The Orbit

Thorsang R1
Neuroradiology topic
04.06.2014
 Outline

Orbit overview

Imaging techniques

Orbital anatomy

Diseases of the orbit

Major anatomic components
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
Differential diagnoses
 Imaging techniques
MRI CT
Initial imaging sequence of An adjunctive tool:
choice for the orbit and CN calcifications
II Small meningioma
Intraaxial, sellar, parasellar Retinoblastoma with
lesions calcification
More sensitive
Imaging techniques
Orbital anatomy
Roof
Floor
Medial wall
Lateral wall
Superior orbital
fissure
Inferior orbital
fissure
Optic canal
Orbital roof
Frontal bone
with frontal
sinus
Anteriorly thin
Fossa for
lacrimal gland
Orbital floor
Orbital plate of
maxilla
Thin
-> blowout fx*
Orbital process
of palatine bone
Orbital surface
of zygomatic
bone
Medial orbital wall
Lacrimal groove
Lamina
papyracea
Medial blowout
fracture*
Spontaneous
dehiscence of
orbital fat
Into ethmoid
sinus
Sphenoid bone
Lateral orbital wall
Orbital surface
of zygomatic
bone
Superior orbital fissure
Location
Between roof
and lateral wall
Surrounded by
sphenoid
Beneath optic
canal
Optic strut
Superior orbital fissure
Content
Superior
opthalmic vein
CN III, IV, VI
CN V1

>> Conduit
between orbital
apex-cavernous
sinus
Inferior orbital fissure
Location
Between floor
and lateral wall
PPF fossa-NSP
masticator space
Inferior orbital fissure
Content
CN V2, V3
N.
from PP gg.
V.
inf. Opthalmic v.-
pterygoid plexus

>> Deep face

inflammatory and
neoplastic lesions
 Optic canal

Lesser wing of
sphenoid bone
Optic nerve +
ophthalmic artery
(in dural shealth)
From suprasellar
cistern
Conal-intraconal area
 Intraconal space pathology
Capillary hemangioma Optic neuritis
Venous vascular
Venous malformation
dilatation Optic
MS
nerve lesions
Carotid Cavernous Fistula Devic's syndrome
Varices Neuritis due to infection
(e.g.herpes zoster)
Autoimmune (e.g.Lupus)
Drugs (e.g.chloramphenicol,
ethambutol)
Optic nerve glioma
Optic nerve meningioma
Pseudotumor
Other cranialnerves
Schwannoma of 3rd, 4th and
6th cranial nerve
 Conal space pathology
Thyroid eye disease
usually enlargement of the inferior and medial rectus
Pseudotumor:
idiopathic orbital inflammation
Adjacent inflammation:
sinusitis
Uncommon causes of enlargement of the extra-
ocular muscles
glycogen storage disease and lymphoma
Extraconal area
Fat
Lacrimal gland
Lens-shape
Anterior,
superolateral
Lies on levator
palpabrae and
lateral rectus
Bone
 Extraconal pathology
Abscess due to sinusitis
Schwannoma of the V1 and V2
Bone lesions:
Fibrous dysplasia of the sphenoid wing
Metastases
Multiple myeloma
Diseases of the orbital appendages
 Orbital appendages
Lacrimal gland
superolaterally in the orbit
granulomatous, epithelial/glandular, and
developmental
lacrimal sac
lacrimal duct
-> inferior terbinate into the nose
Diseases of the orbit
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
 The globe

The ocular
space
Rupture of the globe
Anterior
chamber:
Hemorrhage: also known as anterior hyphema
cornea

Cataract anteriorly and the lens and iris posteriorly

Keratitis: inflammation of the cornea

Periorbital cellulitis
Posterior chamber:
posterior to the iris
Glaucoma
Uveitis
Ciliary melanoma
 The globe
Vitreous body
posterior to the lens
Rupture
Hemorrhage
CMV infection: especially in HIV
Persistent Hyperplastic Primary Vitreous (PHPV)
 Persistent hyperplastic primary
vitreous

Unilateral leukokoria in male infants

Persistent hyaloid canal

Persistence of the primary vascular vitreous

connective tissue
Hyperplasia of the residual
PHPV is the second embryonic
most common cause of
leukocoria
Also develops glaucoma and cataract
 Persistent hyperplastic primary
vitreous
Findings:
Microphtalmic globe with enhancing increased
density in the vitreous humor.
Unilateral/bilateral tissue density band from back
of the lens to the posterior inner globe (Persistent
Cloquets canal)
Retinal detachment (occurs in 30-55%)
Persistent hyperplastic primary
vitreous
The globe
 Retina
Retinoblastoma:
a common tumor in children
Hemangioblastoma:
most common retinal tumor in the adult
von Hippel Lindau disease
Detachment:
most common retinal lesion
diabetic retinopathy
 Retinoblastoma
Common tumor in the first year of life
Child < 3 years of age (98%)
Other presentations: leukokoria, strabismus,
decreased visual acuity, family hx, eye pain,
proptosis
Believed to arise from neuroectodermal cells
 Retinoblastoma
75% unilateral; 25% bilateral
All bilateral cases are hereditary (AD) >>
deficient tumor suppression gene on
chromosome 13
90% calcified
 Retinoblastoma
The other tumors in this age group are
Neuroblastoma
Wilm's tumor
Leukemia
Teratoma.
 Retinoblastoma
Techniques
CT is prefered to detect calcification
MR serves an adjunctive role
Findings:
CT: an intraocular mass with calcification
in a child<3 yr -> should consider retinoblastoma
Extraocular spreading along the optic nerve
Together with pinealoma
-> trilateral retinoblastoma
Retinoblastoma
 DDx in a child with leukokoria

Toxocara
endophthalmitis
Persistent
hyperplastic vitreous
Retinopathy
of prematurity
Coats disease
 Calcifications
In adults
most common at the annulus of Zinn
at the optic nerve head within the eye -> optic
disc drusen
(asymptomatic, but pseudo-papilledema from PE)

In children
retinoblastoma until proven otherwise
 Coats disease
A rare eye disorder: congenital non-hereditary
vascular malformation of the retina
multiple telangiectatic vessels -> exudate leakage ->
retinal detachment
A 6-to-8-year-old boy with unilateral leukokoria
Symptoms occurs when the retina detaches ->
central visual loss
Findings:
Increased density of the vitreous
Normal globe size, lack of calcification
Coats disease
 Choroid
Melanoma:
choroid contains the melanin cells
Metastases:
choroid is the most vascular structure in the eye
Detachment:
usually post-traumatic
 Uveal melanoma
The most common primary intraocular
malignancy in adult
A 50-to-70-year old with unilateral ocular
complaint
85% from choroid, 9% from ciliary body, and
6% from iris
Dx from PE + U/S
CT/MR when opaque ocular media prevents a
clear view
 Uveal melanoma
Findings
Soft tissue mass adjacent to outer layer of the globe
that bulges inward toward the vitreous
Mushroom cloud appearance
Hyperintense on T1 and hypointense on T2
 Sclera

Infection:
Sinusitis
Viral
Pseudotumor
Detachment
 Scleritis
granulomatous inflammatorydisorder
Erythema and chemosis
Characteristics
optic disk edema
exudative retinal detachment
Choroidal folds and scleral thickening >> elevated mass
 Scleritis
Findings:
CT:
thickened posterior sclera, may enhanced
Thickening of extraocular muscles can also be seen
Magnetic resonance imaging
nodular elevation into the vitreous
Iso-to-hyperintense to normal sclera on T1
Hypointense on T2
moderate to marked inhomogeneous enhancement with Gd
A retinal detachment appears as a crescent-shaped area that i
hyperintense on both T1 and T2
T1 fs + C
 Globe Rupture
collapsed globe (flat tire appearance)
 Globe Rupture

presence
of intra-ocular air or a foreign body
thick
posterior sclera
hazy
outline of the globe
enlarged anterior chamber
Retinal and choroidal detachment
Retinal epithelium ends at the ora serrata
> retinal detachment will not go beneath it
Retinal detachment with hemorrhage
adults with DM and HTN
shaken baby syndrome
Choroidal detachment
recent intraocular surgery
trauma
 Detachment

Retinal Choroidal
Retinal
Choroidal
 Coloboma
A congenital globe defect, usually at the optic
nerve insertion point
Often there is microphtalmia and the eye
protrudes inferiorly.
10% also have other CNS anomalies.
Findings:
a small globe with a cystic outpouching of vitreou
at the optic nerve attachment site.
Retroocular cyst
 Coloboma

Coloboma
Coloboma

can be part of the CHARGE syndrome:
Heart anomalies

choanal Atresia

Retardation of growth and development

Genital and Ear anomalies.

Coloboma can also be part of the COACH syndrome:

Cerebellar vermis hypoplasia,

Oligophrenia (MR)

congenital Ataxia

Coloboma

Hepatic fibrosis.
Diseases of the obit
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
 Optic neuritis
Presentations
Visual loss over hours to days
Pain on movement and tenderness when pressure
applied to globe
Sporadic/multiple sclerosis
If found, T2 whole brain should be done
Less common causes
pseudotumor, sarcoidosis, radiation, viral, TB,
syphilis neuritis
Optic neuritis
Multiple sclerosis
 Devic's syndrome
Neuromyelitis optica
 Optic nerve/sheath tumor
Optic nerve sheath meningioma
Optic nerve glioma
 Meningioma

Themost common optic nerve sheath tumor

ischemic neuropathy due to venous obstruction

progressive visual loss, papilledema, optic atrophy, pale di
association with NF-2
Strong

Abnormal enhancement of the optic nerve sheath

Calcifications 20-50%
Abnormal enhancement of
the optic nerve sheath
 Optic nerve glioma
Juvenile pilocytic astrocytomas WHO type 1
Anywhere along the optic tract from the occipita
region to the chiasm and the optic nerve

50% of optic nerve glioma have NF1.

Only 10% of NF1 have optic nerve glioma.
Less commonly cystic in NF than in non-NF

Age 4-5 years and only 20% of patients have

visual symptoms.
 Neurofibromatosis type I

At least 2 of
Six cafe au lait macules
Two neurofibromas of any type or 1 plexiform neurofibroma
Multiple freckles in the axillary or inguinal region
Bone dysplasia
Optic glioma
Two or more iris hamartomas (Lisch nodules)
A first-degree relative with NF1
Optic nerve glioma

T2 T1fs + Gd
Diseases of the obit
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
 Thyroid opthalmopathy
Compressive optic neuropathy
Direct compression by the muscles
Ischemic by compression of the vessels
diopathic inflammatory pseudotumor
Idiopathic inflammation of the orbit
Any part of the orbit: muscles, tendons, fat, optic
nerve, nerve sheet, lacrimal gland
Pseudotumor
Thyroid eye Pseudotumor
Diseases of the obit
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
Periorbital abscess
 Periorbital abscess
Complications
Venous thrombosis of the superior and inferior
ophthalmic vein
Cavernous sinus thrombosis and cavernous-
carotid fistula in certain fungal sinusitis
(e.g.aspergillosis)
 Orbital and periorbital cellulitis

Orbital VS periorbital cellulitis ->

A
>>'red
thehot eye' septum
orbital and proptosis
<<

Periorbital cellulitis
The orbital septum and the superficial part
OPDPO ATB
Orbital cellulitis
the structures posterior to the septum are also involved.
IPD--IV ATB
Orbital cellulitis
Proptosis with sphenoid wing lesion

our sphenoid wing lesions that can cause proptosis:

Fibrous dysplasia
Paget's disease
Meningioma with hyperostosis of the sphenoid wing
 Orbital appendages
Lacrimal gland
lacrimal sac
lacrimal duct
 Lesions of the lacrimal gland
Inflammation
The most common lesions of the lacrimal gland
i.e. Sjoegren's, TB, fungus, pseudotumor
Do not cause masses
Children
congenital obstructions of the valves in the
lacrimal duct -> dacryocystoceles
adults
strictures from ethmoid sinusitis or stones ->
epiphera or increased tearing
Dacryocystocele
 Lesions of the lacrimal gland

Lacrimal gland mass

Lymphoma
Pleomorphic adenoma
Epithelial tumors (adenoid cystic tumor)
 Tumors
Epidermoid and dermoid tumors
Developmental
Usually arises anteriorly between the globe and
orbital periosteum
Well-circumscribed cystic masses containing
debris
Dermoid may contain fat, teeth, and hair
 Vascular malformation

Carotid
cavernous sinus fistula
Cavernous
hemangioma
Capillary
hemangioma
Lymphangioma
Carotid cavernous sinus fistula

Cavernous carotid a.
cavernous sinus
 Capillary hemangioma

months of age
6-12

Involutional at 5-7 years of age

Mostly skin, but also in the extraconal of the eye

Posterior fossa malformations,
PHACE-syndrome:

Hemangiomas

Arterial anomalies

Cardiac malformation
Eye abnormalities such as coloboma, glaucoma
CTirregular margin
Capillary hemangioma
Venous vascular malformations

The most common intraconal mass in an adult

45-year-old women with slow progressive
proptosis, diplopia, visual loss
Most are unilocular

Phleboliths
CT

sharply circumscribed, round, dense mass
apex
Spare
erosion
Bony
Venous vascular malformation
 lymphatic malformation
Lymphangioma
Childhood onset (60% before 16 y/o)
May abruptly hemorrhage resulting in fluid-fluid
levels
May be extraconal or multicompartmental
Association with intracranial malformations
Turner's
fetal alcohol syndrome
Absence of contrast enhancement in pure
lymphatic lesions
lymphatic malformation
 orbital varix

Propulsion of the eye during straining

During valsalva -> extreme dilation

Valsalva
 Conclusion

Orbital anatomy
Disease approach
Diseases of the orbit
Approach to diseases
Globe
Optic nerve &
sheath
Conal-intraconal
area
Extraconal area
The globe
Disease approach
Q&A
 References
Harnsberger, H. Ric., and H. Ric.
Harnsberger. Handbook of Head and Neck
Imaging. St. Louis: Mosby, 1995. Print.
Grossman, Robert I., and David M.
Yousem. Neuroradiology. Philadelphia, PA:
Mosby Elsevier, 2010. Print.
"Orbita - Pathology." The Radiology Assistant
N.p., n.d. Web. 20 May 2014
Thank You