: Steps of surgery Lateral Rhinotomy: A; Incision

exposing the lateral wall of the nasal bone. B: Periosteum was incised
and reflected, and a small window was made in the nasal bone
(white arrow), which was extended (C) to expose the mass lesion
(yellow arrow). Note the clear space after excision of the mass (D)
Total Maxillectomy
an osteotome. The upper part of the maxilla is freed.

Then hard palate is separated from the soft palate.
Then the medial and lateral pterygoid plates are
removed to separate the posterior wall of the maxilla.
Then the free maxilla bone is separated from the
mucosal fibers and muscle fibers.
A Weber-Fergusson skin incision is marked out

starting at the philtrum of the lip and going upto the
columella. The incision is continued round the margin
of the ala of the nose and up, along the lateral wall
of the nose to the medial corner of the eye, turning
laterally in a rounded fashion to go 5 mm below the
lid margin on the lower lid. The remainder of the
incision is intraoral and follows the alveolar buccal
sulcus, around the maxillary tuberosity and across
the palate at the junction of the soft palate with
posterior end of hard palate. The final incision is
slightly lateral to the midline to join the original
incision in the region of the upper first incisor tooth.
Facial skin is elevated. Clearance of the orbit should
be done first. If the orbital contents are left in situ
they can be elevated. Then ethmoid labyrinth should
be exenterated. The bony orbital floor is divided with
Figures 21.12A and B: The external markings for incision (A), and
in the oral cavity for total maxillectomy
Figures 21.13A to D: External marking for the incision (A) intraoral incision made and the cheek flap reflected (B)
Anterior wall of the maxilla exposed (C), which was opened and the fungal granuloma excised (D)
Craniofacial Approach
Preliminary temporary tarsorraphies are made to
protect the cornea.
The classical lateral rhinotomy incision is
continued upto forehead either in the midline or in a
prominent frown crease. The soft tissues are
dissected off the underlying facial bones. The
periosteum of frontal bone is carefully elevated and
dissected laterally. The window craniotomy is
created. Dura is carefully separated.
Then the dissection is extended to encompass
both ethmoids and the anterior wall of the sphenoid
sinus. The specimen is finally freed by dividing the
perpendicular plate of the ethmoid. The lamina
payracea, anterior wall of the sphenoid and medial
antral wall must all be removed.
Trans palatal approach to remove postnasal

tumors
Under general anesthesia, a curved incision
bowed forwards is made between the maxillary
tuberosities, keeping internal to the greater palatine
foramen.
If the operation is performed for the removal of
the tumor which extends forward into the nasal
cavity, the incision should be carried more anteriorly.
The incision is deepened through mucosa and
periosteum. The mucosa on the upper surface of the
palate is divided transversely and postnasal space is
examined. Depression of the soft palate with a
retractor will give adequate exposure of the postnasal
space.
Figures 21.14A to C: Incision for cranio-facial approach (A), which is indicated when the lesion
involves the sinuses, nasal cavity, orbit and the brain as seen in the CT scan images (B and C)
A
A
Figures 21.15A and B: Incisions similar to lateral Rhinotomy, and

exposure of the nasal bone
Figures 21.16A and B: After removing the lateral wall of the nose,
the part of the tumor in the nasal cavity and ethmoid sinus was
excised by the ENT surgeon
Figures 21.17A to D: The incision is extended

by the neurosurgeon, onto the fore-head and
the horizontal part of it is behind the hair-line.
The Frontal bone is exposed (A) Bur-holes are
made (B) and the frontal bone is removed as a
flap(C), exposing the dura covering the brain.
The frontal sinus was exposed (D)
Figures 21.18A to D: Note the frontal sinus

component (yellow arrow) and the intracranial
component (white arrow) of the mass (A) The
entire mass could be visualized (green arrow)
after removing the overlying bone (B). The mass
was excised. The incision was closed in
layers with interrupted sutures (C). The
preoperative condition with eccentric proptosis
is seen in (D)
Neurosurgical Approach to Proptosis

The first known craniotomy for an intraorbital tumor
producing proptosis was performed by Durante in
Rome in 1887.
The choice of various approaches to the orbit
requires that the surgeon has a perfect knowledge
of the orbital pathology and a thorough knowledge
of the diverse surgical approaches as well as their
advantages, indications, limitations and contraindications.
Various surgical approaches to the orbit may be
divided into two groups: Extracranial approach and
transcranial approach.
Extracranial approach
Lateral orbitotomy
Figure 21.19: Incisions for transpalatal approach
Anterior orbitotomy
Figures 21.20A and B: Bony ostium is made in the medial wall of

orbit and fat is being removed (white arrow A). Note how big a bony
ostium can be made in the medial wall of the orbit for decompression
of orbit in thyroid associated orbitopathy
Functional endoscopic sinus surgery( FESS)

During the past decade and half, FESS has become
more popular and is increasingly used to treat lesions
of the sinuses with orbital extension, and for orbital
decompression surgeries as in Thyroid Associated
Orbitopathy. It can also be very safely used for
biopsy, debulking or excising the mass lesion. The
procedure is safe, gives a very good access to all the
sinuses without a surgical scar.
Removal of disease and relevant contact areas
while conserving normal mucosa without exposing
bony margins and preserving normal anatomy to
the extent possible is the advantage, with restoration
of ventilation and restoration of mucociliary
transport.
Transethamoidal orbitotomy
Frontal trans-sinusoidal
Inferior orbitotomy
Transcranial approaches
Sub frontal
Frontolateral
Frontoorbital
Frontotemporo-orbital
Pterional
Kronlein, 1889
Rowbotham, 1949
Krayenbuhl, 1967
Stalard, 1947
Berke, 1953
Brihaye, 1968
Kennerdell & Maroon, 1976
Knapp, 1847
Benedict, 1949
Niho, 1961
Colohan, 1941
Davis, 1940
Dandy, 1941
Housepain, 1969
Naffziger, 1948
Tym, 1961
Birhaye, 1968
Jane, 1982
Kennerdell & Maroon, 1976
Hamby, 1982
Because of its outline, the orbit has five sides,

each of which may be used to reach the orbital
contents.
1. The anterior surgical approach, anterior
orbitotomy can be performed through the
eyelids or the conjunctiva.
2. The orbitonasal route approach is termed transethmoidal orbitotomy.
3. Transmaxillary orbitotomy through the inferior
orbital wall.
4. Temporal orbitotomy or external lateral
approach through the temporal fossa.
5. Transcranial or transcranial orbitotomy through
the superior wall.

The temporal and superior or transfrontal
approaches are of surgical interest in neurosurgery.
Transcranial approach to the orbit is the most
complex and demanding route.
Indications
Cranio-orbital disease
Sphenoid wing or anterior fossa meningiomas with
orbital extension
Fronto-orbital fibrous dysplasia
Chondroma
Chondrosarcoma
Epidermoid cyst
Osteoma
Aneurysmal bone cyst
Primary intraorbital tumors-those arising in the
orbital apex or in the superointernal quadrant of
the orbit.
Optic nerve sheath meningiomas
Optic nerve glioma
Neurinoma
Intraconal cavernous hemangioma
Inflammatory pseudotumors
Perioptic metastases
Vascular malformations
Lymphangioma
In addition, two wall transcranial decompression
and extended pterional orbital decompression has
been tried in the management of graves
ophthalmopathy.
Supraorbital Craniotomy
Under general anesthesia
Supine position with head slightly extended to allow
separation of the frontal lobe from the orbital roof
by the gravity. The sagittal axis of the skull is rotated
10 toward the contralateral side.
Incision-hemicoronal or bicoronal skin incision

A cutaneous flap is raised and dissected from the
pericranium and from the temporal muscle
aponeurosis, including the superior branch of the
facial nerve in the folded planes.
The most anterior portion of the temporal muscle
is dissected and the burr hole is made in the temporal
fossa behind the zygomatic process of the frontal

bone, exposing the dura of the anterior fossa in its
upper portion and the periorbita in its lower portion
both separated by the orbital roof.
A second opening is made immediately above
the orbital rim in the superointernal angle outside
the glabella. Standard precautions are required
because of presence of frontal sinus. Both openings
may be connected with the craniotome, otherwise,
third frontal burr hole is made equidistant from the
first two and approximately 4 to 5 cm from the orbital
roof. From the lateral burr hole a Gigli saw is passed
toward the lateral rim which is then sectioned. From
this same opening the duramater and the periorbita
are protected with small spatulas the orbital roof is
sectioned with the chisel directed toward the midline.
From the medial frontal opening the superior orbital
rim and the orbital roof was sectioned with the chisel
directed laterally and posteriorly toward the
osteotomy previously performed. The frontal bone
flap is then easily raised together with the superior
orbital rim and the anterior and wider sector of the
orbital roof, exposing the frontal duramater and
periorbita.
It is necessary to complete bone resection of the
orbital roof and the upper margin of the optic canal
in apical tumors.
The surgical microscope and self retaining
retractors are placed with only minimal frontal
displacement. An anteroposterior incision is made
medially to the levator palpebraris muscle in the
periorbita. Next, the inner and superior rectus
muscles are explored by microsurgical technique until
the optic nerve is located. This method carries the
least risk of causing irreversible ophthalmoplegia.
In optic nerve gliomas, the nerve is often
extremely thickened in its distended meningeal
coverings, showing regular contours. In these cases
the lesion is dissected laterally, the anterior portion
from behind the eyeball and the posterior one at the
level of the annulus of Zinn, followed by opening of
the frontobasal dura to explore the optic nerve along
its intracranial course. The nerve is then resected
from the prechiasmatic level toward the optic canal,
including the foraminal portion.
In sheath meningiomas presenting as diffuse
thickening the dural sheath may be opened antero
posteriorly and the tumor may be dissected jointly

with the invaded sheath by following the
subarachnoid space. In few cases particularly in
lesions located anteriorly sight preservation is
achieved provided that careful dissection is carried
out and vascularization is spared in the nerve and
retina. When the tumoral mass is large and not
clearly restricted to the nerve course, exhibits micro
nodular infiltration of the muscles at the apex, the
eye is most likely amaurotic.Thus it is advisable to
resect this type of mass with the nerve. In such cases
it is essential to perform intracranial exploration of
the optic nerve which may be surrounded by the
tumor extending through the optic canal.
In neurinomas and cavernous hemangiomas,
once the lesion has been located and may be removed
in toto because of attachment to critical structures is
unusual. In rare cases of hour glass neurinomas
extending toward the middle cranial fossa combined
intracranial and intraorbital procedure is required.
Transcranial approach with resection of the

orbital roof
Tumors invading or arising from the orbital roof
demand a technique different from the one used for
purely intraorbital lesions.
Frontal or frontotemporal craniotomy is done
according to the lesion extension. When marked
hyperostosis is present, the procedure begins with
gradual resection of the pathological bone with
burrs, drills and gouges. It is often necessary to
continue bone resection toward the roof and lateral
orbital wall leaving the orbital contents amply
exposed. In purely osseous lesions this stage
ends with total removal of the lesion.The final step

is to perform plastic reconstruction of the resected
dura with pericranium.The roof and the lateral wall
of the orbit is reconstructed with methylmethacrylate.
Summary
The choice of the most suitable surgical approach for
orbital tumors is made on the basis of tumor location,
size, and apparant site of origin, propagation route
and probable histological type.
Superior orbitotomy is indicated in frontoethmoidal mucoceles, dermoid cyst and lacrimal
gland tumors.
Lateral orbitotomy is indicated in cavernous
hemangiomas, neurinomas, inflammatory pseudo
tumors and metastasis.
Inferior orbitotomy is less frequently used
approach in rare lesions such as lymphomas and
metastasis.
Transantral approach is indicated for tumors
originating from ethmoid cells and maxillary sinus.
Larger lesions require extended approaches for
example superolateral and inferolateral according to
tumor topography and used for large hydatid cyst
or huge cavernous hemangiomas.
Transcranial approach is indicated for cranio
orbital meningiomas of the sphenoid wing, and with
anterior cranial fossa disease with orbital extension.
The transcranial route is the only one that allows
decompression of the optic canal and intracranial
exploration of the optic nerve along its prechiasmatic
course.
Figures 21.21A and B: Axial proptosis of right eye in a male of 35 years (A) due to Schwannoma. Note that the well defined tumor is
partly in the orbit and partly in the brain (arrow) as it is extending through superior orbital fissure
Figures 21.22A and B: After making the transcoronal incision, the burr-holes are made in the frontal bone (A)
and the frontal bone is reflected as a flap (B)
D
Figures 21.23A to D: The tumor is exposed (A) (yellow arrow), which was excised (B).
The incision was closed in layers with interrupted sutures (C and D) shows postoperative recovery
CASE ILLUSTRATIONS
Case 1
Female 48 years (Figure 21.24A) presented with
eccentric proptosis of 2 years duration, and defective
vision since 2 months. She gives history of recurrent
episodes of sinusitis. On examination, she had
eccentric proptosis of left eye with the globe pushed
up (6 mm), outwards (7 mm) and forwards by
7 mm.The ocular motility was restricted mildly in all
directions. Pupil showed RAPD, and her BCVA was
20/60 and color vision was 6/17. Fullness of left
cheek was noted. CT scan of orbit (Figures 21.24B
and C) showed a large, hyperdense lesion filling

entire maxillary sinus, with orbital extension.
Extension in front of maxilla into cheek was found.
The optic nerve was pushed by the mass. In view of
recurrent sinusitis, involvement of the sinuses, fungal
granuloma was the clinical diagnosis. The lesion was
debulked through a modified lateral rhinotomy
incision, along with my ENT colleague. The orbital
component was debulked through the horizontal
incision, exposing the floor of the orbit. She received
amphotericin-B for the fungal granuloma. Her vision
improved to 20/30, and there was no recurrence in
the past 4 years.
Figure 21.24A to C: Note the eccentric proptosis of left eye with fullness of left cheek. CT scan of orbit (B and C) showed a large, hyperdense
lesion filling entire maxillary sinus (yellow arrow) with orbital extension (green arrow) and extension in front of maxilla into cheek (red arrow).
Note how the optic nerve (white arrow) was pushed by the mass
Figures 21.25A to C: The lesion in front of the maxilla was exposed (white arrow A) and excised through a modified lateral rhinotomy incision.
Then the anterior wall of maxilla was opened, the mass was exposed (yellow arrow B) and removed. The orbital component was debulked
through the horizontal incision, exposing the floor of the orbit. At the end of the surgery (C) note the concavity in the cheek due to excision of
the mass in the cheek
Case 2
Female of 16 years presented with eccentric proptosis
of left eye since 1 year and blurring of vision since
1 month. She was an agricultural worker. She had an
eccentric proptosis of left eye with the eyeball pushed
up and out (Figure 21.26A). Ocular motility was
restricted in elevation and abduction pupil showed
RAPD in the left eye. Her BCVA was 20/40. CT scan
(Figures 21.26B and C) showed a mass lesion of the
maxillary and ethmoid sinuses, with bony destruction

and orbital extension. Since most of the orbital floor
was destroyed, we planned for a swinging lower
eyelid approach, which gives adequate exposure to
the floor of orbit (Figure 21.27A) and the mass was
debulked (B). Postoperative recovery was good (C).
However there was significant enophthalmos. She
improved with antifungal drugs and there was no
recurrence during 3 years follow-up.
Figures 21.26A to C: This female of 16 years presented with eccentric proptosis of left eye since 1 year and blurring of vision since1 month.
Left eye was pushed up and out (A). CT scan showed a mass lesion of the maxillary and ethmoid sinuses, with bony destruction and orbital
extension (B and C)
Figures 21.27A to C: Swinging lower eyelid approach, which gives adequate exposure to the floor of orbit (roof of maxilla A) and the
mass was debulked (B). Postoperative recovery was good. Note the minimal scar (C)
Case 3
A 22 years male presented with complaints of left
sided nasal obstruction since 5 months, bleeding from
the left nasal cavity since 4 months, swelling over
the left cheek since 3 months and protrusion of the
left eye since 3 months. On anterior rhinoscopic
examination a pinkish mass was seen in the left nasal
cavity extending into the posterior choana which was
observed in the posterior rhinoscopy. There was a
proptosis of the left eye (Figure 21.28A). Ocular

movements and vision were normal. CT scan showed
a space occupying lesion arising in the left maxillary
sinus which is extending into the nasal fossa causing
displacement of inferior and medial walls of the orbit
s/o neoplasm (Figure 21.28B). The mass was excised
through the Moure's lateral rhinotomy approach.
Histopathology showed it to be nasopharyngeal
angiofibroma (Figure 21.28C and D).
C
A
Figures 21.28A and B: Eccentric proptosis of left eye with the globe
pushed up. Note the fullness below the lower eyelid (A) CT scan (B)
shows a hyperdense mass with contrast enhancement, involving the
maxillary sinus, extending into the nasal cavity. Note the bony
expansion of the sinus, leading to proptosis
Case 4
Boy of 2 years was brought with complaints of right
sided nasal obstruction since 2 months, protrusion
of the right eye since 1 month and watering of the
right eye since 1 month (Figure 21.29A). On anterior
rhinoscopic examination a grayish mass was seen in
the right nasal cavity. Right eye was proptosed.
Ocular movements were normal. CT scan (Figure
21.29B) revealed a well defined, non enhancing lesion
in the right anterior ethmoidal air cells with central
hyperdense component causing expansion of adjacent
Figures 21.29A and B: Eccentric proptosis of right eye (A) with the
globe pushed laterally and down. Note the fullness of superior sulcus.
CT scan (B) shows a large, heterogenous lesion involving the ethmoid
sinuses, with bony expansion
Figures 21.28C and D: Histopathology showing a picture of vascular

spaces with less stroma of fibrous tissue (C) suggestive of Angiofibroma (D) shows postoperative picture with recovery from proptosis
bony structures and proptosis of the right eye. The

mass was excised through medial orbitotomy
approach and the mass was sent for HPE which
showed infected granuloma (Figures 21.30A and B).
Case 5
Male 15 years, presented with complaints of left sided
nasal obstruction since 5 months and protrusion of
the left eye since 3 months (Figure 21.31A). On
anterior rhinoscopic examination a grayish white
mass was seen in the left nasal cavity. There was
eccentric proptosis of the left eye. Ocular movements
and visual acuity were normal. CT scan showed a
large well defined soft tissue lesion seen in the region
of the ethmoidal air cells on the left side extending
into left nasal cavity and sphenoid sinus. The lesion
was surrounded by thick irregular sclerotic bony
margins and there was expansion of the bony outlines
into the left orbit causing proptosis, suggestive of
fibrous dysplasia. In view of his symptoms and
cosmetic considerations, the mass was excised
through external ethmoidectomy approach.
Histopathology confirmed the diagnosis of fibrous
dysplasia (Figures 21.32A and B).
A
A
B
Figures 21.30A and B: Excised mass (A) and the early
postoperative recovery (B)
Figures 21.31A and B: Note the eccentric proptosis with the globe
pushed outwards (A) CT scan revealed an expansile lesion of the
ethmoid bone (B) with sclerotic margins, heterogenisity and cystic
spaces within, suggestive of fibrous dysplasia
Figures 21.32A and B: Microphotography showing well demarcated

islands of cartilage with interwoven fibro-osseous tissue, suggestive
of fibrous dysplasia (A) The early postoperative recovery with
improvement in proptosis is evident (B)
Case 6
Female 34 years, presented with proptosis of right
eye of 6 months duration, and defective vision since
1 month. She had axial proptosis of 5 mm and
downward displacement by 4 mm (Figure 21.33A).

RAPD was observed. Her BCVA was 20/30 in right
eye. The past history was significant in that she had
convulsions 2 years back, and was using
anticonvulsants since then CT scan of the orbit
revealed an intraconal, heterogenous, apical mass
with very distinct margins (Figures 21.33B and C).
The CT scan of brain (Figures 21.34A to C) showed
calcified lesions without any surrounding edema in
the frontal and occipital cortex. The possibility of
hemangioblastoma was considered. Fundus
examination was normal. In view of the apical
location, a transcranial approach (A) was performed
(Figures 21.35A and B). Histopathology confirmed
it to be hemangioblastoma. There was no recurrence
in the 2 years follow-up.
Figures 21.33A to C: Female 34 years, with proptosis of right eye of 6 months duration, and defective vision since 1 month. She had axial
proptosis of 5 mm and downward displacement by 4 mm (A). CT scan of the orbit revealed an intraconal, heterogenous, apical mass with very
distinct margins (B and C)
Figures 21.34A to C: The CT scan of brain showed calcified lesions without any surrounding edema in the frontal and occipital cortex
Figures 21.35A and B: In view of the apical location, a transcranial

approach (A) was performed. Histopathology confirmed it to be
hemangioblastoma. Postoperative figure (B) showing complete
recovery
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of laryngology and otology.
2. Balasingam V, Noguchi A, McMenomey SO, Delashaw JB
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annals of otology, rhinology and laryngology.
4. Canalis RL, ethmoidal mucocele. Archives of otolaryngology.
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Fibrous dysplasia of the orbital region: current clinical
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otolaryngology-head and neck surgery.
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malformations of the orbit: a distinct entity? A review of
own experiences. Neurosurg Rev. 2007;30(1):50-4;
discussion 54-5. Epub 2006.
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22
Orbital Exenteration
CHAPTER
Ramesh Murthy, Anirban Bhaduri, Sima Das, Santosh G Honavar
Exenteration refers to removal of the eyeball along

with the orbital contents. This disfiguring and
destructive procedure is reserved for the treatment
of life-threatening conditions where other
approaches have failed.
The first mention of orbital exenteration was
400 years ago by Bartische in his treatise on eye
diseases. 1 He described partial exenteration.
Extensive orbital exenteration was described
by Golovine, Nowikoff and Filatov in the early
1900s.2
Indications
We usually perform this procedure in the following
situations.
1. Malignancies
a. Primary orbital lesions like extensive
adenoid cystic carcinoma of the lacrimal
gland
b. Intraocular lesions like orbital extension of
retinoblastoma, choroidal melanoma
c. Orbital extension of periocular malignancies
i. Carcinoma of the paranasal sinuses
ii. Eyelid tumors
iii. Skin malignancies
2. Infections like sino-orbital mucormycosis and
other fungal infections.
3. Relative indications
a. Severe orbital contracture with inability to
wear a prosthesis
b. Neurofibromatosis with orbital deformity
c. Orbital meningioma and lymphangioma

causing disfiguring proptosis
d. Recalcitrant orbital inflammations unresponsive to other treatment modalities
Patient Preparation
The patient needs to understand the need for such a
destructive surgery and the gross disfigurement
caused by the procedure. We also counsel our patients
extensively that their facial appearance can never be
restored to what it was before. However, an orbital
prosthesis can restore appearance to an acceptable
extent, although it will never provide eyelid or ocular
motility. Psychological help is sometimes sought when
we feel that the patient may not be able to withstand
the consequences of the surgery.
Surgical Procedure
We always perform the procedure under general
anesthesia. In the rare situation when this is not
possible, local anesthesia can also be used with 2%
lignocaine with 1:200,000 adrenaline given as a
retrobulbar injection and followed by injections
around the orbital rim and in addition with
infraorbital, nasociliary and frontal nerve blocks. For
every patient we have a pint of blood ready for
transfusion if required. In addition hypotensive
anesthesia is used for all the cases. We also ensure
that the cauteries are working well and have sufficient
gauze on table to control bleeding.
The skin is marked all around with a methylene
blue marker. A 4-0 silk suture is passed through the
eyelid skin and orbicularis to exit from the tarsal
Orbital Exenteration 319
Types
Type
Contents removed
Contents preserved
Final appearance
Complications
Anterior exenteration/
extended enucleation
Globe, posterior lamella of

eyelid, conjunctival sac
Periorbita, posterior
orbital contents
Shallow socket,
immobile eyelids present
Delayed healing
Immobile ill fitted prosthesis
Lid sparing exenteration/

Subtotal exenteration
Orbital contents including

periosteum of orbital walls
Eyelid margins
Anterior lamina of the

eyelid including skin and
some orbicularis muscle
Hematoma behind the skin

flaps
Necrosis of skin flaps
Total exenteration/
Eyelid sacrificing
Orbital contents,
periorbita and lids
Bare orbital bones with

or without a skin graft
Deep orbit. Residual skin

and orbicularis edges
sutured together forming
a smooth lining
Spectacle mounted prosthesis can be fitted after
the healing is complete
Radical exenteration
Dissection involves
paranasal sinuses,
face, jaw, palate,
skull base.
Frontal bone replaced,

cavity covered with
myocutaneous vascular
flap with vascular
anastomosis
Cavity can be filled with

myocutaneous vascular
flaps or a maxillofacial
prosthesis can be used
to close the palatal defect
along with split skin graft.
Exposure of intracranial
contents and associated
complications
Poor cosmesis
plate at the lid margin and then passed similarly

through the tarsal plate and skin and tied to secure
the lids together. This suture is left long to provide
traction during the procedure.
A lid sparing exenteration is less disfiguring than
a total exenteration. In such a case we make the
incision a short distance away from the lid margin.
If however there is involvement of the lid, the incision
is made just inside the orbital rim. The skin incision
is made with a 11 no. Bard Parker knife, starting
inferiorly and progressing laterally, superiorly and
medially. The superonasal quadrant is approached
last as it bleeds extensively. If the lid skin is being
spared, it is dissected along with orbicularis to reach
the periosteum of the orbital rim. Once the lid skin
is cut, the underlying tissues can then be cut with a
monopolar cautery.
The periosteum is cut 6 millimetres or so from
the orbital rim and then elevated using a periosteal
elevator. The sharp end of the periosteal elevator is
used to separate it from the orbital rim and the blunt
end for the intraorbital portion. The attachments in
the region of the medial and lateral canthal tendon
and the trochlea are lifted off from the underlying
bone. The neurovascular bundle entering through
the infraorbital and supraorbital foramen is usually
cauterised before cutting it. We separate the
Sino-orbital fistula
CSF leak
Hematoma formation.
Keratinization of the skin
graft causing a malodorous
socket
Infection
periorbita from the bone superiorly, laterally and

inferiorly first using the blunt end of the periosteal
elevator or a lens spatula. The periorbita is firmly
attached to the bone near the orbital rim and loosely
within the bony orbit. Medially the lacrimal sac is
elevated and cut near the nasolacrimal duct.
Occasionally if there is a suspicion of tumor spread
through the nasolacrimal duct we remove bone in
that region using the Kerrison's rongeur. In cases of
tumors where there is bone involvement, we remove
all the involved bone. If such a step is pre-empted,
then occasional help from a neurosurgeon for tumors
extending superiorly and sometimes our ENT
colleague especially for sino-orbital mucormycosis
is sought.
Once we reach the orbital apex, we are wary of
the vessels that might bleed before we finally remove
all the contents of the orbit. We clamp the tissues
near the apex with a curved artery forceps before
cutting the apical stump. For visualization, we use a
lid spatula to push the contents to one side. Bleeding
can be a problem and we use a strong cautery to
stem it. Bone wax is needed for the occasional
recalcitrant bleeder. We wait for the blood pressure
to return to normal before we close the lid skin (if
performing a lid sparing exenteration). We use
absorbable 6-0 vicryl for the underlying tissues and

6-0 prolene continuous suture for the skin. If the lid
skin has been removed, the socket is packed with
Betadine soaked gauze.
We give the patient systemic antibiotics and antiinflammatory medication and give a pressure patch
for 2 days. On the third day following surgery we
remove the patch and perform daily aspiration of
the contents ( in lid sparing) or cleaning and packing
in total exenteration.
Management of the Exenterated Socket

It is important to note that the primary objective of
exenteration is to remove all disease. Even after
exenteration, the patient needs to be monitored
carefully clinically or by imaging for any recurrence.
Spontaneous Granulation
This has to be performed when the surgical excision
has been radical and the lid skin excised. In these
cases we leave the remaining skin to line the socket
and pack the socket with gauze soaked in Betadine
as a pressure pack and for about 2 weeks with regular
changing of dressings in the hospital, or by the patient
at home. The entire socket is allowed to heal by
granulation. Of course the advantage of this is the
fact that any recurrent disease can be identified easily.
However the healing is a slow process.
Skin Grafting
Partial thickness skin grafts have been in vogue for a
long time since they were popularized by Wheeler.4
Using a dermatome, skin is harvested from a non
hair bearing area such as the inner thigh and
abdomen. The graft is then fitted to the exenteration
cavity, trimmed and sutured using 6-0 prolene
sutures. Slits are made to facilitate drainage and
prevent collection of fluid underneath. Healing
usually takes 6 weeks, following which the patient is
fitted with prosthesis.5
Skin Flaps
This is my preferred technique where the lid skin is
spared and used to close the cavity. Postsurgery
pressure patch is placed and aspiration of the socket
is performed daily for a week to remove any
collection of fluid. Once the healing is complete, a
smooth surface is achieved over which prosthesis can
be placed easily.
Myocutaneous Flaps
Other techniques like temporalis muscle transposition, forehead, cheek, pectoralis flaps have also
been described.6-9
Prosthesis
Exenteration prosthesis is our preferred technique
of managing the cosmetic issues following
exenteration, which is discussed as a separate chapter
in the book.
Complications of Exenteration
Bleeding can be life threatening especially during the
surgery and adequate support from the anesthetic
team is a must. In addition blood for transfusion
should be available. One should have a good cautery
machine.
Socket infection can occur and needs to be
managed by systemic and local antibiotics. Any gape
in the sutured wound should be closed if possible or
in the presence of infection should be allowed to
granulate spontaneously.
Recurrence of the primary disease can occur and
hence monitoring at regular intervals postsurgery is
essential.
CASE ILLUSTRATIONS
Case 1
A 51-year-old female presented with pain and
swelling of the left eye for 15 days. She had a history
of gradual loss of vision in the left eye for 2 years
followed by gradual protrusion of that eye for 1 year.
On examination, there was proptosis of the left eye,
with swelling of the lids and ptosis (Figure 22.1A).
On lifting the ptotic lid, a brown, firm perilimbal
nodule was seen next to an opaque cornea (Figure
22.1B). There was complete restriction of eye
movements. CT scan of the orbit showed a soft tissue
mass filling the orbit almost upto the apex (Figures
22.1C and D). The globe contour was distorted and
no normal orbital structures were recognizable. A
diagnosis of uveal melanoma with secondary orbital
extension was made. There was no lymphadenopathy
in the head and neck region. Systemic workup was
done which did not reveal any systemic metastasis.

The patient underwent a lid-sparing exenteration.
The floor and medial wall bones were deficient
because of pressure effect, but the periorbita was
intact. Subsequently, the patient underwent external

beam radiotherapy to the left orbit and paranasal
sinuses.
Steps of Surgery (Figures 22.1E to Q)
Figure 22.1A: A 51-year-old lady presented with proptosis of the

left eye with associated lid swelling and ptosis
Figure 22.1B: A black mass was seen to be prolapsing out of the

cornea of the left eye suggestive of extraocular spread of melanoma
D
Figures 22.1C and D: CT scan of the orbit revealed a diffuse soft tissue mass of variable density filling the entire orbit
Figure 22.1E: The skin was marked on both the upper and lower
lids to identify the site of incision
Figure 22.1F: A rolled up wet cotton gauze was placed over the
conjunctiva. A 4-0 silk suture is passed through the eyelid skin and
orbicularis to exit from the tarsal plate at the lid margin and then
passed similarly through the tarsal plate and skin and tied to secure
the lids together
Figure 22.1G: 3 such sutures are passed and the ends kept long
for traction during the procedure
Figure 22.1H: The skin incision is made with a 11 no. Bard Parker
knife, starting inferiorly and progressing laterally, superiorly and medially
Figure 22.1I: In a lid sparing exenteration, the skin incision is made

a short distance away from the lid margin
Figure 22.1J: The underlying tissues are retracted and cut with a
radiofrequency monopolar cautery
Figure 22.1K: The skin and subcutaneous tissues are incised up to

the level of the periosteum
Figure 22.1L: The periosteum is cut about 6 mm from the orbital rim
and elevated using the periosteal elevator
Figure 22.1M: The periorbita is separated from the bone superiorly,

laterally and inferiorly first using the blunt end of the periosteal elevator
or a lens spatula. The periorbita is firmly attached to the bone near the
orbital rim and loosely within the bony orbit. Medially the lacrimal sac
is elevated and cut near the nasolacrimal duct
Figure 22.1N: Near the orbital apex, the tissues are clamped with a
curved artery forceps before cutting the apical stump
Figure 22.1O: The exenterated contents are dark brown in color

suggestive of a melanoma
Figure 22.1P: 6/0 vicryl sutures are used to suture the

subcutaneous tissue once hemostasis has been achieved
Figure 22.1Q: The skin is closed with 6/0 prolene
Figure 22.2A: A 40-year-old male presented to us with progressive proptosis of the left eye of 4 months duration
Case 2
A 40-year-old male presented to us with eccentric
protrusion (outward and lateral displacement) of the
left eye which was gradually increasing for last 4
months (Figure 22.2A). A non-tender mass, firm to
hard in consistency was palpable deep in the superior
and nasal part of left orbit. There was no apparent
nasal or paranasal sinus pathology. On presentation,
he had best corrected visual acuity of 6/7.5 and 6/12
in right and left eyes respectively. Ocular movements
in the left eye were grossly restricted; adduction
more than abduction. Anterior segment examination
was unremarkable in both eyes. Fundus in the right
eye was normal and in the left eye showed presence
of early disc edema. The patient was non-diabetic
and non-hypertensive. Ultrasound B scan of the left
eye revealed diffuse thickening of medial rectus with
maximum diameter being 14.3 mm. CT scan showed
presence of a fairly well defined, uniformly isodense,
intraconal mass located between medial rectus and
optic nerve. Medial rectus could not be appreciated
separately from the mass posteriorly (Figures 22.2B
and C). An excision biopsy by a lid split medial
orbitotomy approach was done. Postoperatively his
proptosis was reduced but the vision in left eye was
reduced to PL with inaccurate projection of rays, and
he had grade 1 relative afferent pupillary defect.
Histopathology of the biopsy specimen showed
presence of giant cells and elongated budding fungal
filaments. Microbiological evaluation confirmed a
diagnosis of aspergillosis.
C
Figures 22.2B and C: CT scan revealed the presence of a fairly well
defined uniformly isodense intraconal mass between the medial rectus
and optic nerve

4 months later the patient came back with
exacerbation of proptosis with a firm mass palpable
in the medial canthal region (Figure 22.2D).
Exophthalmometry readings were 19 and 30 mm in
the right and left eye respectively. He had no vision
in the left eye at this visit. CT scan showed a solid
mass, which was filling almost the entire orbital
cavity; extraocular muscles could not be made out
separately from the mass (Figures 22.2E and F). The
mass could be seen extending up to the superior
orbital fissure. Lateral wall of orbit showed
excavation of bone. Contiguous middle cranial fossa
and paranasal sinuses appeared uninvolved.
Lid sparing exenteration was done in view of
extensive nature of disease.
Figure 22.2D: 4 months later the patient came back with exacerbation
of proptosis and a firm palpable mass in the medial canthal region
F
Figures 22.2E and F: CT scan axial (E) and coronal (F) sections revealed a hyperdense mass filling most of the orbit
Figure 22.2G: Periodic acid schiff (PAS) stain (400) showed the
presence of dark filaments confirming the diagnosis of aspergillus
flavus
Figure 22.2H: Gomoris methanamine silver (GMS) stain showed of

dark filaments aspergillus flavus

The patient was maintained on oral antifungals
and was stable with no recurrence till last follow up
3 months postsurgery (Figure 22.2I).
REFERENCES
Figure 22.2I: 3 months postsurgery the skin wound had healed

and the patient did not show any signs of recurrence
Histopathology of the exenterated specimen

revealed multiple septate branching fungal
filaments within the giant cells and stroma. There
was no definite evidence of vascular invasion. There
was no involvement of the globe. Microbiology
confirmed the presence of Aspergillus flavus [Figure
22.2G periodic acid schiff (PAS) stain showing the
dark filaments and Figure 22.2H Gomori's
methanamine silver (GMS) stain showing the dark
filaments against a green background] in the
exenterated orbit.
1. Bartische G Ophthalmodouelia, das ist Augendiest.

Dresden, Matthes Stockwell, 1583; 217-19.
2. Golovine SS Orbitosinus exenteration. Ann Ocul
1909;141:413-31.
3. Nowikoff V Extirpation of the orbit. Lyon Chir 1927; 26:
17-27.
4. Wheeler JM The use of epidermic graft in plastic eye surgery.
Internat Clin 1922; 3:292-300.
5. Shields JA, Shields CL, Demirci H, Honavar SG, Singh AD
Experience with eyelid-sparing orbital exenteration: the
2000 Tullos O. Coston Lecture. Ophthal Plast Reconstr Surg
2001;17(5):355-61.
6. Donahue PJ, Liston SL, Falconer DP, Manlove JC.
Reconstruction of orbital exenteration cavities. The use of
the latissimus dorsi myocutaneous free flap.Arch
Ophthalmol. 1989; 107(11):1681-3.
7. Uusitalo M, Ibarra M, Fulton L, Kaplan M, Hoffman W, Lee
C, Carter S, O'Brien J. Reconstruction with rectus abdominis
myocutaneous free flap after orbital exenteration in
children. Arch Ophthalmol. 2001; 119(11):1705-9.
8. Bonavolonta G Frontalis muscle transfer in the
reconstruction of the exenterated orbit.Adv Ophthalmic
Plast Reconstr Surg. 1992; 9:239-42.
9. Arlyan S, Cuono CB.Use of the pectoralis major
myocutaneous flap for reconstruction of large cervical, facial
or cranial defects. Am J Surg. 1980;140(4):503-6.
Orbital Prosthesis 327
23
Orbital Prosthesis
CHAPTER
Kuldeep Raizada
INTRODUCTION
Loss of an eye has a very traumatic effect. It leads to
loss of self belief, can make the patient and family
very depressed. Eye to eye contact is very important
in conversation, which is usually lost if the person
has a very disfiguring eye or an empty socket. They
seek aesthetic improvement in their cosmetic
appearance. Patient's rehabilitation with prosthesis
is very challenging, especially when it is a facial
prosthesis where a natural looking prosthesis is the
final goal.
Orbital prosthesis and ocular prosthesis deal with
the fabrication of an artificial substitute for different
kind of orbital deformities, which can be due to
diseases, surgery, trauma or congenital malformation.
Several cases of devastating facial injuries, incurred
in battle, were treated in the early nineteenth century
with indigenous appliances and these reconstructive
procedures gave definite push to the field of facial
replacement.1 Great strides in the field have been
made in the past decades.
Orbital Prosthesis
Orbital prosthesis is meant for the face to improve
the cosmetic appearance of the individuals and most
often it is required in conditions where there is an
additional loss of periocular tissues like eyelids,
eyelashes and eyebrows.3 While fabricating an orbital
prosthesis utmost care should be taken to not only
replace lost periocular tissue but also to match them
in terms of color and texture to the surrounding
tissues and the fellow orbit.
Types of Prosthesis
Orbital
Complete
Spectacle mounted prosthesis
Adhesive retained prosthesis
Magnetic retained prosthesis
Partial
Adhesive retained prosthesis
Complete prosthesis: means prosthesis with not only
an ocular prosthesis but also it contains eye lids, eye
brow and eye lashes, to restore the normal anatomical
appearance to the patient.
On the basis is retention it can be further
classified.1-4,8
Spectacle mounted prosthesis: is used in conditions
where surface remains moist and a silicon prosthesis
is not going to stay. A facial prosthesis made up of
acrylic can be attached to spectacle, can be an option
for such kind of cases.
Adhesive retained prosthesis: in conditions where
surface is dry and a silicon prosthesis is going to
stay. A facial prosthesis made up of medical grade
silicon can be attached externally in such cases.
Magnetic retained prosthesis: this is fabricated in a
very special situation where patient had radical
orbital exenteration. The surface is provided with
the support of magnetic anchors that helps in
retaining the prosthesis.
Partial prosthesis: described in literature earlier for
the nasal and auricular prosthesis. Recently Raizada
et al.5-6 described the method of fabricating the partial
orbital prosthesis where at first a custom made ocular

prosthesis is placed and later on the lower eyelids
are designed using the medical grade silicon and then
it can be retained with epithane.
Factors that Affect the Fit of an Orbital

Prosthesis
There are many factors that affect the fit of an orbital
prosthesis.It is important to evaluate the orbital
defect and later on choosing the modalities of
retaining the orbital prosthesis.
1. If the orbit had an incidence of tumors, there
should be no recurrence.
2. The surface should be well healed; there should
be no edema, or infected external surface if the
case is of open defects like in the cases of radical
orbital exenteration.
3. If it is a case of orbital trauma and severe
deformity like lid coloboma, they may benefit
with partial upper or lower lids prosthesis.
4. If the patient had extensive damage to the orbital
cavity, a glue based prosthesis will be interfering
with patient eyelids and may not be very
comfortable. Such cases may be either
considered for blepharorrhaphy or else an
spectacle mounted orbital prosthesis with
vaulted back surface.
5. If the patients have ocular disfigurement in the
lower lids due to the extensive trauma and lids
cannot be constructed, a partial prosthesis with
integrated lower lids can be fabricated.
In this chapter I shall be discussing the technique
of fabricating orbital prosthesis (a hybrid type as a
complex prosthesis is made up of two different
materials, so called as hybrid prosthesis).
There are many steps involved but the following
steps involved fabricating an orbital prosthesis are
of more concern:
Preperation of the patient
Impression
Casting
Sculpting
Moulding
Coloring
Using the desired material
Fabrication of ocular prosthesis.
Preparation of the Patient

This is a very important step as patient needs to
understand what we are going to do. A proper
counselling about the whole procedure makes the
patient very calm, and make the work very
comfortable. I believe that the more the patient
understands, the better he co-operates. Explain to
the patient the whole procedure using the other
patient's pictures or illustrations. You can now ask
the patient to lay down on a couch or bed in order
to make patient completely relaxed as this is very
tiring because the whole procedure takes about 30
to 45 minutes.
Impression of the Orbital Defect

Impression is taken while the patient is lying in the
supine position. Vaseline is applied on the places of
eyebrows, eyelashes as well as in the raw area of the
defect if present.
There are many ways to take an impression.
Many people believe that only defective side
impression makes work much easier, but I believe
that taking the impression of the whole face gives
better pictures, hence forth I prefer to take the
impression of both sides of the face so as to obtain
accurate information about the defective as well as
normal side too.
We make use of hydrophilic colloid as the basic
material of taking the impression of the orbital cavity.
It is not only just an impression material that is
needed but you also require the reinforcing materials
such as metal clips, gauge piece and the final layer of
casting stone.
Prior to taking an impression of the defect, it is
essential to tell the patient about the type of
impression you are going to take as some time the
defects are open as in case of radical exenteration,
and slight mistakes give really a hard time. By using
the base plate wax and adhesives like micropore tape,
mark the boundary of defect. Use Vaseline on the
area of the eye lashes and eyebrow, so that while
taking out, the impression comes out easily. (Figures
23.1A to C)
Figure 23.1E : This is a lateral view showing how
the base plate wax and micropore adhesive makes a
boundary of the defect and make easier to take an

impression. Use the hydrophilic colloid in the ratio
of 1:1 with water and after mixing thoroughly with
the flat spatula, pour it first on the defect area and
later in the surrounding tissues (Figure 23.1D). Use
the reinforcing materials to make the impression so
that while putting the second layer of die stone, it
gets adhered to the surface and makes a stable
impression (Figure 23.1F).
As hydrophilic colloid gets set very fast it is
recommended that mix the die stone in ratio of 1:1
with water and pour above the hydrophilic colloid
material. Meanwhile use the reinforcing material to
make the die-stone mechanically strong (Figure 23.1G).
We prefer to use even gauze piece over the dye
stone (Figure 23.1H), so that it get integrated with
dye-stone. In about 10 minutes the die-stone gets
set and exothermic reactions starts and we use mild
water on the surface so that it remains cool. Once
the die-stone is set, ask the patient to squeeze the
face and remove the impression which is called as
negative impression (Figure 23.1I).
Casting
Casting is replicating a negative impression to
positive impression, and this will reflect the defect
of the patient. Stablize the impression material using
the clay or sand box and slowly pour the mixture of
dye-stone, step by step. Let it set for its optimal time
of about 15 minutes (depends on the type of dye
stone used), (Figures 23.1J to L shows the junction
line of dye-stone and hydrophilic colloid from where
you can separate the negative and positive
impression). Remove dye-stone cast at the junction
line slowly from the hydrophilic colloid and this will
represent the patients deformity (Figures 23.1I and
L). You should always compare the impression of
the defected area and the patient's actual orbital
defect. Once you are sure then only proceed ahead.
(Figure 23.2A).
Sculpting
Use the tinfoil of 0.01 mm on the defect to make the
model easier to take out from the cast and to check
Figures 23.1A to L: (A) patient with front view and left orbital exenteration (B) base plate wax boundary to hold the impression material
(C) front view with base plate wax boundary in place with applied vaseline on the face (D) front view with the placement of hydrophilic colloid
and reinforce material (E) lateral view shows the use of adhesive tape on the boundary of base plate wax (F) placements of uniform layer of
die-stone (G) placement of tiny reinforce material so that while taking out the impression cast should not break (H) placement of a gauge piece
on the same (I) after removal of cast from the cavity called negative impression (J) on the negative impression after placing layers of die-stone
(K) once the die-stone is set, you can see the differentiation zone of these two (L) after separation of the two-piece

it on the patient face (Figure 23.2B). There are various
kinds of materials used such as clay, waxes or, direct
silicon for sculpting model. We prefer to start the
procedure with the base plate wax as it has property
of moulding into desired shapes (Figure 23.2C).
Now we need to choose an ocular prosthesis for
the same anterior curvature of the patient, preferably
a flatter one when you have shallow defect of the
orbit (Figure 23.2D). By using the Purkinje's images
of the fellow eye and measuring the inter pupillary
distance (distance from the mid line of face to the
center of the fellow eye) to locate the exact corneal
reflex and even the use of hurtle exophthalmometer
for assessing the exact amount of proptosis helps
greatly in fabrication. Once satisfied with the corneal
position, I use the thin strip of the base plate wax to
sculpt the eyelids. It is preferable to see the patient's
face from all angles so that when prosthesis is
fabricated, it should meet the criteria of having equal
amount of elevation from the base of the orbit
(Figures 23.2E to H).
I also take the pictures of the patient with the
final sculpted model and download in the computer,
using the 'Adobe Photoshop 6.0' as graphical

visualization makes it much easier to correct further
the lids alignment, and to create a better symmetry
(Figure 23.2I). Once I am done with these all steps I
again go back and check the sculpted model and
compare to the patient's defect, use the desire
spectacle frame and cut in that fashion (Figure 23.2J).
Moulding
Making a two-piece mould is not a very complicated
job, but of course making a mould is an art. Once the
wax model is finished, apply the vaseline on the rear
side of the prosthesis in order to get a smooth
moulded surface. Mix die-stone in a ratio of 1: 1 with
water. Use the vibrating unit to remove all the air
bubbles and pour the mixture of die-stone and water
in to a metal flask, apply some of the mixture on the
back side of the model in case of any undercuts and
then invest this model in the metal flask. Look very
carefully as some of the dye-stone may be fast setting
because before it sets you need to remove the excess
material from the mould surface and check if any
undercuts are there, as presence of undercuts in the
Figures 23.2A to O: (A) positive case from the impression of the face (B) use of tin foil so that model can be taken out with out distortion (C)
Use of the base plate wax a base on the top of the thin tin foil (D) placement of an ocular prosthesis, front view looking at the Purkenje's images
(E) eye lids after attaching the lump of wax and carved with the metal spatula (F) lateral view of the same patient on the normal side (G) lateral
view of the same patient on the affected side (H) placement of final wax model on the face (I) use of computer programme Adobe Photoshop
to make the grid and analyse further, in order to get a better symmetry (J) final wax model placed on the positive cast of the impression (K) invest
of the wax model in the metal flask using the die-stone (L) once the die-stone is set, apply thin layer of "Cold Mould Seal" so that mould can be
open (M) once the thin layer of the "Cold Mould Seal" is dried second layer of die-stone is also poured in the metal flask (N) open the mould, see
that how beautifully the mould opens up (O) do remove the all wax from mould using the hot water and soap

mould will finally affect the quality of prosthesis.
Once the stone is set, apply the separating media
(from DPI) and pour the other in the same fashion.
Once the mould is set, open it very carefully and
remove the wax model from the mould, clean it with
the hot water and reapply the separating media
(Figures 23.2K to 23.2O). Now your mould is ready
for pouring the desired material.
Using the Desired Material

Attach the prosthesis to the stone moulds in its
original position. Using the cynoacrylate, fix the
prosthesis into its curvature so that it remains in same
position as done while sculpting (Figures 23.3A to
C). Till this step everything is common, and now
you have to decide which type of prosthesis you are
looking for.
Here I describe the making of silicon prosthesis.
I mix MDX4210 with Dow corning silicon in 1:1 along
with combinations of artist colors and dyes, try to
match the shades of the patient skin and add more
flocking so that it give much better skin appearance.
I usually prefer intrinsic coloring MDX4210. As it gets
set, pour into the moulds and take out all the air
bubbles. Now close it from back to front, so that in
case any air bubble remains, it will come on the back
of the prosthesis and can be later taken care by doing
the patch work (Figures 23.3D to F).
Cure the silicon at room temperature under high
pressure. Once the silicon is cured, open the moulds
and you have the prosthesis ready in your hand.
Trim the extra margins using the 3M (Factor II)
trimming wheels in a tapering fashion, so that it
mingles well with the surrounding tissues.

Sometimes one may need an extra touch up to the
colors to make the appearance better (Figures 23.3G
and H).
Fabrications of Ocular Prosthesis

Make the mould of same prosthesis,7 locate the Iris
position and make a fresh mould, paint an iris button
of the same curvature as the fellow eye and
polymerize with the white base using the pressurized
curing unit. Once it is cured, open the mold, create
the blood vessels using the cotton rayon threads.
Paint the scleral shades using dry earth pigments and
cure with the clear layer of PMMA. Trim the extra
portions, polish and insert into the patient orbital
prosthesis, attached the eye lashes and eyebrows
(Figure 23.3I).
Assemble the Prosthesis

Once you are done with fabrication of ocular
prosthesis and the facial prosthesis, your ocular piece
can go very easily in the cavity that has been formed
in place of dummy prosthesis.
Attach the eyelashes on the upper lid and lower
lids. If needed some external coloring can be done
in order to look better (Figure 23.3J). Once satisfied,
you have to instruct the patient regarding the use of
the prosthesis.
Care of Your Prosthesis

Preparing Your Skin and Your Prosthesis
1. Repeatedly practice positioning your prosthesis
without adhesive to ensure accurate placement.
Figures 23.3A to J: (A) mould shows smooth surface without pit and holes ideal for using the silicon (B) place the ocular prosthesis in the eye
cavity groove (C) use the cynoacarylate glue to fix this in same place (D) shows the silicon from factor II and intrinsic colors (E) shows the
steering of silicon along with flock and intrinsic colors in the silicon (F) using the thin cellophane sheet and checking the final color with patient
skin tone (G) shows the room temperature cured prosthesis (H) indicated the rough edge of the prosthesis (I) fabrication of an ocular
prosthesis (J) final finished prosthesis
Figures 23.4A and B: (A) patient with left orbital exenteration due to
Basal cell carcinoma of the left eye (B) patient with silicon glue on
prosthesis in place, further cosmetic appearance improved with simple
pair of glasses
2. Wash and thoroughly dry your hands and skin

where your prosthesis is to be placed.
3. Clean your prosthesis with a soft, bristled
toothbrush, mild soap, (e.g. Ivory liquid) and
warm water.
Applying Your Prosthesis

1. If adhesive is used, it should be applied with
cotton tipped swab by evenly spreading a thin
layer of the adhesive along the outer edges of
the backside of your prosthesis according to the
manufacturer's instructions.
2. Allow the adhesive to reach its proper
reapplication state depending on the type of
adhesive used (e.g. dried clear for Pros-Aide).
3. Using a mirror, carefully position and press your
prosthesis onto your skin to ensure good contact.
Removing Your Prosthesis

1. Remove your prosthesis from skin on a daily
basis to keep your tissues healthy and to maintain
hygiene. Grasp the thickest edge of your
prosthesis and gently remove it very slowly so
as not to tear the edges or irritate your skin.
2. If necessary, use a moist washcloth over the
surface of the prosthesis to loosen adhesive from
your skin.
Cleaning Your Prosthesis

1. If adhesive was used, remove it gently rolling
the adhesive off the prosthesis (starting from
the center to the outer edges) with your
fingertips, using gauze or textured cloth.
Soaking the prosthesis in a cup of warm water
helps to soften adhesive and makes it easier to
remove.
2. Clean the prosthesis with a soft, bristled

toothbrush, mild soap (e.g. Ivory), and warm
water.
3. Remove any traces of adhesive or oil by gently
wiping the tissue side with a gauze or softtextured cloth moistened with rubbing alcohol.
Repeat this step using a gauze or soft-textured
cloth moistened with Listerine on the backside
of the prosthesis.
4. If your prosthesis has an ocular component,
remove and clean it with soap and water. The
ocular component should NOT be cleaned with
rubbing alcohol. Place a drop of mineral oil on
the eye and shine it once a week. Replace the
eye carefully and adjust the location by
squeezing the prosthetic eyelids together.
5. If your prosthesis is retained with magnets,
clips, or plastic buttons, take care to clean
around each fixture with a soft, bristled brush,
soap and water.
Cleaning Your Skin

1. Wash your face with soap and water after
removing the prosthesis and remove any residue
of adhesive from the skin. Avoid the use of
harsh solvents such as benzene or xylene.
2. Apply a moisturizing lotion on nightly basis to
restore natural body oils.
3. Report any areas of inflammation or irritation
to the office or clinic.
Color Changes
1. Avoid smoking, as it will stain prosthesis
yellow.
2. Avoid prolonged exposure to sunlight, which
can cause color dissolution and weakening of
the prosthetic material.
3. Avoid the use of strong solvents, such as benzene
and xylene, which can cause dissolution and
weakening of the prosthetic material.
Storing the Prosthesis

1. Store the prosthesis in a dry, inconspicuous but
safe place (for example, a bedside table drawer).
Keep it out of the reach of children and animals.
2. If you have an orbital prosthesis, store it in an
upright position.
Preventing Mishaps
1. Avoid extreme temperature changes, which can
cause adhesive to fail.
2. Carry extra-adhesive and pre-packaged alcoholsoaked cotton balls in a small plastic bag.
3. Avoid placing the prosthesis in purses or pockets
close to items such as ink pens and makeup that
could stain it.
4. If adhesive are prescribed, be careful not to spill
the adhesive bottle. To prevent evaporation,
keep the lid tightened when not in use.
CONCLUSION
In majority of cases facial prosthesis restores the
cosmetic appearance as well as self confidence
(Figures 23.4A and B). However, the patient should
be educated and motivated to use the prosthesis and
maintain it properly. A cosmetic rehabilitation
without the proper counselling is not effective.
Overall a facial prosthesis remains an option in
minority of cases.
REFERENCES
1. Prince JH, A short history of the development of artificial
eyes. In: Ocular prosthesis. E and S Livingstone Ltd,
Edinburgh, 1946:6-7.
2. Jackson, IT, Tolaman, DE, Desjardins, RP,Branemark, PI: A
new method for fixation of external prosthesis, Plast
Reconst. Surg. 1986;77:668-72.
3. Raizada K, Murthy R, Honavar SG. Ocular prosthesis with
lower lid augmentation for disfigured lids following
chemical burns. Journal of Ophthalmic Prosthesis J Ophth.
Prosth. Volume II PP 24-6.
4. Raizada K, D Deepa Rani, Naik M, Honavar SG, Journal of
Facial and Somato Prosthesis. Post Enucleation Socket
Syndrome: an ocularist View, Journal of Facial and
Somatoprosthesis, 2005;1-12.
5. Yeatts RP. The esthetics of orbital exenteration.Am J
Ophthalmol. 2005;139(1):152-3.
6. Bulbulian AH. Prosthetic reconstruction of the exenterated
orbit. In: Facial Prosthetics. Charles C Thomas, Springfield,
IL, 1973: 48-63.
7. Jahrling RC. Contracted socket following enucleation after
multiple surgical procedures. Problems and treatment of
enucleation, evisceration, exposure. Intercontinental
Medical book corporation 1974:12;27-9.
8. Bulbian AH, Facial Prosthetics, method of retention of facial
prosthesis 364.
24
CHAPTER
Medical Management
of Proptosis
Some of you may be surprised to know that nearly

half the cases of proptosis can be managed without
surgery. This is because of the advances in diagnostic
and therapeutic interventions made in the past few
decades which made medical managements more
scientific, evidence based and safer. Many patients
of proptosis obviously are to benefit from these
advances. We are confident that most of the active
orbital surgeons across the globe agree with this
statement.
Many etiological factors involved in proptosis can
now be safely managed medically. This chapter
outlines the standard protocols and recent trends
invading this arena. The role of medical management
in thyroid orbitopathy, and the role of chemotherapy
were dealt in detail in separate chapters and hence
not included in this.
Let us consider the medical management in the
following headings:
Nonspecific inflammations of the orbit
Specific inflammations of the orbit
Vascular lesions
Structural lesions
Lymphoproliferative and other neoplastic
lesions.1
NONSPECIFIC INFLAMMATIONS OF
THE ORBIT (NSOIS)
Based upon the location of inflammation the nonspecific orbital inflammation or idiopathic orbital
inflammation can present as five entities : Myositic,
lacrimal, anterior, diffuse and apical. Apart from

clinical presentation, imaging is very helpful in
diagnosis. NSOIS is usually acute or subacute in onset
and painful. It is usually unilateral, and occasionally
bilateral. Rarely it is recurrent. The visual symptoms
include diplopia, and defective vision. Histologically
NSOIS is characterized by polymorphous infiltrations.
Nonspecific Myositic Inflammation

This is the most common presentation in our
experience. We manage patients presenting as a single
muscle disease with nonsteroidal anti-inflammatory
drugs and low dose corticosteroids. Recurrence is
unlikely in them. Patients presenting with multiple
muscle disease, are prone to recurrences. We treat
them more aggressively with oral Prednisolone 2
mg/kg body weight tapered over 4-6 weeks or with
intravenous methyl prednisolone2 pulse therapy.
Patients with recalcitrant disease require
immunosuppressives. Such patients were found to
benefit from methotrexate in a dose of 15-25 mg per
week3 usually marked clinical response is evident
within a week. Those cases which fail to respond to
the drugs warrant a biopsy to exclude a lymphoma.
Nonspecific Lacrimal Inflammation

Nonspecific dacryoadenitis should prompt a
suspicion for systemic disease where a percutaneous
biopsy is recommended first. Management includes
moderate doses of steroids such as 1mg/kg oral
prednisolone which can be tapered over 4-6 weeks.
Majority of nonspecific dacryoadenitis resolves over
6-12 weeks.
Specific Inflammations of the Orbit

These are the most common etiological factors in
proptosis for which medical management is
commonly carried out. Therapeutic options in
management of inflammations are ever expanding
not only because biologically targeted agents are
becoming increasingly available that can act on
specific segments of inflammatory cascades but also
because of advances in our understanding of
etiopathogenesis.
Orbital Cellulitis
This is the most common cause of painful proptosis,
acute in onset and most often unilateral. The principles
of management of a case of orbital cellulitis are control
of infection by the use of appropriate antibiotics,
preventions of ocular as well as nonocular
complications, surgical drainage when necessary and
careful follow up. We insist on imaging on an
emergency basis in every case of orbital cellulitis for
two reasons : (1) To make sure that we are not missing
other neoplastic lesions like Rhabdomyosarcoma,
Retinoblastoma which clinically mimic orbital
cellulitis (2) To plan the course of action: We prefer
surgical drainage of orbital or sub-periosteal abscess.
In the absence of abscess, medical management is
preferred. The patient should be carefully monitored
during the treatment for any threat of loss of vision,
and clinical response to the drugs. From our
experience and also from the literature we wish to
emphasize that there is a great difference in the
prognosis and hence management strategies of orbital
cellulitis in children and adults. Usually in children
under the age of 9 years the infection is by a single
aerobic organism such as pneumococci4 and respond
to medical management, the threat to affect vision is
rare and surgery is rarely needed. In contrast adults
harbor polymicrobial infection and the threat to vision
is common and hence drainage is frequently required
in addition to systemic antibiotics.5
Orbital cellulitis secondary to sinusitis is known
to harbour organisms like Strep pneumoniae, H
influenzae, Bacteroids and anaerobic cocci. Recommended antibiotics include third generation
cephalosporins like cefotaxime, ceftriaxone and
cefuroxime. Alternatively piperacillin with
taxobactum or ticarcillin with clavulanate can be used.
Orbital cellulitis secondary to trauma or foreign-body
are known to be harbouring organisms like S aureus,

S epidermidis, Streptococci and anerobes. Recommended antibiotics for such cases include
Vancomycin along with third generation cephalosporins or imipenem1 Imaging with a CT or a MRI
can accurately monitor the progress and effect of
treatment. Septic thrombosis of cavernous sinus
either due to spread from contiguous structures or
septicemia demands prompt recognition and
treatment with broad spectrum antibiotics as
discussed above for optimal clinical outcome.
Rhino-orbital Mucormycosis
This is one of the very harmful infections and can
lead to death. It is almost always associated with
uncontrolled diabetes mellitus and usually with
ketoacidosis.6-8 Once the diagnosis is established
based on clinical findings, microscopic fungal
examination and culture, a multidisciplinary approach
is commonly practiced. Diabetes should be managed
simultaneously. Following wide excision of
devitalized tissue, the area is daily irrigated with
amphotericin. Systemic treatment with amphotericin
is also recommended. Some believe in the usefulness
of hyperbaric oxygen for such cases.9
Chronic Granulomatous Infections

Since the advent of AIDS, incidence of certain
granulomatous infections of importance in proptosis
like tuberculosis and syphilis is on a rise.
Orbital involvement in tuberculosis is usually by
direct invasion from sinuses or hematological
dissemination. Periostitis, cold abscess and orbital
tuberculomas are well recognized lesions.10 In doubtful
cases PCR is helpful. Orbital involvement is diagnosed
with a high index of suspicion and aspiration biopsy.
Systemic anti-tuberculous drugs are recommended in
coordination with a chest physician.
Though literature says that syphilis is on a rise
with immunosuppressive syndromes, we are yet to
come across a case of syphilis with orbital involvement. Periostitis, acute and chronic inflammations are
recognized lesions. Systemic antibiotic therapy usually
with penicillins is useful in resolution of the disease.
Parasitic Infestations
Cysticercosis, echinococcosis and trichinosis are
common parasitic etiological factors in the causation
of proptosis.
Medical Management of Proptosis 339

Cysticercosis is caused by C.cellulosae, the
larval form of tapeworm, Taenia solium. Though orbit
is considered to be a rare site in the west, many
reports in the literature suggests that orbital
involvement is most frequent among Asians.11 Once
diagnosis is established with the help of imaging and
serology, systemic albendazole (15 mg/kg) along
with steroids (prednisolone 1-2 mg/kg) for a period
of four weeks is found to be effective11 If there is
evidence of associated neurocysticercosis, treatment
is with steroids (prednisolone 1mg/kg) and
praziquantel 50 mg/kg in three divided doses for a
period of 15 days. Association between orbital
myocysticercosis and neurocysticercosis is not very
common. We prefer to refer these patients to a
neurologist for in-patient treatment since there is a
rare possibility of generalized seizures. The response
can be monitored and progress can be documented
with imaging.
Echinococcosis or hydatid cyst as it is
commonly called is an intestinal infestation of dogs.
Orbital cysts are seen in 1% of echinococcosis.
Systemic albendazole has been found to be effective
in resolving the cyst.15 Steroids are recommended
for violent inflammatory reactions following rupture
of the cyst during aspiration or attempted surgical
removal16 Certain studies have shown high efficacy
in disease resolution when combination of
praziquantel with albendazole12-14 is used.
Trichinosis occurs as cysts in extraocular muscles,
which may show evidence of calcification on imaging.
Treatment recommended include systemic thiabendazole along with steroids to reduce
inflammation. Personally we have no experience as
we are yet to come across a case of trichinosis with
orbital involvement.
Vasculitis
Vasculitis or angiitides as some may call it is a clinical
syndrome that encompasses acute or chronic
inflammation of vessels with vaso-obliterative signs
and symptoms17 Most common vasculitis that involve
the orbit include Wegener's granulomatosis and
Polyarteritis nodosa. Diagnosis is usually established
by imaging, biopsy with a histopathological
examination and specially for wegener's a serological
examination in the form of C-ANCA 15 Dramatic
improvement is noticed when systemic steroids are
combined with an alkylating agent like cyclo-
phosphamide.21,22 Recent studies have suggested a

role of anti-TNFs (tumor necrosis factors) like
Infliximab and Etanercept. The development of novel
approaches focusing on blockade of specific molecules
including TNF alpha is awaited. 22 Another novel
approach that is showing promise in the management
of refractory Wegener's and C-ANCA related
vasculitis is the use of Rituximab, a chimeric antiCD20 monoclonal antibody.23,24
Tolosa-Hunt Syndrome
This nonspecific granulomatous inflammation though
rare is nevertheless an important differential diagnosis
of apical orbital inflammations. The clinical course is
marked by remissions and recurrences. SPIR MRI
(spectral presaturation with inversion recovery MRI)
has been recommended for diagnosis1 Management
includes a high dose of systemic steroids which often
produces a dramatic clinical improvement. SPIR MRI
before and after corticosteroids have been found to
be useful in some studies for definite diagnosis and
monitoring of the disease.1
Vascular Lesions
Treatment is indicated when vision is threatened by
amblyopia as a result of anisometropia, ptosis or
strabismus.
Intralesional injection of steroids is the most
frequently used method. Usually 40-80 mg of
triamcinolone with 25 mg of methylprednisolone is
directly injected into the lesion. 1 Alternatively
Triamcinolone 40 mg in combination 4 mg
betamethasone can be used. The tumor usually begins
to regress in two weeks but if necessary injection
may be repeated after about two months. Early
recognition and prompt treatment with intralesional
steroid prevents amblyopia exanopsia, but followup and management of refractive amblyopia with
glasses and patching is necessary in the longer term.
Potential complications include skin depigmentation,
fat atrophy, eyelid necrosis and rarely central retinal
artery occlusion.
Systemic steroids are indicated for extensive
lesions specially if associated with visceral
involvement. Recommended dosage used is
1.5 mg/kg to 2.5 mg/kg Prednisolone daily over a
few weeks with titration downward depending on
response.1

Though steroids are effective in large majority
of patients, a recurrence is not infrequent. Recurrent
or resistant cases are being treated with recombinant
interferon alpha-2a and 2b with variable results16
Recent studies have demonstrated good efficacy of
interferons when given subcutaneously in a dose of
3 million units/m 2. During clinical follow-up
diagnostic ultrasound evaluation ( the depth
dimension) proved helpful. One report suggested
high efficacy of treatment when a combination of
interferon alpha-2a with a low dose of cyclophosphamide.17
In the presence of very large platelet-consuming
lesions as seen with Kasabach-Meririt syndrome,
systemic antifibrinolytics like aminocaproic acid or
tranexemic acid are used.18
Structural Lesions
Acute Intraorbital Hemorrhage and Emphysema
Post-traumatic fractures, soft tissue injury, contusions
and retrobulbar blocks may be associated with acute
rise in intraorbital pressure as blood is trapped in
confined spaces. The effects of such rapid rise in the
pressure include optic nerve ischemia and retinal
hypoperfusion. The optic nerve head may
demonstrate arterial pulsations. Hence the
importance of prompt recognition and early
management cannot be overemphasized. Though
severe visual threat is a surgical emergency, for
moderate degrees of orbital tension, treatment
includes 500 mg of acetazolamide i.v., and mannitol
1-2 ml/kg i.v over 30 minutes has been advocated.1,19
Orbital emphysema is another cause of acute
orbital tension and is almost always secondary to
trauma. This rarely requires decompression as the
air tends to absorb rapidly. Though most of the
patients are managed with antibiotics, prophylactic
use is usually not required for clean wounds.20
advances. Among the lymphoproliferative lesions,

reactive lymphoid hyperplasia appears to be steroid
sensitive as it responds to moderate doses of
Prednisolone. Failure to respond to steroids can be
managed by cytotoxic agents and low dose
radiotherapy.1
Another clinicopathological entity; the indeterminate lymphoproliferative lesions are steroid
resistant and may require treatment with immunosupressives or radiotherapy.1 The widespread use
of chemotherapy for lympho-proliferative lesions and
other neoplastic conditions is being dealt in detail in
a separate chapter.
CASE ILLUSTRATIONS
Case 1
Mrs.K, female 54 years presented with acute, painful
proptosis of left eye of 5 days duration. She had
severe pain, nausea and mild fever. There was a very
severe edema of the lids and periorbital edema
(Figure 24.1A). The upper lid had complete ptosis.
On everting the upper lid, the globe was found to be
proptosed. The conjunctiva was congested and
chemosed. Ocular motility was restricted (Figures
24.1B and C).CTscan showed orbital cellulitis without
any abscess. She was given intravenous (Amoxicillin
and clavilanic acid) and Metronidazole, with which
she showed a marked improvement within 5 days.
She was relieved from pain, proptosis reduced, and
the ptosis improved markedly( Figure 24.1D) The
conjunctival chemosis and congestion improved and
the ocular motility restored to normal (Figures 24.1E
and F).
Lymphoproliferative and Other Neoplastic

Lesions
These disorders encompasses a wide range of clinical
syndromes. The advent of immunodiagnostics and
molecular techniques had a profound effect on better
understanding of pathogenesis and therapeutic
Figure 24.1 A: Female 54 years presented with acute, painful proptosis of left eye. Note the severe edema of the lid and periorbital
edema, and the gross ptosis
C
Figures 24.1B and C: On elevating the lid, note the conjunctival congestion and chemosis.
Note the restricted ocular motility both in adduction (B) and abduction (C)
Figure 24.1D: After medical management, note the improvement in the edema
of the lids, ptosis, chemosis and congestion of the conjunctiva
F
Figures 24.1E and F: Note the restoration of ocular motility both in adduction (E) and abduction (F)
Case 2
Female 17 years, presented with proptosis of right
eye associated with mid pain since 1 month. There
was no history of trauma, defective vision or
diplopia. Examination revealed mild proptosis of the
right eye with fullness of right upper lid in the
supero-temporal region (Figure 24.2A). CTscan of
the orbit revealed enlarged lacrimal gland molding

to globe (Figure 24.2 B). The possibility of lymphoma
was thought off. FNAC and immunohistochemistry
were negative for lymphoma. Hence, a diagnosis of
nonspecific orbital inflammation, involving the
lacrimal gland was made; the girl was treated with
systemic steroids to which she responded well
(Figure 24.2C).

CT scan revealed inflammation at the apex of the
orbit (Figures 24.3E and F) with mild enlargement
of superior ophthalmic fissure. In view of subacute
onset, associated pain, restricted ocular motility and
defective vision, a diagnosis of superior ophthalmic
fissure syndrome was made, and he was treated with
systemic steroids. The patient responded very well.
The vision improved from 20/200 to 20/30 in a
fortnights time.
Figure 24.2 A: Note the fullness at the supero-temporal region of the
right upper lid, with mild displacement of the globe and minimal ptosis
Figure 24.3A: Male 28 years, presented with severe ptosis of right

upper eye lid and mild proptosis of right eye
Figure 24.2B: Coronal section of CT scan of orbit showing

enlarged lacrimal gland molding to the globe
Figure 24.3B: Note the restricted adduction in the right eye
Figure 24.3C: Note the restricted depression in the right eye

Figure 24.2C: One week after oral prednisolone, note the improvement in the fullness of the supero-temporal region of the right upper
lid, and in ptosis
Case 3
Male 28 years presented with proptosis of right eye,
subacute in onset and associated with mild pain and
defective vision( Figure 24.3A) On examination, he
had mild proptosis associated with ptosis, and
restricted ocular motility (Figures 24.3B to D).
Figure 24.3D: Note the restricted abduction in the right eye

8. Nithyanandam S, Jacob MS, Battu RR, Thomas RK, Correa
MA, D'Souza O. Rhino-orbito-cerebral mucormycosis. A
retrospective analysis of clinical features and treatment
outcomes Indian J Ophthalmol. 2003;51(3): 231-6.
9. Ferry AP, Abedi S. Diagnosis and management of rhinoorbitocerebral mucormycosis. A report of 16 personally
observed cases. Ophthalmology 1983;90:1096-104.
F
Figures 24.3E and F: CT scan, Axial and sagital sections of the orbit
show a hyperdense lesion abutting the optic nerve at the orbital apex.
Its margins are indistinct. Axial section of the CT shows enlarged
superior ophthalmic fissure
REFERENCES
1. Rootman J: Diseases of the orbit ; A multidisciplinary
approach. Lippincott Williams and Wilkins, (2nd ed): 455506.
2. Nugent RA, Rootman J, Robertson WD, et al. Acute orbital
pseudotumors: AJNR 1981;2:431-6.
3. Hemady R, Tauber J, Foster CST. Immunosuppressive
drugs in immune and inflammatory ocular disease. Surv
Ophthalmol 1991;35:369-85.
4. Donahue SP, Schwartz G. Preseptal and orbital cellulitis in
childhood: a changing microbiologic spectrum.
Ophthalmology 1998; 105:1902-6.
5. Harris GJ. Subperiosteal abcess of orbit. Age as a factor in
the bacteriology and response to treatment. Ophthalmology 1994;101:585-95.
6. Ameen M, Arenas R, Martinez-Luna E, Reyes M, Zacarias
R: The emergence of mucormycosis as an important
opportunistic fungal infection: five cases presenting to a
tertiary referral center for mycology. Int J Dermatol. 2007
Apr;46(4):380-4.
7. Bhadada S, Bhansali A, Reddy KS, Bhat RV, Khandelwal N,
Gupta AK : Rhino-orbital-cerebral mucormycosis in type 1
diabetes mellitus, Indian J Pediatr. 2005;72(8):671-4.
10. Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal

11. Honavar SG, Sekhar.G, Orbital Cysticercosis. Orbit 1998;
17(4): 271-84.
12. Srivastava VK, Srivastava A, Singhal KC. Albendazole
therapy in orbital cysticercosis. Ind J Physiol Pharmacol
1996; 40:265-66.
13. Richards KS, Morris DL Effect of albendazole on human
hydatid cysts: an ultrastructural study. HPB Surg 1990; 2:
105-13.
14. Gomez MA, Croxatto JO, Crovetto L, Ebner R. Hydatid
cysts of the orbit. A review of 35 cases. Ophthalmology
1998; 95:1027-32.
15. Perry SR, Rootman J, White VA. The clinical and
pathological constellation of wegener's granulomatosis of
the orbit. Ophthalmology 1997; 104:683-94.
16. Nolle B, Coners H, Duncker G. ANCA in ocular
inflammatory disorders. Adv Exp Med Biol 1993;336:
305-7.
17. Teske S, Ohlrich SJ, Gole G, et al. Treatment of orbital
capillary hemangioma with interferon. Aust N Z J
Ophthalmol 1994; 22: 13-7.
18. Neidhart JA, Roach RW. Successful treatment of skeletal
hemangioma and Kasabach-Merritt syndrome with
aminocaproic acid. Am J Med 1982; 73: 434-8.
19. Rootman J, Stewart B, Goldberg RA. Orbital Surgery:
A conceptual approach. Philadelphia: Lippincott-Raven,
1995.
20. Fleishman JA, Beck RW, Hoffman RO. Orbital emphysema
as an ophthalmologic emergency. Ophthalmology 1984;
91:1389-91.
21. Selamet U, Kovaliv YB, Savage CO, Harper L. ANCAassociated vasculitis: new options beyond steroids and
cytotoxic drugs. Expert Opin Investig Drugs. 2007;16(5):
689-703.
22. Svozilkova P, Rihova E, Brichova M, Diblik P, Kuthan P,
Poch T. Infliximab in the treatment of Wegener's
granulomatosis: case report. Cesk Slov Oftalmol.
2006;62(4):280-6.
23. Tamura N, Matsudaira R, Hirashima M, Ikeda M, Tajima
M, Nawata M, Morimoto S, Kaneda K, Kobayashi S,
Hashimoto H, Takasaki Y. Two cases of refractory
Wegener's granulomatosis successfully treated with
rituximab.
24. White ES, Lynch JP Pharmacological therapy for Wegeners
granulomatosis. Drugs. 2006;66(9):1209-28.
25
CHAPTER
Management of Ophthalmic
Tumors: Role of Chemotherapy
and Radiation Therapy
Vijay Anand P Reddy, Nitin More, Ramesh Murthy, Anirban Bhaduri, Santosh G Honavar
INTRODUCTION
Ocular Oncology deals with the diagnosis, surgical
and nonsurgical management of tumors involving
the eyelids, external ocular surfaces, intraocular
structures and the orbit. Ocular tumors invariably
are managed by multidisciplinary team of ocular
surgeon and an oncologist experienced in the
treatment of such tumors.
Radiation Oncology is the clinical and scientific
discipline devoted to the management of patients
with cancer and other diseases with ionizing
radiation alone or combined with other modalities
like surgery and chemotherapy. The aim of radiation
therapy is to deliver a precisely measured dose of
radiation to a defined tumor volume with minimal
damage to surrounding healthy tissue.
Radiation used for cancer treatment is called
ionizing radiation because it forms ions in the cells
of the tissues it passes through, as it dislodges
electrons from atoms. Ions are atoms that have
acquired an electric charge through the gain or loss
of an electron. This can kill cells or change genes.
Other forms of radiation, such as radio waves,
microwaves, and light waves are called non-ionizing.
They have lower energy and hence can not ionize
cells.
Ionizing radiation is of two major types

a. Non-particle Photons (X-rays and -rays), which
are most widely used.
b. Particle radiation (electrons, protons, neutrons).
The common types of radiation used for cancer
treatment are:
1. High-energy photons come from radioactive

sources such as cobalt, cesium, or a machine
called a linear accelerator. This is by far the most
common type of radiation treatment in use
today.
2. Electron beams produced by a linear accelerator
or beta particle emitting radioactive source like
Strontium and Ruthenium. They are used for
tumors close to a body surface, e.g. skin,
conjunctiva and sclera.
3. Protons are a newer form of treatment. Protons
are parts of atoms that cause little damage to
tissues they pass through but have maximum
effect at the end of their path. This means that
proton beams may be able to deliver more
radiation to the cancer while causing fewer side
effects to normal tissues nearby. Although it is
used routinely for certain types of ocular and
brain tumors, it still needs more study in others.
4. Neutrons are used for some cancers of the head,
neck, and prostate. They can sometimes be
helpful when other forms of radiation therapy
do not work especially when the tumor has
anoxic zones.
Radiation therapy delivery methods are as

follows:
1. External Beam Radiation (Teletherapy): It is
the most widely used type of radiation therapy.
The radiation is focused from machine outside
the body onto the area affected by the cancer.
This type of radiation is most often given by
either radioactive source like cobalt or cesium
or with linear accelerators (Figure 25.1). The
Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 345

radiation is aimed at the tumor, but also affects
the normal tissue it passes through on its way
into and out of the body. External beam
radiation allows large areas of the body to be
treated and allows treatment of more than one
area such as the primary tumor and nearby
lymphnodes. External radiation is usually given
in daily treatments, 5 days per week over
several weeks.
2. Internal Radiation Therapy (Brachytherapy):
It is also known as brachytherapy, which means
short-distance therapy. With this method,
radioactive sources are placed directly into the
tumor or into a cavity close to the tumor. The
advantage of brachytherapy is the ability to
deliver a high dose of radiation to a small area.
It is useful in situations that require a high dose
of radiation. The main types of internal radiation
are:
a. Interstitial radiation: The radiation source is
placed directly into or next to the
tumor using small pellets, wires, tubes, or
containers. For example, carcinoma tongue
b. Intracavitary radiation: A container of
radioactive material is placed in a cavity of
the body such as the vagina, nasopharynx.
c. Surface (mould): Radiation sources are placed

over the tissue to be treated, e.g. Ca hard palate.
d. Plaque Brachytherapy: Concave shaped
radioactive plaque is placed over the sclera
for ocular melanomas, retinoblastoma, etc
(Figure 25.3A).
e. Intraluminal: Sources are placed in a lumen,
e.g. carcinoma esophagus.
f. Intravascular: A single source is placed into
small or large arteries such as coronary artery
for prevention of stent restenosis.
Based on the duration of treatment, brachytherapy
is classified as:
a. Permanent (low dose rate) Brachy therapy: Dose is
delivered over the lifetime of the source until
complete decay.
b. Temporary (high dose rate): Dose is delivered over
a short period of time and the sources are
removed after the prescribed dose has been
reached.
B
Figure 25.1: Teletherapy machine for external
beam radiation therapy
Figure 25.2A and B: Interstitial brachytherapy

Radiation therapy may be used alone or in
combination with other cancer treatments, such as
chemotherapy or surgery. In some cases, a patient
may receive more than one type of radiation therapy.
External Radiotherapy in Ocular Tumors

Linear accelerators equipped with both photon and
electron facility and multileaf collimators (MLC) are
mainly used for the external radiation therapy of
orbital tumors where multiple radiation beams are
focused on the tumor. Simulator with all the
parameters similar to linear accelerator but capable
of only diagnostic X-rays is used for radiation
therapy planning. It simulates the beams of the
external radiotherapy machines before the patient is
being taken for the actual treatment.
Conventionally radiation therapy planning is
carried out by immobilizing the patient in treatment
position and a CT scan is done in the treatment
position with the immobilization in place. The images
are transferred to the computerized treatment
planning system. The tumor volume and the critical
structures are delineated by the radiation oncologist
and then the medical physicist plans the various beam
angles and energies and gives various treatment
options. The optimal plan is selected by the radiation
oncologist and the patient is simulated according to
bony landmarks visible under fluoroscopy and the
data given by the treatment planning system. The
computerized scan is invariably being used in all
orbital tumors and the 3-dimensional conformal
radiation therapy (3D-CRT) is planned.
Figure 25.3A: Radioactive plaques for intraocular tumors
All the efforts in the development of radiation

therapy techniques are directed towards proper
inclusion of tumor in the target volume and to spare
the surrounding normal tissues. Intensity modulated
radiation therapy (IMRT) is newer form of external
radiation therapy that is capable of obtaining desired
dose distribution in irregular and concave shapes
sparing the adjoining critical organs like optic chiasm
and pituitary gland.
Plaque Radiotherapy
A radioactive plaque is a device that can be used to
deliver a high dose of radiation precisely and
selectively to a tumor and negligible dose to the
surrounding structures. It is made with radioactive
Cobalt, Ruthenium, Iridium, Palladium or Iodine
sealed within. Plaques come in various shapes
and sizes ranging from 10 to 25 mm in diameter
(Figure 25.3B).
The procedure is done under anesthesia.
Preoperative assessment of the lesion: location, size
and thickness are measured by means of ultrasound
B scan and the details are given to the ocular
radiation oncologist. The radiation oncologist along
with the radiation medical physicist plans the
treatment with the help of computerized automated
dosimetry software. An appropriate plaque, the
radiation dose, dose rate and treatment time is
selected. The treatment time may vary from 24 to 96
hours based on type and size of tumor. This process
needs expertise of a radiation therapist and a
radiation physicist well versed in brachytherapy.
Figure 25.3B: Ruthenium 106 plaque being placed on the eye for a
choroidal melanoma

After obtaining a proper consent the patient is taken
for the operative procedure. The concave shaped
dummy plaque similar to the radioactive plaque is
placed and checked and then the radioactive plaque
is placed and sutured in place (Figure 25.4). Patient
is placed in isolation till the entire period of
radiotherapy. The plaque is removed under
anesthesia after the required dose of radiation is
delivered.
Indications of plaque brachytherapy are as
follows:
1. Retinoblastoma measuring < 16 mm in basal
diameter and < 8 mm in thickness a primary
treatment, as an adjuvant to chemoreduction,
and for failure of focal therapy.
2. Primary treatment for most medium-sized and
some large choroidal and ciliary body
melanomas in an eye with salvageable vision.
3. Choroidal hemangioma.
4. Choroidal metastasis.
5. Extensive retinal capillary hemangioma.
Cell Cycle and the Principles of Anti-neoplastic

Therapy
It is important to understand the growth pattern of
tumor cells that affect the overall biological behavior
of tumor and response to anti-neoplastic therapy,
either radiation or cytotoxic chemotherapy. Cell cycle
is composed of four distinct phases. The G1 phase

consists of cells that have recently completed division
and are committed to continued proliferation. After
a variable period of time, these cells begin to
synthesize DNA, marking the beginning of the S
phase. After DNA synthesis is complete, the end of
the S-phase is followed by the premitotic rest interval
called the G2 phase. Finally, chromosome
condensation occurs and the cells divide during the
mitotic M phase. Resting diploid cells that are not
actively dividing are described as being in the G0
phase.
Ionizing radiation generally affects the neoplastic
cells those are in synthetic and mitotic phase where
the DNA of the cell is damaged either temporarily
or permanently, causing subsequent cell death.
Radiation therapy is usually delivered in multiple
fractions to target the tumor cells which were in
resting phase during earlier fraction of radiation.
Similarly, some anticancer agents induce their
cytotoxic effects during specific phases of the cell
cycle. Chemotherapeutic agents are used either as
single agent or in the combination of different agents.
Combination chemotherapy agents are selected
according to different mechanism of action to have
synergistic effect and with different toxicity profile.
Cumulative doses of individual drugs are typically
low in combination chemotherapy regimens,
potentially minimizing the long-term toxicity and
improving the therapeutic ratio.
Management of Ophthalmic Tumors

Ocular tumors with proptosis as the first symptom
arise from eyelid and lacrimal gland, orbital soft
tissues, optic nerve and orbital bones. Prominent ones
among those are squamous and basal cell carcinoma
of eyelid, rhabdomyosarcoma, non-hodgkin's
lymphoma, optic nerve glioma, meningioma and
idiopathic orbital inflammation. Occasionally,
retinoblastoma in advanced stage could present with
proptosis of eye.
Tumors of the Eyelid
Fig. 25.4: Cell cycle
Most eyelid masses are benign tumors such as skin

squamous cell papilloma, melanocytic nevus or
congenital and acquired cysts. Common malignant
tumors of eyelid include basal cell and squamous cell
carcinoma.
It is the most common pediatric eyelid tumor. It may
be a component of Hippel-Lindau syndrome or
Surge-Weber syndrome. The natural history of
lesion is spontaneous regression over 3 to 4 years,
therefore usually these lesions are observed.
Indications of treatment are obstruction of the vision,
amblyopia, ulceration of the eyelid due to vascular
compression. Steroids are the first line of treatment.
Radiation therapy is reserved until other treatment
methods have failed. Low energy photons or
electrons are used in dose of 500 to 750 cGy in 2-3
fractions or fractionated low dose to a total of 1600
to 2000 cGy.1
Basal Cell and Squamous Cell Carcinoma

Basal cell carcinoma (BCC) represents 90% of
malignant eyelid tumors. Its four morphological types
are the nodular, ulcerative, pigmented and the
morpheaform tumor. It mostly involves the lower
eyelid. More likely to affect fair skinned persons
with high solar exposure. It may be mistaken for a
chalazion or chronic blepharitis. Complete excision
with frozen section control of tumor margins using
cryo is the standard treatment for localized tumors.
Larger lesions are treated with definitive surgery
and appropriate reconstruction.
Squamous cell carcinoma (SCC) is the second most
common malignancy of the eyelids. Sun exposure is
the most important factor in developing SCC of the
skin. It may occur in previously normal appearing
skin or more commonly arises from a pre-existing
lesions like actinic keratoses, skin damaged by
ionizing radiation or xeroderma pigmentosum.
Unlike BCC of the eyelid, SCC can be an aggressive
tumor and has the potential to invade the orbit,
metastasize to lymph nodes and distant sites.
Primary excision is curative for small lesions in
basal cell and squamous cell carcinoma of eyelid.
Irradiation could be used for unresectable and
recurrent basal cell carcinoma. It also could be used
as an alternative to surgery with more than 90% of
cure rate if patient prefers radiation therapy over
surgery. Cryotherapy could be used for recurrent
lesions; usually in medial canthus. Orbital
exenteration is reserved for deep invasive lesions.
Radiation dose of 50 to 60 Gy should be delivered
with low energy X-rays or electrons with appropriate

shielding of lens.2
Sebaceous Carcinoma
Ocular sebaceous carcinoma is a very rare but
aggressive tumor, most commonly occurs in patients
60 to 80 years of age although the range is from early
childhood through the nineties. It usually arises from
the meibomian glands followed by glands of Zeiss
and caruncle. It may be multicentric resulting in local
recurrences. The incidence appears to be somewhat
greater in women and in Asian population.3
General principle of treatment is wide excision
(with 5 to 6 mm surgical margins) with either frozen
section or permanent section control as primary
management of sebaceous carcinoma.4 Map biopsies
of eyelids and conjunctiva should be carried out.
Radiotherapy provides acceptable cosmesis both for
primary treatment and for treatment of recurrent
disease. Somewhat higher irradiation dosages in the
range of 60 to 65 Gy in six to seven weeks are
recommended, and the control rates are in the range
of 80 to 90% or better.5
Tumors of Lacrimal Gland

Epithelial tumors of lacrimal gland could be benign,
e.g. pleomorphic adenoma (benign mixed tumor) or
malignant, e.g. adenoid cystic carcinoma or
pleomorphic adenocarcinoma (malignant mixed
tumor). On imaging, pleomorphic adenoma appears
as round to ovoid superotemporal orbital mass which
may cause bony indentation, without bone erosion.
Malignant tumors have irregular margin, often with
adjoining bone destruction.
Complete excision of mass (excisional biopsy) is
curative for pleomorphic adenoma. Incomplete
excision can lead to recurrence and malignant
transformation.
Malignant tumors should be completely excised
if possible. Routinely postoperative radiation therapy
to a dose of 5000 to 6000 cGy is delivered. In locally
advanced lacrimal gland carcinoma, neoadjuvant
chemotherapy with cisplatin and 5-fluorouracil
followed by surgery and postoperative radiation
therapy is given. Results of intracarotid
chemotherapy with Cisplatin and Doxorubicin
followed by surgery and radiation therapy are
encouraging6,7
Malignant Conjunctival Tumors

Squamous cell carcinoma is the most common primary
malignant tumor of conjunctiva, manifests usually as
a fleshy vascularized mass at the limbus. Conjunctival
tumors are treated by complete excision biopsy with
frozen section control of tumor margins and
cryotherapy of the tumor bed. Reconstruction can
be done then by simple closure, conjunctival grafting
or amniotic membrane transplant.
Primary or adjunctive use of local treatment with
some chemotherapeutic agents such as MitomycinC, 5- Flourouracil and Interferon alpha 2-b have been
reported. In some diffuse radiosensitive tumors such
as lymphoma, fractionated external beam radiotherapy or application of a radioactive plaque may
be employed.
Intraocular Tumors
Intraocular tumors arise from iris, choroid, retina or
optic nerve head. Iris masses could be melanocytic
as nevus and melanoma, granuloma, hemangioma,
leiomyoma, lymphoma, metastases, and extension
from a ciliary body tumor. Choroidal melanoma is
the most common intraocular tumor in adults. Other
common intraocular pigmented tumors include optic
nerve head melanocytoma, retinal pigment
epithelium adenoma and combined hamartoma of
retina and retinal pigment epithelium. Choroidal nonpigmented masses include amelanotic melanoma,
uveal granuloma, lymphoma, osteoma and choroidal
metastases. Retinal non-pigmented masses include
retinoblastoma and toxocara granuloma.
Choroidal Melanomas
Small Lesions <1.5 mm height without high risk
factors like juxta papillary location, presence of
subretinal hemorrhage, presence of orange pigment
are closely observed. Lesions of 1.5 to 10 mm height
are treated according to its location. Peripheral lesions
are locally excised. Central and mid peripheral lesions
with size < 4 mm are treated with trans-pupillary
thermotherapy (TTT). Lesions with > 4 mm of size
are treated with plaque therapy or external radiation
therapy with photons or protons. Lesions of >10 mm
in height are treated with enucleation or external
radiation therapy.
Ruthenium-106 is currently the most commonly
used isotope for plaque radiotherapy of choroidal
melanomas, although cobalt-60, Iodine-125, iridium192, strontium-90, and palladium-103 have also been
used. Modern techniques for plaque brachytherapy
involve suturing a shielded plaque containing seeds
of the radioactive isotope to the sclera.8,9 This remains
in place for a specified number of days in order to
deliver the proper dose of radiation. Most melanomas are treated with a calculated apex dose of 70
to 85 Gy.10
Intraocular Lymphoma
This is a rare variety of non-Hodgkin's lymphoma,
large cell lymphoma being the most common
histology. Uveal tract, retina, vitreous or optic nerves
are usually involved. Vitreoretinal involvement is
usually associated with central nervous system
lymphoma. Diagnosis is usually done by vitreous
biopsy. Usually they do not have systemic
manifestation. Recommended treatment is external
radiation therapy to a dose of 3600 to 4000 cGy at
1.8-2 Gy fractions.11
Retinoblastoma
For over 100 years or longer the treatment of
intraocular retinoblastoma has been enucleation.
Various forms of radiation treatment have been used
in the management of retinoblastoma since World
War II. The goal of radiation has been to destroy the
tumour, save the eye, and maximise visual potential.
Since the radiation in the paediatric age group has
its potential long-term side effects newer modalities
like chemotherapy has been attempted. Since the
introduction of platinum and etoposide based
chemotherapy there has been tremendous
improvement in tumor control and survival.
Current standard treatment options for
retinoblastoma include the following:
1. Cryotherapy: used in addition to radiation or in
place of photocoagulation for lesions smaller
than 4 disc diameters in the anterior portion of
the retina.
2. Photocoagulation: occasionally used alone with
small tumors. It is used for posteriorly located
tumors that are smaller than 4 disc diameters,
distinct from the optic nerve head and macula,
and without involvement of large nutrient
vessels or choroid involvement in patients with
early-stage disease (in addition to radiation

therapy) or when there is limited recurrence
following radiation therapy. Thermotherapy
delivered via infrared radiation is an alternative
to laser photocoagulation.12
3. Chemoreduction: Systemic chemotherapy is used
to reduce tumor volume with intraocular
tumors making them suitable for treatment with
cryotherapy or photocoagulation.13,14 Factors
such as tumor location (macula), patient age,
and tumor size correlate with responsiveness
to chemotherapy.15,16 Most tumors are treated
with combination chemotherapy, Inj.Vincristine,
Inj. Etoposide and Inj. Carboplatin. The dose of
these drugs depends on the age, stage and the
intention of treatment, either chemoreduction
or adjuvant chemotherapy (Table 1). They also
require additional local therapy. Overall, the
response rate is highest for tumors that are
unilateral or unifocal and without vitreous
seeding.
4. Subtenon (subconjunctival) chemotherapy:
Carboplatin is administered by the treating
ophthalmologist into the subconjunctival space.
This modality is undergoing testing in phase I
and II trials and is generally used in conjunction
with systemic chemotherapy and local
ophthalmic therapies for retinoblastoma with
vitreous seeding. This approach offers some
promise in this group of patients.17,18
5. Surgery (enucleation) is usually undertaken
when unilateral disease is massive and there is
no expectation that useful vision can be
preserved. Careful examination of the enucleated specimen by an experienced pathologist
is necessary to determine whether high-risk

features for metastatic disease are present.
Postoperative external radiation therapy is
indicated in the presence of optic nerve
extension to transection, scleral infiltration and
extrascleral extension. Systemic standard
chemotherapy for six cycles is indicated for
anterior chamber seeding, infiltration of iris,
ciliary body infiltration, massive choroidal
infiltration or optic nerve extension beyond
lamina cribrosa. High dose chemotherapy for 6
cycles is used in patients with combined
choroidal infiltration and optic nerve extension
beyond lamina cribrosa or with scleral
infiltration. High dose chemotherapy for 12
cycles is used in patients with extrascleral
extension and optic nerve extension to
transection. Vincristine, doxorubicin, and
cyclophosphamide, or vincristine, carboplatin,
and etoposide are the drugs in different dose
schedules as described in (Tables 2 and 3).
Intrathecal methotrexate is given if CSF is
positive or there is radiological evidence of
intracranial extension.
6. External beam radiotherapy: Retinoblastoma is
generally a radiosensitive tumor. Presently, with
effective chemotherapy drugs the indications for
radiotherapy are minimized to avoid the late
effects of radiotherapy, like facial deformities
and second malignancies. External beam
radiotherapy is a method of delivering whole
eye irradiation to treat advanced retinoblastoma,
particularly when there is diffuse vitreous
seeding. Presently, the indications of external
radiotherapy are residual disease after chemo-
Table 1: Chemoreduction regimen

Standard dose regimen
Inj. Vincristine 1.5 mg/m2
(0.05 mg/kg for children </= 36 months of age; max 2 mg)
Day 1
Inj. Etoposide 150 mg/m2
(5 mg/kg for children </= 36 months of age)
Day 1+2
Inj. Carboplatin 560 mg/m2
(18.6 mg/kg for children </= 36 months of age)
Day 1
(0.05 mg/kg for children </= 36 months of age; max 2 mg)
Day 1
(12 mg/kg for children </= 36 months of age)
Day 1+2
(28 mg/kg for children </= 36 months of age
Day 1
High dose chemotherapy

Inj. Vincristine 1.5 mg/m2
Inj. Etoposide 250 mg/m
Inj. Carboplatin 750 mg/m

Table 2: Management of intraocular retinoblastoma
group I to IV-B
a. Focal therapy alone when appropriate
b. Standard chemoreduction 6 cycles + appropriate
sequential focal therapy
c. Chemocryotherapy at each cycle
d. Standard chemoreduction 12 cycles, when suboptimal
regression with chemotherapy at 6 cycles and RT is not
feasible (e.g. age < 1year)
e. Alternative chemotherapy regimen or RT when there is
no regression after 3 cycles of standard chemotherapy
or recurrence during chemotherapy.
Table 3: Management of intraocular retinoblastoma
group V-A and V-B
a. Unilateral Primary enucleation
b. BilateralHigh dose chemotherapy 6 cycles +
appropriate sequential focal therapy
c. Chemocryotherapy at each cycle
d. Periocular Carboplatin augmentation for group V-B
e. High dose chemoreduction 12 cycles when suboptimal
regression with chemotherapy at 6 cycles and RT is not
feasible (e.g. age < 1year)
therapy and local therapy, diffuse vitreous

seeds, recurrent after chemotherapy, post
enucleation (Scleral involvement, extraocular
extension, optic nerve involvement). Externalbeam radiation with dose ranges from 3,500 to
4,600 cGy. Special expertise is very essential to
treat pediatric ocular and orbital tumors. Newer
methods of delivering external-beam radiation
are being used in an attempt to reduce adverse
long-term effects. This includes intensitymodulated radiation therapy (IMRT),
stereotactic radiation therapy, and proton-beam
radiation therapy.19
7. Brachytherapy with radioactive plaques. These
are radioactive plaques in concave shapes, either
beta or gamma emitting. The commonly used
radioactive sources are Iodine125, Gold,
Iridium192 and Ruthenium106. Ruthenium and
125I plaque therapy is preferred because of its
favorable physical properties.20,21 They are used
for either focal unilateral presentations or
recurrent disease following previous externalbeam radiation. Indications of brachytherapy
are lesion < 16 mm in basal diameter, < 8 mm in
thickness, adjuvant to chemo reduction, failure
of local therapy.
Extraocular disease may be localized to the soft tissues

surrounding the eye or to the optic nerve beyond
the margin of resection. However, further extension
may occur into the brain and meninges with
subsequent seeding of the spinal fluid, as well as
distant metastatic disease involving the lungs, bones,
and bone marrow. High dose chemotherapy for 3 to
6 cycles are given and disease is reassessed.
Enucleation is performed followed by orbital external
beam radiation therapy. Further, high dose
chemotherapy is continued for total 12 cycles. In
extraorbital retinoblastoma, palliative therapy with
radiation (including craniospinal irradiation when
there is meningeal involvement) and/or intrathecal
chemotherapy with methotrexate, cytarabine, and
hydrocortisone, plus supportive care have been used
(Tables 4 and 5).
Table 4: Management of extraocular retinoblastoma
a. Baselins CT scan or MRI; bone marrow and CSF
cytology
b. Intrathecal methotrexate if CSF is positive or there is
radiological evidence of intracranial extension
c. High dose chemotherapy for minimum three cycles and
reassess
d. Enucleation and continue high dose chemotherapy for
6-12 cycles and external beam radiation therapy (see
post-enucleation protocol)
e. If systemic metastasis at the time of presentation,
modify timing of local therapy depending on the extent
of tumor.
Table 5: Post-enucleation adjuvant treatment protocol
a. Systemic standard chemotherapy for 6 cycles for
1. anterior chamber seeding,
2. infiltration of iris,
3. ciliary body infiltration,
4. massive choroidal infiltration
5. optic nerve extension beyond lamina cribrosa.
b. High dose chemotherapy for 6 cycles in patients with
1. combined choroidal infiltration
2. optic nerve extension beyond lamina cribrosa or
3. with scleral infiltration. High dose chemotherapy for
12 cycles is used in patients with extrascleral extension
and optic nerve extension to transection.
c. High dose chemotherapy for 12 cycles in patients with
1. extrascleral extension
2. optic nerve extension to transection.

With emerging dose-intensive chemotherapy
regimens and the use of high-dose chemotherapy
with autologous stem cell rescue, clinical trials are
ongoing to improve the dismal outcome for this
relatively small group of patients. The agents used
in the past included vincristine, cyclophosphamide,
and doxorubicin; although they produce an initial
response, overall survival has been less than optimal.
Carboplatin, ifosfamide, and etoposide have shown
more promise for remission and may be used in
conjunction with high-dose chemotherapy followed
by stem cell rescue. Patients presenting with
extensive non-CNS metastases have been treated
successfully with myeloablative chemotherapy with
stem cell rescue.
Ocular Metastasis
Intraocular metastasis is now considered the most
common malignancy of the eye. The frequency of
ocular metastasis varies significantly among primary
sites. Ocular metastasis, and particularly choroidal
metastasis, can precede the diagnosis of the primary
malignancy. Lung cancer is the most common primary
tumor (35 and 41%) detected in patients with no
neoplasm at the time of ocular diagnosis followed
by breast cancer, leukemia, lymphoma, multiple
myeloma and sarcoma. Rarely metastases from
malignancies of prostate, cervix, thyroid, skin, GI
tract and kidney can occur.
A number of options are available for the therapy
of ocular metastasis, including observation,
chemotherapy, photocoagulation, cryosurgery,
surgical resection, or radiotherapy. The specific
therapy chosen for a patient is an individualized
process that considers the clinical condition of the
patient. The most commonly applied treatment is
external-beam radiotherapy. In general, 30 to 40 Gy
in 10 to 20 fractions could be considered a standard
course of radiotherapy. For patients with a long life
expectancy, a higher total dose with lower dose per
fraction can be considered.
Orbital Tumors
Pediatric primary orbital masses include dermoid
cyst, capillary hemangioma and lymphangioma,
inflammatory lesions, lymphocytic and leukemic
infiltrates, and pilocytic astrocytoma of the optic
nerve, rhabdomyosarcoma and primary neuroectodermal tumor or Ewing's sarcoma. In adults, the
most common localized tumors of the orbit include

cavernous hemangioma, fibrous histiocytoma,
schwannoma, orbital pseudotumor, Grave's ophthalmopathy and hemangiopericytoma. The most
common lacrimal gland tumors include pleomorphic
adenoma and adenoid cystic carcinoma.
Rhabdomyosarcoma
Orbital rhabdomyosarcoma is treated with combination of chemotherapy and radiation therapy after
local excision or biopsy. Chemotherapy is usually
given for 2 to 3 cycles prior to the initiation of
radiation therapy, with the exception of patients with
parameningeal disease and evidence of meningeal
extension in whom radiation therapy generally
begins as soon as possible. Vincristine,
cyclophosphamide, doxorubicin, actinomycin-D,
ifosfamide and etoposide are the drugs used for the
treatment of RMS. Radiation therapy is effective for
achieving local control of tumor for patients with
microscopic or gross residual disease following
biopsy, initial surgical resection, or chemotherapy.
The radiation therapy dose depends predominantly
on the extent of disease following the primary
surgical resection. Patients with completely resected
tumors (group I) of embryonal histology do well
without radiation therapy but radiation therapy
benefits patients with group I tumors with alveolar
or undifferentiated histology.22-24 In general, patients
with microscopic residual disease (group II) receive
radiation therapy to approximately 4,100 cGy. 25
Patients with gross residual disease (group III) should
receive radiation dose of 5,040 cGy. The treated
volume should be determined by the extent of tumor
at diagnosis prior to surgical resection and prior to
chemotherapy. A margin of 2 cm is generally used,
including clinically involved nodes. Precautions
should be taken to limit the dose to the lens, cornea,
lacrimal gland, and optic chiasm.
Orbital Lymphoma
Most of the orbital lymphomas are confined to the
orbit and are of low grade. Patient needs a staging
workup (CBP, chest X-ray, USG abdomen, or CT scan
of chest and abdomen, serum LDH, Bone marrow
biopsy and CSF cytology) to rule out systemic
lymphoma. Radiotherapy is a well-established
treatment modality for orbital lymphoma. Primary
chemotherapy has minimal efficacy in localized low-

grade orbital lymphoma and thus is not advocated
as a first-line treatment. There have been numerous
series advocating low dose radiation for treatment
of orbital lymphomas. In general, radiation dose of
3000 cGy is recommended for low grade lymphomas
and 4000-4500 cGy for intermediate grade
lymphomas.26,27 If it is associated with systemic
disease, it is treated with chemotherapy constituting
cyclophosphamide, doxorubicin, vincristine and
prednisolone for 6 cycles followed by local radiation
therapy.
Idiopathic Orbital Inflammation (IOI)

Idiopathic Orbital Inflammation could be inflammatory, reactive lymphoid hyperplasia or atypical
lymphoid hyperplasia. Corticosteroids have been the
recommended initial drug (prednisone 1 mg/kg/d),
Recently, antimetabolites (azathioprine, methotrexate, and leflunomide), T-cell inhibitors
(cyclosporine and tacrolimus), and alkylating agents
(cyclophosphamide and chlorambucil) were shown
to be useful in the management of NSOI in different
series.28 Use of low-dose external beam radiation
2000 cGy demonstrated a 50 to 80% efficacy in a
previous series.29
Grave's Ophthalmopathy
Severe exophthalmos may occur in some patients
with thyrotoxicosis with involvement of extraocular
muscles. Indications for therapy are corneal exposure
which may cause corneal ulceration that progress to
scarring, optic nerve compression. CT scan shows
thickened extraocular muscles. Steroids and diuretics
are the first-line treatment, administered for 2 weeks.
Radiation dose of 2000 cGy in 10 fractions provides
good symptomatic relief avoiding the need for
further steroid therapy or surgical decompression.30
Optic nerve Meningioma

The diagnosis of optic nerve meningioma is usually
presumptive and based on the appropriate clinical
picture supported by appropriate neuroimaging.
Biopsy is not routinely advocated, as surgical
intervention carries significant morbidity and
mortality. Patients often undergo reimaging at 3
months, and they are followed radiographically at 6
to 12 month intervals after the disease has stabilized.
Treatment strategy should be individualized.
Radiation therapy is recommended as soon as serial

examination documents a new decline in acuity and/
or visual field. Tumor enlargement without loss of
visual function, as determined by serial imaging, may
also provide an indication for radiotherapy.
Recommended dose of radiation therapy is
5400 cGy. 31 It should preferably be delivered via
fractionated, 3-dimensional stereotactic and IMRT
techniques that provide the most precise conformal
application of the dose to affected tissues.
Theoretically, this approach should reduce the risk
of side effects to surrounding radiosensitive ocular
and neural tissues.
Optic Nerve Glioma

Chemotherapy is the first-line treatment, followed
by radiation if chemotherapy fails. Standard
chemotherapy for optic-pathway gliomas consists of
vincristine and carboplatin, whereas second-line
therapy is often thioguanine, procarbazine, and
vincristine. 32 When these fail, chemotherapeutic
agents used in other progressive low-grade gliomas
can be considered. These include cyclophosphamide,
topotecan, and oral VP-16.Stabilization or improvement in visual function and tumor size is considered
a response to treatment. Surgery has a limited role.
Biopsies are performed when clinical and radiologic
features are atypical. Radiation therapy dose above
5000 cGy is required for tumor control. Optic-nerve
gliomas should be operated upon only when grossly
proptotic and the eye is blind or near blind. In large
series from the Mayo Clinic with a median followup time of 10 years, patients with glioma confined to
the optic nerve survived almost twice as long as those
with involvement of optic chiasm.33
Sequelae of Radiation Therapy 34-38

Postradiotherapy complications can be classified as
acute (usually occurring within 3 months of treatment)
or late (occurring many months to years after
completion of treatment). Acute lesions generally
affect rapidly proliferating cells, and most can be
reversed by appropriate medical management. Such
acute effects involving the ocular anterior segment
include blepharitis, conjunctivitis, and keratitis.
However, residual stromal lesions and interstitial
fibrosis may follow. Late effects are primarily caused
by permanent vascular damage and resultant

ischemia. Retinopathy, cataract, and optic neuropathy are examples of such late effects. (Table 6)
Keratoconjunctivitis sicca, another late sequela to
radiotherapy, may often be clinically nonmanifest or
insignificant but can result in ulcerative thinning and
corneal perforation.
Eyelid and periorbital skin radiation effects can
be acutely controlled with topical corticosteroids,
wound debridement, and antibiotic therapy.
Occasionally, reconstructive surgery is indicated to
treat lid deformities. Patients should be encouraged
to wear ultraviolet protective sunscreens and avoid
using harsh soaps and lotions.
Nasolacrimal duct occlusion may require silicone
intubation or dacrycryocysto-rhinostomy, whereas
severe lacrimal punctal stenosis may necessitate a
conjunctivo-dacryocystorhinostomy.
Severe noninfectious inflammation may require
a short course of corticosteroid therapy, but
indiscriminate use of steroids should be avoided
because it can promote extracellular matrix
Table 6: Radiation effects on the eye and orbital tissues
Eye lashes and Eyelid Spared by megavoltage (Cobalt, LA)
Becomes thinner; function not altered
Lash loss at 4060 Gy
Telangiectasia at 55 Gy
Lacrimal system
Dryness in 8-25% pts at 30 to 45 Gy

Dryness in all pts over 4-8 yrs at >55 Gy
Atrophy at 5060 Gy
Stenosis at 6575 Gy
Lens
EBRT
Single dose 2 Gy -Cataract

Fractionated 8 Gy Cataract in 33% of pts
> 11 Gy Cataract in 100% of pts
< 50 Gy Cataract; vision not impaired
> 60 Gy Vision impairing cataracts
Plaque
50 Gy at limbus 33% cataracts
Conjunctiva
Conjunctivitis at 5575 Gy
Telangiectasia at 30 Gy
Cornea
Superficial keratitis- edema, epithelial

defects at 3050Gy
Severe keratitis Ulcer, scarring, perforation
> 60Gy
Retina
40-60 Gy retinopathy 10%

> 60 Gy retinopathy 30%
CRA thrombosis may lead to edema and
pallor of optic disc, retinal hemorrhages,
blindness in 2-3 years
Optic nerve
Neuropathy at >55 Gy
breakdown. Prolonged ocular surface inflammation

or ulceration frequently requires prophylactic
antibiotics. Artificial tears and ointments are
indicated for dry eye relief. It is important to
recognize that the irradiated cornea often has a poor
capacity to heal, despite neovascularization, because
of the degree of epithelial toxicity. Mild punctate
keratopathy needs aggressive lubrication. Tear
replacement therapy with nonpreserved artificial
tears and ointments facilitates epithelial wound
healing.
Infected corneal ulcers require prompt diagnostic
and therapeutic measures, with initiation of broadspectrum antibiotics modified as needed on culture
and susceptibility results. Although hydrophilic soft
contact lenses can be used as protective bandaging
to promote corneal healing, they may not be well
tolerated in severe dry eyes; furthermore, they may
increase the risk of an infection in patients who
are often additionally immunosuppressed by
chemotherapy. Gas-permeable glued-on contact
lenses have been used to treat radiation-induced
keratitis effectively, but experience with this is
limited. If necessary, a conjunctival flap can control
severe pain caused by persistent corneal defects.
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ophthalmology, vol 2. Hagerstown, MD: Haper and Row,
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2. Fitzpatrick PJ, Thompson GA, Easterbrook WM, Gallie BL,
Payne DG. Basal and squamous cell carcinoma of the eyelids
and their treatment by radiotherapy. Int J Radiat Oncol
Biol Phys. 1984;10(4):449-54.
3. Luxenberg MN. Sebaceous gland carcinoma. Arch
4. Tan KC, Lee ST, Cheah ST. Surgical treatment of sebaceous
carcinoma of eyelids with clinico-pathological correlation.
Br J Plast Surg 1991;44:117-21.
5. Pardo FS, Wang CC, Albert D, et al. Sebaceous carcinoma
of the ocular adnexa: radiotherapeutic management. Int J
Radiat Oncol Biol Phys 1989;17:643-7.
6. Meldrum ML, Tse DT, Benedetto P. Neoadjuvant
intracarotid chemotherapy for treatment of advanced
adenocystic carcinoma of the lacrimal gland. Arch
Ophthalmol. 1998;116(3):315-21.
7. Tse DT, Benedetto P, Dubovy S, Schiffman JC, Feuer WJ.
Clinical analysis of the effect of intraarterial cytoreductive
chemotherapy in the treatment of lacrimal gland adenoid
cystic carcinoma. Am J Ophthalmol. 2006.

8. Journe-de Korver JG,Keunen JEE. Thermotherapy in the
management of choroidal melanoma. Prog Retin Eye Res.
2002;21:303- 17.
9. Shields CL, Cater J, Shields JA. Combined plaque
radiotherapy and transpupillary thermotherapy for
choroidal melanoma: tumor control and treatment
complications in 270 consecutive patients. Arch Ophthalmol.
2002;120:933-40.
10. Nath R, Anderson LL, Luxton G, et al. Dosimetry of
interstitial brachytherapy sources: recommendations of the
AAPM Radiation Therapy Committee Task Group No 43.
American Association of Physicists in Medicine. Med Phys.
1995;22:209-34.
11. Jorge E Freire, Luther W. Brady. Jerry A. Shields, Carol L.
Shields, Eye and Orbit, Principles and practice of Radiation
Oncology, (4th ed), 2004;876-96.
12. Shields CL, Santos MC, Diniz W, et al.: Thermotherapy for
retinoblastoma. Arch Ophthalmol 1999;117 (7): 885-93.
13. Friedman DL, Himelstein B, Shields CL, et al.
Chemoreduction and local ophthalmic therapy for
intraocular retinoblastoma. J Clin Oncol 18 (1): 12-7, 2000.
14. Shields CL, Honavar SG, Meadows AT, et al.
Chemoreduction plus focal therapy for retinoblastoma:
factors predictive of need for treatment with external beam
radiotherapy or enucleation. Am J Ophthalmol 2002;133
(5): 657-64.
15. Lumbroso L, Doz F, Urbieta M, et al.: Chemothermotherapy in the management of retinoblastoma.
Ophthalmology 2002;109(6):1130-6.
16. Gombos DS, Kelly A, Coen PG, et al.: Retinoblastoma
treated with primary chemotherapy alone: the significance
of tumour size, location, and age. Br J Ophthalmol 2002;86
(1): 80-3.
17. Abramson DH, Frank CM, Dunkel IJ: A phase I/II study of
subconjunctival carboplatin for intraocular retinoblastoma.
Ophthalmology 1999;106(10):1947-50.
18. Villablanca JG, Jubran R, Murphree AL: Phase I study of
subtenon carboplatin I with systemic high dose
carboplatin/etoposide/vincristine (CEV) for eyes with
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Proceedings of the XIII Biannual Meeting of ISGED and the
X International Symposium on Retinoblastoma, 2001, Fort
Lauderdale, Fla. USA.
19. Krasin MJ, Crawford BT, Zhu Y, et al.: Intensity-modulated
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20. Shields CL, Shields JA, Cater J, et al.: Plaque radiotherapy
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21. Merchant TE, Gould CJ, Wilson MW, et al.: Episcleral plaque
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22. Maurer HM, Beltangady M, Gehan EA, et al.: The Intergroup

Rhabdomyosarcoma Study-I. A final report. Cancer
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23. Maurer HM, Gehan EA, Beltangady M, et al.: The Intergroup
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24. Wolden SL, Anderson JR, Crist WM, et al.: Indications for
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25. Raney R, Hays D, Tefft M, et al.: Rhabdomyosarcoma and
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28. Cockerham KP, Hong SH, Browne EE: Orbital
inflammation. Curr Neurol Neurosci Rep 2003;3:401-9.
29. Smitt MC, Donaldson SS: Radiation therapy for benign
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31. Tsao MN, Hoyt WF, Horton J, et al. Improved visual
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371-81.
36. Merriam GR, Szechtzer A, Focht EF. Theeffects of ionizing
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346-85.
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38. Bhandare N, Monroe AT, Morris CG et al. Does altered
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26
CHAPTER
Carotid-Cavernous Fistulae:
Role of Interventional
Radiologist
D Ravi Varma, D Radhika Varma
The cavernous segment of the carotid artery is unique,

as it is the only anatomical location in the body where
an artery is completely surrounded by a venous
structure. Carotid-cavernous fistulae (CCF) are
spontaneous or acquired communications between
the carotid artery and the cavernous sinus without
an intervening capillary bed. The arterial supply to
these lesions may come from the internal carotid
artery (ICA), from the dural branches of external
carotid artery (ECA), or from both. The cavernous
sinus, being at the crossroads of the cranial and facial
venous circulations, has rich communications with

the facial veins through the superior and inferior
ophthalmic veins, with the transverse and sigmoid
sinuses through the superior and inferior petrosal
sinuses, with the cerebral cortical veins through the
sphenoparietal sinus and with the pterygoid plexus
(Figure 26.1). Thus these lesions can have a wide
range of clinical presentations, the severity of which
is dependent not only on the volume of flow across
the fistula site, but also on the adequacy and
preferred pathway of venous drainage.
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
Common carotid artery,

Internal carotid artery,
External carotid artery,
Dural branches of external carotid artery supplying the
cavernous sinus,
Cavernous segment of internal carotid artery,
Dural branches of internal carotid artery (meningiohypophyseal trunk and inferior cavernous sinus artery),
Cavernous sinus,
Superior petrosal sinus,
Inferior petrosal sinus,
Superior ophthalmic vein,
Inferior ophthalmic vein,
Facial vein,
Pterygoid venous plexus,
Sphenoparietal sinus,
Superior sagittal sinus,
Deep venous system,
Transverse sinus,
Sigmoid sinus,
Internal jugular vein.
Figure 26.1: Arterial and venous anatomy around the cavernous sinus
Carotid-Cavernous Fistulae: Role of Interventional Radiologist 357
(A) Type A CCF: Internal carotid angiogram in lateral projection reveals opacification of cavernous sinus (arrow) and retrograde flow in the
superior ophthalmic vein (double arrow), through a rent in the arterial wall. Note that there is immediate opacification of the entire
cavernous sinus and superior ophthalmic vein during the arterial phase as is common with these high flow lesions
(B) Type B CCF: Internal carotid angiogram in lateral projection reveals opacification of cavernous sinus (double arrow) through small dural
branches (arrow) arising from the cavernous segment of internal carotid artery. In these lesions, there is a significant delay in
opacification of the cavernous sinus and its draining veins suggesting a slow flow across the fistula
(C) Type C CCF: Right external carotid angiogram in frontal projection reveals opacification of the right and left cavernous sinuses (arrows)
through multiple small dural branches of the middle and accessory meningeal arteries (double arrow). Though the CCF was located on
the right side, this middle aged lady was symptomatic in the left eye as the predominant venous drainage was through the left superior
ophthalmic vein.
Type D CCF has arteriovenous fistulae with supply from dural branches of internal and external carotid arteries
Figures 26.2A to C: Barrow classification of CCF
The widely used system to classify CCF is based on

their angioarchitecture. Barrow in 1985 classified
these lesions based on their arterial supply1 (Figures
26.2A to C). Type A CCF are direct fistulae between
the ICA and the cavernous sinus, and are most
commonly secondary to trauma. Type B, type C and
type D CCFs, are low flow indirect fistulae where
multiple microfistulae are located within the wall of
the cavernous sinus and drain into it. The arterial
supply to these lesions may originate from the dural
branches of ICA (Type B), dural branches of ECA
(Type C) or from dural branches of ICA and ECA
(Type D). (The salient features differentiating direct
and indirect CCF are depicted in Table 1).
PATHOPHYSIOLOGY
The elevated pressure in the cavernous sinus leads
to dramatic orbital changes as the superior and
inferior orbital veins are devoid of valves. Though
most obvious manifestations of CCF are reflected in
the eye, we should not forget to look for other less
evident changes which may be vital clues to the
diagnosis, or may result in disaster if overlooked.
Symptoms such as headache may indicate elevation

of cerebral cortical venous pressure and may
result in potentially fatal complications such as
cerebral edema, intracerebral or subarachnoid
hemorrhage. The pathophysiological mechanisms
behind the common clinical symptoms are detailed
in Figure 26.3.
Clinical Features
CCF that have principal drainage into the superior
and inferior ophthalmic veins, present with
predominant orbital symptoms. 2 Though most
patients have orbital symptoms on the same side as
the fistula, we have seen several patients with
unilateral CCF in whom the orbital symptoms were
present on the contralateral side, or even on both
sides, depending on the available paths of venous
drainage (Figure 26.2C). The symptoms and clinical
signs of CCF and their relative incidence are listed
in Table 2.2,5
The clinical signs may be difficult to differentiate
from other sequelae of craniocerebral trauma such
as orbital hematomas and cranial nerve injuries. There
should be a high index of suspicion when a patient
Figures 26.3: Pathophysiology of CCF
presents with orbital symptoms after a head injury.

The low flow at the shunt and nonspecific nature of
symptoms makes indirect CCF even more difficult
to diagnose. A useful clinical test to identify these
lesions is to look for reduction of symptoms

with digital compression of the carotid artery in the
neck.
Table 1: Comparison of direct and indirect CCF

Sex prediliction
Etiology
Pathology
Hemodynamics
Arterial supply and Barrow
Classification Type
Symptoms
Spontaneous closure
Direct
Indirect
Male (Higher incidence of trauma)

Trauma (commonest)
Ruptured cavernous ICA aneurysm
Surgical trauma
Ehlers Danlos syndrome
Fibromuscular dysplasia
Arterial dissection
Idiopathic
Rent in the wall of the cavernous segment
of internal carotid artery
Usually high flow
Cavernous segment of ICA
(Type A)
Commoner in female
Spontaneous (commonest)
Cause unknown
Predisposing factors
Pregnancy
Trauma
Sphenoid sinusitis
Abnormal small dural arterio-venous shunts in the wall of
the cavernous sinus
Usually low flow
Dural branches of ICA (Type B)
Dural branches of ECA (Type C)
Combination of dural branches of ECA and ICA (Type D)
Usually insidious onset, with slow progression
May be abrupt following trauma or may

present after a delay of days or weeks.
Usually progress rapidly.
Extremely rare
May be achieved in 34-60% by conservative management

Table 2: When to suspect CCF
Past History
Craniocerebral trauma
Connective tissue disorders
Symptoms
Swollen red eye
Orbital pain
Diplopia
Progressive vision loss
Headache
Pulsatile tinnitus
Signs
Proptosis
Ptosis
Chemosis
Cranial nerve palsies
Elevated intraocular pressure
Papilledema
Optic nerve atrophy
Rare presentations
Neurological deficits
Intraparenchyma / Subarachnoid bleed
Epistaxis
CASE ILLUSTRATION (Figure 26.4)

Radiological Investigations
In a patient with clinical features of CCF, contrast

enhanced CT scan is most often adequate to confirm
the diagnosis. In addition to the characteristic orbital
and cavernous sinus features (Figures 26.5A to C),
skull base fractures and bony spicules are
demonstrated. Though CT angiography, MRI and
MR angiography (Figures 26.6A to C) elegantly
demonstrate the abnormality, they add little in term
of diagnosis or treatment planning, as the exact site
of fistula and hemodynamics of flow across it are
Figure 26.4: Clinical features of CCF. Mr. P (same patient as in Figure

26.2A) was referred to us with proptosis of left eye that progressed
over 4 months following a road traffic accident. Note the proptosis,
orbital congestion and conjunctival chemosis. He had elevated
intraocular pressure and had lost vision in the left eye. As is common
in most patients with CCF, his presenting complaints were limited to
the orbit. Only on specific queries, he admitted that he often heard a
pulsatile "whooshing" sound in the left ear and that he had severe
headaches that appeared after the head injury
rarely demonstrated. Duplex Doppler studies of the

orbit are useful in follow-up of lesions that are on
conservative management or have undergone partial
occlusion.
Definitive planning of treatment requires digital
subtraction angiography with selective injections of
the internal and external carotid arteries on either
side. Information regarding the arterial supply, site
and size of the fistula, volume of flow across the
fistula, patency of cavernous sinus, pattern of venous
drainage and adequacy of collateral circulation at the
circle of Willis can be obtained on this study.
Figures 26.5A to C: CT scan findings in CCF Plain (A) and contrast enhanced (B) axial CT scans of the orbit in a 24-year-man with posttraumatic direct CCF, reveal proptosis (arrow), enlarged extraocular muscles (arrow heads) and prominent cavernous sinus (double arrow)
on the left side. Dilatation of the superior ophthalmic vein (double arrowhead) should be specifically looked for in such cases (B) as it may be
the only indicator of an underlying vascular abnormality. This characteristic appearance has been termed as the "Hockey stick" sign.
Coronal CT scan (C) in another patient with post-traumatic direct CCF, reveals enlarged extraocular muscles (arrow heads) and dilated
superior ophthalmic vein (arrow) in the right eye. In subtle cases, comparison with the normal structures in the contralateral orbit will help in
identification
Figures 26.6A to C: MR and MR Angiographic features of CCF. 21-year-old man with history of head injury presented with pulsatile proptosis.
Axial T2 (A) and T1 (B) weighted MR scans of the brain reveal dilatation of the superior ophthalmic vein (arrow) and prominence of cavernous
sinus (double arrow) on left side. On MRI, high velocity blood flow produces "flow voids" within blood vessels that usually appear black on all
sequences.
Basal projection of MR Angiogram (C) reveals flow from the left internal carotid artery (arrow) into the left cavernous sinus (double arrow).
The cavernous sinus is seen to drain into inferior petrosal sinuses (arrow head) and superior ophthalmic veins (double arrowhead) on either
side
Management of CCF
Management of CCF must start with treating its
secondary manifestations such as glaucoma.
Aggressive medical management of the elevated
intraocular pressure with adrenergic blockers or
acetazolamide should be started while definitive
treatment should be directed towards closing the
fistula. Surgical measures such as lateral canthotomy
or tarsorrhaphy may be used to decompress the orbit
and to prevent exposure keratopathy.
Direct CCF
High flow lesions such as Type A CCF usually
progress rapidly and may result in vision loss,
ophthalmoplegia, elevated intracranial pressure and
intracranial hemorrhage. Spontaneous thrombosis is
extremely rare and these lesions must be managed
without delay. The indications of emergency
treatment4 of CCF are listed in Table 3.
Surgical ligation of the carotid artery is
ineffective in treating these lesions and may infact
worsen the neurological symptoms, as the fistula
steals blood from the intracranial circulation. On the
other hand, endovascular management seals off the
fistula and preserves the patency of the carotid artery.
Occlusion of the fistula using silicone or latex

detachable balloons is the treatment of choice for
direct CCF3 (Figures 26.7A to F). These balloons are
negotiated through the rent in the wall of the artery,
into the cavernous sinus and are inflated so as to
seal off the fistula. After confirming satisfactory
position of the balloon and verifying the patency of
the carotid artery, the balloon is detached (Figures
26.8A to L). Though these balloons eventually deflate,
they occlude the fistula long enough to cause
thrombosis within the fistula and cavernous sinus.
Transarterial balloon embolization has been reported
to be successful in 80-90% of cases.
Table 3: Indications for emergency management of CCF
Clinical features
Epistaxis
Elevated intracranial pressure
Progressive proptosis
Diminishing visual acuity
Intraocular pressure > 40 mm Hg
Transient ischemic attacks
Imaging features
Presence of cortical venous drainage
Pseudoaneurysm / cavernous sinus varix
Figures 26.7A to F: Technique of detachable balloon embolization of CCF. The delivery catheter (A) is 165 cm long. The stiff proximal shaft is
0.86 mm in diameter while the distal supple part is 0.57 mm in diameter. (B) The valve of the detachable balloon is threaded onto the tip of the
delivery catheter and the assembly (C) is introduced into the carotid artery. Once the balloon crosses the fistula and enters the cavernous
sinus, it is inflated by injecting contrast medium into the hub (D), so that the balloon takes the shape of the cavernous sinus (E) and occludes the
orifice of the fistula. Once satisfactory occlusion of the fistula is achieved, the balloon is detached by gentle traction on the delivery catheter (F)
Figure 26.8A: 29-year-old man who was operated for a traumatic

extradural hematoma, presented 8 months later, with proptosis, diplopia
and right sided headache. He had loud bruit on auscultation over the
right orbit. Note the ptosis, proptosis and lateral deviation of eyeball
on the right side
Figures 26.8B and C: His brain CT (B) revealed a prominent superior ophthalmic vein (double arrowheads) on right side. Diagnostic right
internal carotid angiogram (C) showed opacification of cavernous sinus (bold arrow), with absent flow into distal branches of internal carotid
artery. From the cavernous sinus, blood was seen to flow retrogradely through the superior ophthalmic vein (double arrowheads) into the
orbit, into the inferior petrosal sinus (long arrow) and into cortical veins (double arrows). As he had high flow towards the orbit and significant
flow into the cortical veins, we planned emergency endovascular management. Such large hole direct CCF are best treated with trans-arterial
embolization using detachable balloons
Figures 26.8D and E: We threaded a detachable balloon (9 x 14 mm size) onto a delivery catheter (D)
and introduced the assembly (arrow) through a guiding catheter (arrow heads) into the internal carotid artery (E)
Figures 28.8F to H: We negotiated the balloon across the orifice of the fistula and placed it in the cavernous sinus (arrow head). We slowly
inflated the balloon with contrast medium, with frequent check angiograms (F). On complete inflation of the balloon, though we could achieve
cessation of flow in the cortical veins, flow persisted in the superior ophthalmic vein as seen on check angiogram (G) and follow-up Doppler
study (H)
Figures 26.8I and J: We negotiated another detachable balloon (arrow head) through the fistula into the anterior part of the cavernous sinus
(I) and inflated it so that flow in the superior ophthalmic vein was arrested (J). The ophthalmic artery (arrow) and intracranial branches of
internal carotid artery (arrow heads) are seen to fill now
Figures 26.8K and L: In the weeks following the embolization we confirmed the absence of flow in the superior ophthalmic vein on followup Doppler studies of the orbit (K). Though Doppler gives us information only about the orbital component of CCF, it is an inexpensive and reliable
modality to confirm the efficacy of treatment. Plain radiograph of the skull performed a month after treatment (L) showing the two balloons
within the cavernous sinus
Indirect CCF
multiplicity of the microfistulae and their propensity

to parasitize additional arterial supply after
embolization, these lesions are relatively difficult to
eliminate. Cure rates of 60-80% have been reported
in literature.
Occlusion of the cavernous sinus using detachable
coils is another treatment option in cases where the
above techniques fail. Extremely soft platinum coils
are introduced into the cavernous sinus through
arterial or venous routes, so as to completely occlude
its lumen. Complications such as transient cranial
nerve palsies usually resolve over a few months.
Permanent complications occur in less than 5% of cases.
In contrast, indirect CCF are low flow lesions and

usually progress slowly. These lesions must be
carefully followed up with periodic clinical
examination, measurement of intraocular pressure
and angiographic studies as required. Special
attention should be paid to changes in the
angioarchitecture and quantum of flow on
angiography. Patients with visual deterioration,
elevated intraocular pressure, obtrusive diplopia,
intolerable bruit or headache and malignant proptosis
with exposure keratopathy require definitive
management. Table 1 summarizes and compares
direct and indirect CCF.
Prognosis
Indirect CCF have multiple tiny arterovenous

shunts in the wall of the cavernous sinus. These are
usually embolized using polyvinyl alcohol particles,
which not only mechanically occlude the lumen of
the fistulae, but also incite an inflammatory reaction
in the vessel wall (Figures 26.9A to F). Owing to the
With advances in imaging techniques and development of newer interventional techniques, consistently
good results are being achieved in the management
of CCFs. The key to effective management of these
lesions however, is early diagnosis by maintaining a
high index of suspicion.
Figures 26.9A to C: Diagnostic left external carotid angiogram in frontal (A) and lateral (B) projections, reveal multiple arteriovenous fistulae
(arrows) along the wall of the left cavernous sinus. Selective angiogram of the feeders (C) shows the fistulae and opacification of the
cavernous sinus (arrow) as well as superior ophthalmic vein (arrow heads)
Figures 26.9D and E: The feeders were embolized using polyvinyl alcohol particles (D) and (E)
Figure 26.9F: Postembolization check angiogram shows absence of opacification of the fistulae and cavernous sinus
REFERENCES
1. Barrow D, Spector R, Braun I, et al. Classification and
treatment of spontaneous carotidcavernous sinus fistulas.
J Neurosurgery 1985; 62: 248-56.
2. Debrun GB, Lacour P, Fox AJ, et al. Traumatic
carotid-cavernous fistulas: etiology, clinical presentation,
diagnosis, treatment, results. Semin Intervent Radiol 1987;
4:242-8.
3. Higashida RT, Halbach VV, Tsai FY, et al. Interventional

neurovascular treatment of traumatic carotid and
vertebral artery lesions. Results in 234 cases. AJR 1986; 153:
577-82.
4. Halbach VV, Hieshima GB, Higashida RT, et al. Carotidcavernous fistulae; Indications for urgent treatment. AJR
1987; 149: 587-93.
5. de Keizer R. Carotid-cavernous and orbital arteriovenous
fistulas: ocular features, diagnostic and hemodynamic
considerations in relation to visual impairment and
morbidity. Orbit 2003; 22: 121-42.
27
CHAPTER
Ocular and Systemic

Associations of Proptosis
Some orbital lesions are known to have other ocular

features and also associated with other lesions
elsewhere in the body. It is always essential to have
a knowledge of these conditions, so that we can
investigate accordingly and detect the disease in a
very early stage and sometimes in asymptomatic
stage,and help the patient. If needed we should
refer them to the respective specialists to prevent
complications and ensure overall care of the
patient.
In this chapter an attempt is made to list ocular
and systemic associations of common orbital
disorders presenting with proptosis. Some others like
leukemia, lymphomas are dealt with separately
elsewhere in this book.
Capillary Hemangiomas
(a) Ocular features: Strawberry nevus of the lids,
proptosis, ptosis, astigmatism, amblyopia, optic
atrophy and exposure keratitis.
(b) Systemic associations:
Kasabach-Merritt syndrome (multiple
capillary hemangiomas + thrombocytopenia)
Mafucci syndrome (multiple capillary
hemangiomas + multiple enchondromatosis)
Phaces syndrome (posterior fossa
malformations, hemangiomas, arterial
anomalies, coarctation of the aorta, eye
abnormalities and sternum abnormalities).1,2
Neurofibromatosis
(a) Ocular features: Eyelid neurofibromas,
prominent corneal nerves, glaucoma, Lisch
nodules, congenital ectropion uveae, iris
mamillations, choroidal naevi, retinal
astrocytomas, optic nerve gliomas, optic nerve
meningiomas and spheno-orbital encephalocele
with pulsating proptosis.
(b) Systemic associations: Chiasmal tumors,
hypopituitarism, spinal and gastrointestinal
neurofibromas, pheochromocytoma, juvenile
xanthogranuloma, capillary hemangioma, Wilms
tumor, rhabdomyosarcoma, scoliosis, macrocephaly, aqueductal stenosis and seizures.3,4
Diagnostic Criteria for NF-1
NF 1 is diagnosed if 2 or more of the following group
of seven conditions are met
Six or more caif-au-lait spots > 5 mm in
diameter in prepubescents or > 15 mm in postpubescents
Two or more neurofibromas or one plexiform
neurofibroma
Axillary or inguinal freckling
Optic N glioma
Two or more Lischs nodules
Sphenoid bone dysplasia or thinning of long bone
cortex, with or without pseudoarthrosis
First degree relative with NF-1.
[Source: NIH Consensus development conference. Arch
Neurol. 1988;45:575-580]
Ocular and Systemic Associations of Proptosis 367
Neurofibroma type 2
Features
Meningioma, glioma, schwannoma, vestibular
schwannoma, posterior subcapsular lenticular
opacities, hamartoma of retina and retinal pigment
epithelium.
NF2: Criteria for Diagnosis
Presence of any one of the following features:
Bilateral vestibular schwannoma
First degree relative with NF2 plus unilateral
vestibular schwannoma < 30 years.
First degree relative with NF2 plus any 2 of the
following:
Meningioma
Glioma
Schwannoma
Juvenile posterior subcapsular
lenticular opacities
Juvenile cortical cataract.
[Source: National Institute of Health Consensus
Development Conference Neurofibromatosis: Conference
Statement. Arch Neurol 1988,45:,575-78.]
Diagnostic evaluation for NF2: MRI for
neurological and neuro-otologic evaluation is
mandatory, since nearly 90% of NF2 exhibit bilateral
vestibular schwannomas.10
Thyroid Orbitopathy (Graves disease)

( a ) Ocular features: Lid retraction, chemosis,
proptosis, superior limbic Keratoconjunctivitis,
keratoconjunctivitis sicca, diplopia, optic
neuropathy and choroidal folds.
( b ) Systemic associations: Thyroid acropachy,
plummer nails, tremors, fatigue, tachycardia,
atrial fibrillation, pretibial myxoedema, alopecia,
vitiligo, high output failure and myasthenia
gravis.
Craniofacial Dysostosis
Crouzon Syndrome
( a ) Ocular features: Proptosis, hypertelorism,
V pattern exotropia, hypertropia, optic atrophy,
exposure keratitis, megalocornea, glaucoma,
colobomas, aniridia and blue sclera.
(b) Systemic associations: Wide cranium, midfacial
hypoplasia, parrot-beak nose, frog facies,
mandibular prognathism and acanthosis

nigricans.5,6
Apert Syndrome
(a) Ocular features: Shallow orbits, proptosis,
hypertelorism, exotropia, antimongoloid slant,
exposure keratitis, optic atrophy, keratoconus,
congenital glaucoma and ectopia lentis.
( b ) Systemic associations: Oxycephaly, midfacial
hypoplasia, low set ears, high arched palate, cleft
palate, syndactyly, mental handicap and
anomalies of the heart, lungs and kidney.5,6
Encephalocele
(a) Ocular features: Pulsatile proptosis, dystopia,
medial canthal swelling, microphthalmos,
colobomas and morning glory syndrome.
(b) Systemic associations: Neurofibromatosis,
hypertelorism, broad nasal bridge and cleft
palate.7
Wegeners Granulomatosis
(a) Ocular features: Nasolacrimal duct obstruction,
dacryocystitis, scleritis, peripheral ulcerative
keratitis, nonspecific orbital inflammatory
disease and occlusive retinal periarteritis.
(b) Systemic associations: Necrotizing granulomas
of upper respiratory tract, necrotizing glomerulonephritis, perforation of nasal septum, saddle
shaped nasal deformity, nasal-paranasal fistulae,
vasulitis of the spleen and adrenals, polyneuritis
and meningioencephalitis.7
Wyburn-Mason Syndrome
(a) Ocular features: Arterio-venous malformations
of the conjunctiva, lids and retina, orbital A-V
malformations causing proptosis and bruit,
vitreous hemorrhage and neovascular glaucoma.
(b) Systemic associations: A-V malformations of the
CNS causing headaches and seizures, cranial
nerve palsies, motor and sensory deficits.7
Langerhans Cell Histiocytosis

(a) Ocular features: Proptosis, ptosis, periorbital
swelling, localized pain.
(b) Systemic associations: Osteolytic lesions of
skull, ribs and long bones, diabetic insipidus and
soft tissue lesions of liver and spleen.8
Hurlers Syndrome
Hemangioblastoma
(a) Ocular
features:
Corneal
clouding,
papilloedema, shallow orbits causing proptosis.
( b ) Systemic associations: Stiff joints, coarse face,
chest deformities, dwarfism, hepatosplenomegaly and deafness.9
Von Hippel- Lindau's disease
Nonspecific Orbital Inflammation Syndrome
Crohn's disease,
Systemic lupus erythematosus
Rheumatoid arthritis
Myasthenia gravis
Ankylosing spondylitis.
Sclerosing inflammation of the orbit
Riedel's thyroiditis
Mediastinal fibrosis
Sclerosing cholangitis
Fibrosis of parotid gland, lacrimal gland and
lung.
Osteoma
Gardner's syndrome (Familial polyposis of
large bowel, plus osteoma of skull or jaw plus
epidermal and subcutaneous cysts)
Turcot Syndrome (Familial adenomatous
polyposis plus CNS gliomas).
Fibrous dysplasia
McCune-Albright syndrome (ployostotic fibrous
dysplasia + sexual precocity + cutaneous pigmentation).
Orbital Hamartoma (tuberous sclerosis)

Ocular : Retinal hamartoma
Visceral : Pulmonary lymphangiomatosis
Renal angiolipoma
Cardiac rhabdomyoma
Skin
: Facial angiofibroma
CNS
: Intracranial astrocytomas, ependymoma
Retinal hemangioblastoma
Cerebellar and spinal hemangioblastoma
Renal cell carcinoma
Pheochromocytoma
Others: Pancreatic tumors, cystadenoma of
epididymis.
REFERENCES
1. Haik BG, Karcioglu ZA, Gordon RA, Pechous BP. Capillary
hemangioma. Surv Ophthalmol. 1994;38:399-426.
2. Kushner BJ. Hemangiomas. Arch Ophthalmol. 2000;
118:835-36.
3. Beauchamp GR. Neurofibromatosis type 1 in children.
Trans Am Ophthalmol soc. 1995;93:445-72.
4. Listernick R, Charrow J, Greenwald MJ, et al. Natural history
of optic pathway tumors in children with neurofibromatosis type 1. J Pediatr.1994;125:63-66.
5. Cohen MM. The child with multiple birth defects. 2nd
ed.New york:Oxford 1997:178-96.
6. Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head
and Neck. 3rd ed. Newyork Oxford; 1990.
7. Kanski JJ. Clinical Ophthalmology:A systematic approach.
6th ed. Butterworth - Heinemann. 2007.
8. Huang F, Arceci R. The histiocytoses of infancy. Semin
Perinatol 1999;23:319-31.
9. AAO. Pediatric Ophthalmology and strabismus. Section 6.
Ocular findings in inborn errors of metabolism. AAO
publication 2006.
10. Arun D Singh, Bertil F Damato,, Jacob Pe'er, A. Linn
Murphee, Julian Perry: Clinical Ophthalmic oncology, first
edition, 2007 Saunders Elsevier,Philadelphia.
Index
A
3-D reconstruction of orbit 84
Adenoid cystic carcinoma 194
clinical features 194
imaging 195
management 196
pathology and pathogenesis 195
basaloid variant 195
comedocarcinoma variant 195
cribriform (glandular or swisscheese) pattern 195
sclerosing variant 195
tubular (ductal) variant 195
prognosis 196
Angiosarcoma 76
Applied anatomy of orbit 3
cavernous sinus 8
extraocular muscles 9
globe 8
lacrimal system 13
lids 10
nerves of the orbit 14
optic nerve 14
parasympathetic innervation of the
orbit 16
sensory innervation of the orbit 14
sympathetic innervation of the
orbit 16
orbital apex 6
orbital osteology 3
periorbita 6
Auscultation 51
B
Basal cell and squamous cell carcinoma
348
B-cell lymphoma 174
Benign tumors of orbit 103
capillary hemangioma 103
cavernous hemangioma 103

hemangiopericytoma 103
lymphangioma 103
meningiomas 104
Bone tumors of orbit 180
case-illustration 181
aneurysmal bone cyst 184
cholesterol granuloma 184
chondroma 183
chondrosarcoma 186
Ewing's sarcoma 186
fibrous dysplasia 181
giant cell lesions 184
Langerhans cell histiocytosis (lCH)
186
mesenchymal chondrosarcoma 186
myeloma 186
ossifying fibroma 182
osteoblastoma 182
osteogenic sarcoma 184
clinical presentation 162
clinico-pathological classification of
primary orbital bone disorders 180
osteoma 180
Bony lesions 62
Bony orbit 60
C
Capillary hemangioma 348
Carotid-cavernous fistula 39
Carotid-cavernous fistulae 356
direct CCF 360
indirect CCF 363
management of CCF 360
pathophysiology 357
prognosis 363
radiological investigations 359
Cell cycle and the principles of antineoplastic therapy 347
Cephalocele 201
radiological finding 202

treatment 202
Choroidal melanomas 349
Classification of orbital tumors 102
primary 102
hemopoietic 102
lacrimal gland 102
mesenchymal 102
miscellaneous 102
neural 102
secondary 102
direct extension 102
Cystic lesions of the orbit 78
Cysts of the optic nerve sheath 203
Cytology smear 91
D
Dacryocele 205
Decision making 271
apical conal lesions 279
intraconal lesion 273
lesions of superior peripheral space 279
thyroid associated orbitopathy 285
Dermoid and epidermoid cysts 200
investigations 200
MRI 200
treatment 201
Developmental lesions of orbit 98
dermoid and epidermoids 99
hamartoma 99
meningioencephalocoeles 98
neurofibromatosis 98
sphenoid wing dysplasia 98
Diagnosis of orbital tumors 98
oculomotor paresis 98
optic neuropathy 98
pain 98
papillary abnormalities 98
proptosis 98
Duplex Doppler 56
E
Enlarged extraocular muscle 69
ENT approach to proptosis 300
etiological factors 300
clinical manifestation and evaluation
300
diseases of the lacrimal apparatus
300
infection and inflammation 300
tumors of the orbito-sinual disease
300
Epithelial cyst (dacryops) 190
management 191
prognosis 191
Etiology of proptosis 53
Evaluation of a case of proptosis 53
External radiotherapy in ocular tumors
346
F
Functional endoscopic sinus surgery
(FESS) 309
G
Graves ophthalmopathy 353
H
Hand-Schuller-Christian syndrome 133
Hematic cyst 203
investigations 203
treatment 203
Hertel's exophthalmometer 35
Hertel's exophthalmometry 37
Hutchinson's sign 15
Hydatid cyst of orbit 217
investigations 217
management 217
I
Idiopathic orbital inflammation (IOI) 353
Inflammatory lesions of orbit 100
orbital cellulitis 100
idiopathic orbital inflammation 100
orbital infections 101
aspergillosis 101
cysticercosis 102
neoplastic lesions 102
tuberculosis 101
Internal radiation therapy (brachytherapy) 345

Intraocular lymphoma 349
Intraocular tumors 349
K
Kilppel-Trenaunary syndrome 153
Kimura's disease 132
L
Lacrimal gland tumors 76
Langerhans histiocytosis 133
Letterer-Siwe disease 133
Leudde's exophthalmometry 35
Lid retraction 46
Lymphoma of lacrimal gland 77
Lynch-Howarth's operation 304
M
Malignant conjunctival tumors 349
Malignant tumors of orbit 104
adenoid cystic carcinoma 105
Graves disease 106
histiocytoma 105
lymphoma 105
metastasis 105
optic glioma 106
rhabdomyosarcoma 104
Management of ophthalmic tumors 347
Medical management of proptosis 337
nonspecific inflammations of the orbit
(NSOIS) 337
chronic granulomatous infections
338
nonspecific lacrimal inflammation
337
nonspecific myositic inflammation
337
orbital cellulitis 338
parasitic infestations 338
rhino-orbital mucormycosis 338
specific inflammations of the orbit
338
structural lesions 340
Tolosa-Hunt syndrome 339
vascular lesions 339
vasculitis 339
Meningioma of optic nerve sheath 74
Mesenchymal tumors 170
histiocytic tumors 175
fibrous histiocytoma 175
malignant tumors of uncertain type
175
rhabdoid tumor 175
mesenchymal soft tissue tumors 170

striated muscle tumors 170
rhabdomyoma 172
Microphthalmos with cyst 202
Mucocele 202
Mller's muscle 46
N
Neurofibroma 75
Neurosurgical approach to proptosis 309
Non-Hodgkin's lymphoma 147
Nonspecific orbital inflammatory
syndrome (NSOIS) 131
O
Ocular and systemic associations of
proptosis 366
capillary hemangiomas 366
systemic associations 366
ocular features 366
craniofacial dysostosis 367
apert syndrome 367
crouzon syndrome 367
encephalocele 367
hemangioblastoma 368
Hurler's syndrome 368
Langerhans cell histiocytosis 367
nonspecific orbital inflammation
syndrome 368
orbital hamartoma (tuberous
sclerosis) 368
osteoma 368
sclerosing inflammation of the orbit
368
Wegener's granulomatosis 367
Wyburn-Mason syndrome 367
diagnostic criteria for NF-1 366
neurofibroma type 2 367
criteria for diagnosis 367
diagnostic evaluation 367
features 367
neurofibromatosis 366
ocular features 366
systemic associations 366
thyroid orbitopathy 367
Ocular metastasis 352
Optic nerve glioma 353
Optic nerve meningioma 353
Orbital amyloidosis 129
Orbital diseases 97
classification 97
developmental 97
endocrine 97
Index 371
inflammatory 97
miscellaneous 97
neoplastic 97
traumatic 97
vascular 97
Orbital exenteration 318
complications of exenteration 320
indications 318
management of the exenterated socket
320
myocutaneous flaps 320
patient preparation 318
prosthesis 320
skin flaps 320
skin grafting 320
spontaneous granulation 320
surgical procedure 318
types 319
anterior exenteration 319
lid sparing exenteration 319
radical exenteration 319
total exenteration 319
Orbital fractures 220
anatomy 220
examination 222
floor fractures 233
general operative considerations 229
antibiotics 230
decision repair or not repair 232
timing of surgery 231
imaging 226
implant materials 227
late and secondary fracture repair 239
lateral wall and zygomatico-maxillary
fractures 238
Orbital infections 120
demographic profile 120
diagnosis 121
emergency department care 122
etiological causes 121
bacterial infections 121
fungal infections 121
parasitic infections 121
protozoal infections 121
imaging studies 121
risk factors 121
Orbital lymphoma 146
modified Rye's classification of
Hodgkin's lymphoma 147
classic HD 147
nodular lymphocyte-predominant
HD 147
revised European American
lymphoma classification (REAL
classification) 146
leukemias and lymphomas of T-cell
origin 146
WHO classification of NHL 146

B-cell neoplasms 146
T-cell neoplasms 146
Orbital lymphoma 352
Orbital prosthesis 327
assemble the prosthesis 331
casting 329
fabrications of ocular prosthesis 331
impression of the orbital defect 328
moulding 330
preparation of the patient 328
sculpting 329
types 327
adhesive retained prosthesis 327
magnetic retained prosthesis 327
partial prosthesis 327
spectacle mounted prosthesis 327
using the desired material 331
Orbital tumors 352
Orbital tumors of neurological origin 162
optic nerve glioma 162
optic nerve meningioma 163
orbital schwannoma (neurilemmoma)
and neurofibroma 165
Orbital xanthogranuloma 134
Orbitotomies 288
approaches 289
general principles 288
lateral orbitotomy 290
Stallard-Wright lateral orbitotomy 290
swinging lower lid flap 289
transcarcuncular approach 291
transfrontal orbitotomy 291
complications 291
postoperative management 291
transnasal endoscopic approach and
transantral approach 291
P
Parasitic cysts of orbit 207
investigations 208
treatment 208
Patterson's operation 305
Perception of color vision 41
Peripheral surgical space 91
Plaque radiotherapy 346
Pleomorphic adenoma 191
imaging 192
management 192
prognosis 192
Pleomorphic adenoma 76
Proptosis 28
axial proptosis 29
down and in displacement of the globe

32
down and out proptosis 29
measurement of proptosis 35
upward displacement of globe 35
Pulsations of globe 37
R
Radiation therapy delivery methods 344
Reese-Berke's incision 273
Retinoblastoma 349
treatment 349
chemoreduction 350
cryotherapy 349
photocoagulation 349
subtenon (subconjunctival)
chemotherapy 350
surgery (enucleation) 350
Rhabdomyosarcoma 77, 352
Role of cytology in orbital lesions 85
fine needle aspiration/sampling
technique 85
intraoperative-operative diagnosis by
squash and imprint cytology 85
squash or imprint cytology 85
Rosai Dorfman disease 133
S
Sarcoidosis 130
Sebaceous carcinoma 348
Secondary and metastatic orbital tumors
244
malignant melanoma of eyelid 256
metastatic orbital tumors 263
orbital extension of conjunctival
tumors 258
malignant melanoma of the
conjunctiva 259
squamous cell carcinoma of the
conjunctiva 258
orbital extension of eyelid tumors 252
orbital extension of intracranial tumors
257
orbital extension of intraocular tumors
244
orbital extension of lacrimal sac tumors
250
orbital extension of medulloepithelioma 246
orbital extension of nasopharyngeal
tumors 262
orbital extension of retinoblastoma
244
orbital extension of tumors of the nasal
cavity and paranasal sinus 260

orbital extension of uveal melanoma
247
sebaceous carcinoma of the eyelid 253
squamous cell carcinoma of the eyelid
255
Sequelae of radiation therapy 353
Sinus diseases causing proptosis 301
allergic fungal sinusitis 301
extensive nasal polyposis 301
frontoethmoidal mucoceles 302
mucormycosis 301
purulent infections 301
treatment 301
Soft-tissue lesions 72
Sphenoid wing meningioma 45
Steps of Reese-Berke's approach
Steps of superior lid crease incision 275
Sturge-Weber syndrome 152
Subconjunctival hemorrhage 51
T
Teratomas 201
Thyroid-associated orbitopathy 111
course of disease 114
incidence and epidemiology 112
management guidelines 118
pathogenesis 112
risk factors 112
visa classification 114
appearance/exposure 117
application of the visa classification
118
inflammation/congestion 116
strabismus/motility restriction 117
vision/optic neuropathy 114
Transcranial approach with resection of
the orbital roof 311
Transpalatal approach to remove
postnasal tumors 307
Trauma 65
Tumors of lacrimal gland 348
Tumors of paranasal sinuses causing
proptosis 302
juvenile nasopharyngeal angiofibroma 303
malignant tumors 303
adenoid cystic carcinoma 303
esthesioneuroblastoma 304
non-Hodgkin's lymphoma 303
squamous cell carcinoma 303
various approaches for tumor
removal 304
Caldwell-Luc operation 304
external ethmoidectomy 304
intranasal ethmoidectomy 304
Jansen-Horgan operation 304
lateral rhinotomy/medial maxillec
tomy 305
total maxillectomy 306
Tumors of the eyelid 347
V
Val salva 51
Vascular anatomy of the orbit 17
arterial supply 17
outflow 19
paranasal sinuses 20
venous 19
Vascular lesions of orbit 151
malformations 151
cavernous hemangioma 152
lymphangioma 151
orbital varices 152
other congenital malformations 152
shunts 153
angiosarcoma 155
capillary hemangioma 154
carotid-cavernous fistula 153
hemangioblastoma 155
hemangioendothelioma 155
Kaposi's sarcoma 155
new growths 154
Vascular pulsations 39
Von Recklinghausen disease 75
W
Wegener's granulomatosis 132
Whitall's tubercle 12
Wyburn-Mason syndrome 153

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Subrahmanyam Mallajosyula MS, DO

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

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This book is dedicated

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Golam Haider MS, FCPS

Christopher M Knapp BSc (Hons), FRC Ophth

Jack Rootman MD, FRCSC

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viii Surgical Atlas of Orbital Diseases

Ramesh Murthy MD, FRCS

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basmala blog (always original)

Applied Anatomy of Orbit ................................................................................................................. 03

Clinical Approach to Proptosis ......................................................................................................... 23

Imaging a Case of Proptosis: CT and MRI .................................................................................... 56

Role of Cytology in Orbital Lesions ................................................................................................ 85

Pathology of the Orbital Diseases ................................................................................................... 97

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xvi Surgical Atlas of Orbital Diseases

Part Two : Disease Patterns of Proptosis

Thyroid-Associated Orbitopathy .................................................................................................... 111

Orbital Infections ............................................................................................................................... 120

Orbital Inflammatory Disease ........................................................................................................ 128

Orbital Lymphoma ............................................................................................................................. 146

Vascular Lesions of Orbit ................................................................................................................ 151

Orbital Tumors of Neurological Origin ........................................................................................ 162

Mesenchymal Tumors ....................................................................................................................... 170

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Bone Tumors of Orbit ....................................................................................................................... 180

Tumors of Lacrimal Gland ............................................................................................................... 190

Cystic lesions of Orbit ...................................................................................................................... 199

Parasitic Cysts of Orbit ..................................................................................................................... 207

Orbital Fractures ................................................................................................................................ 220

Secondary and Metastatic Orbital Tumors ................................................................................. 244

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xviii Surgical Atlas of Orbital Diseases

Part Three : Management Strategies: Surgical

Decision Making ................................................................................................................................ 271

Orbitotomies ........................................................................................................................................ 288

Multidisciplinary Approach to Proptosis .................................................................................... 299

Orbital Exenteration .......................................................................................................................... 318

Orbital Prosthesis............................................................................................................................... 327

Part Four : Management Strategies: Nonsurgical

Medical Management of Proptosis ................................................................................................ 337

basmala blog (always original)

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy .. 344

Carotid-Cavernous Fistulae: Role of Interventional Radiologist .......................................... 356

Ocular and Systemic Associations of Proptosis ......................................................................... 366

Index ..................................................................................................................................................... 369

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Applied Anatomy of Orbit

Fundamental to the understanding of orbital