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Orbital Pseudotumor
Dr Renuka Srinivasan MS 1, Dr Datta Gulnar MS 2
Clinical features
IOIS can present with varying range of clinical features
depending on the orbital structures involved, the degree
of inflammation and fibrosis 3,8. The presentation is
usually acute with proptosis, diplopia, orbital pain,
eyelid swelling, ptosis, chemosis and visual loss 1,5,7.
Relapses and remissions with or without treatment are
not uncommon.
Pseudotumour with significant desmoplastic change
Fig. 1. Idiopathic sclerosing orbital inflammation (ISOI)
typically present with slowly progressive visual loss, presenting with proptosis and ophthalmoplegia.
diplopia or proptosis 1. Commonly involved structures
include orbital fat, lacrimal gland, extraocular muscles 3,
insidious presenting with diplopia, decreased vision and
others being, optic nerve, sclera and tenon. Orbital
proptosis 1. ISOI is diagnosed based on the characteristic
involvement may be focal resulting in pseudotumor
histological picture of marked fibrosis with sparse mixed
variants, myositis, dacryoadenitis, optic perineuritis,
chronic inflammatory infiltrate 11. It has a predilection
periscleritis and sclerotenonitis 8. A posterior pattern
for the posterior1 superior or lateral orbit especially the
of pseudotumor presents with symptoms of orbital apex
lacrimal gland, rich in lymphocytes which play a critical
syndrome. Patient has signs of optic nerve dysfunction
role in causing fibrosis 11. The sclerosing variant is
and ophthalmoplegia. These include diplopia,
associated with systemic multifocal fibrosclerosis like
decreased vision, dyschromatopsia, visual field defects,
retroperitoneal fibrosis, mediastinal fibrosis, sclerosing
relative afferent pupillary defect and disc edema.
cholangitis, Riedel’s sclerosing thyroiditis and
The diverse forms of orbital pseudotumour have varying pachymeningitis 1. This form of pseudotumor typically
clinical picture. does not respond to steroid therapy
Dacryoadenitis -Pseudotumour of lacrimal gland has
typical presentation of dacryoadenitis. The characteristic Paediatric pseudotumor
sign is “S” shaped ptosis with associated superotemporal The clinical features of IOIS are peculiar in children.
conjunctival chemosis and congestion 1,9. The lateral 6-16 % of cases occur in the first two decades of
rectus muscle being in close proximity is commonly life 1,6,12. Bilateral involvement is common and is
involved resulting in painful ophthalmoparesis and associated with iritis, and optic disc edema 5,8,12. The
diplopia1. associated constitutional symptoms in children lead to
Orbital myositis – This condition is a common erroneous diagnosis. Recurrences are common and
variant of IOIS presenting with diplopia and pain morbidity is high 1,8. Eosinophilia of peripheral blood
typically exacerbated on ocular movement 1. There is and in tissue biopsy is a feature seen in one third of
restriction of ocular movement in the field of action of cases1. In children it is important to exclude orbital
the affected muscles. Localized conjunctival injection cellulitis, dacryoadenitis, rhabdomyosarcoma,
and chemosis are seen at the tendinous insertion of retinoblastoma, neuroblastoma, dermoid cyst and
involved muscle 1,10. Medial and superior recti are hemangioma before diagnosing pseudotumor 1.
commonly involved 1,10. The entire muscle including
the belly and tendon is enlarged 1,10. Diagnosis
The diagnosis of pseudotumour orbit is usually clinical
Idiopathic sclerosing orbital inflammation and confirmed by prompt response to steroids. In order
Idiopathic sclerosing orbital inflammation (ISOI) to rule out the systemic conditions mimicking
(Fig. 1) is a rare pathological subgroup of pseudotumor pseudotumor complete physical examination is
accounting for 5 % to 7.8 % of cases 11. The onset is essential followed by complete hemogram, erythrocyte
June 2009 Renuka Srinivasan et al. - Orbital Pseudotumor 129
tumors from true neoplasm of orbit. The therapeutic risk of development of gastrointestinal bleeding, and
response to corticosteroids is misleading and provides inability to obtain follow-up on steroid therapy 19.
wrong assurance as some improvement can occur in
Intraorbital injection of triamcinolone
other diseases. Presence of thyroid lid signs and tendon
acetonide 20-40 mg has also been shown to be
sparing extraocular muscle enlargement helps in
effective in the treatment of IOIS. with reduced systemic
differentiating it from IOIS 1. Orbital cellulitis is
side effects of oral steroids 20.
accompanied by signs of systemic toxicity including
fever, and leucocytosis with shift to left 1. A thorough Immunosuppressants: Cyclophosphamide
systemic work up will help in differentiating 200mg/day is used to treat patients with recurrence on
pseudotumor from systemic affections like sarcoidosis, steroid therapy 5. Cyclosporine 2-5mg/kg and methotrexate
lymphoma, leukemia’s 1. Rarely orbital affection may 7.5-12.5mg/kg are the steroid sparing drugs used 5.
be the only sign of the systemic disease. In the absence Immune modulators: Biological immunomodulators
of systemic disease, histopathology aids in have revolutionized the treatment of autoimmune
differentiating IOIS from other conditions. Fine needle diseases. Infliximab (chimeric monoclonal antibody),
aspiration biopsy or open biopsy may be performed for TNF-α blocker at 6 weekly dosage schedule of
this. 3-5 mg/kg has been recently introduced in the
treatment armamentarium of IOIS 21,22. TNF inhibition
Treatment is associated with increase in antinuclear antibodies
and systemic lupus erythematosus, hence concomitant
The spectrum of adjuvant treatment in IOI is broad
methotrexate therapy is recommended 21,22.
and evolving. Options include corticosteroids, radiation
therapy, non steroidal anti-inflammatory drugs, Radiation – Radiotherapy is used to treat patients
cytotoxic agents (chlorambucil, cyclophosphamide), intolerant or resistant to steroids. Dose ranging form
corticosteroid sparing immunosuppressants (for 1500 – 2500 cGy over 10-15 days is appropriate in
example, methotrexate, cyclosporine, azathioprine); steroid resistant cases5. Average time taken for response
intravenous immunoglobulin, plasmapheresis, and the to radiotherapy is 3-8 months. Localized mass, presence
newest class, biologic treatments, which includes anti- of lymphoid follicles, absence of eosinophils and initial
tumor necrosis factor alpha (TNFα) 4. response to steroids are good prognostic factors for
response to radiotherapy 19.
Oral steroids: The mainstay of therapy is
corticosteroid which has diagnostic sensitivity of 78 %
due to the prompt response of the condition to steroids 5. Complications
Recurrence rate of 50-60 % has been reported by Desmoplastic component of pseudotumour results in
previous studies with corticosteroids 7. Dose ranging fibrous entrapment of extraocular muscles resulting in
from 60-100 mg/day is initiated 8. High dose oral restriction of ocular movements and diplopia 1. Mass
steroid for 2-3 weeks followed by slow tapering is effect caused by both inflammation and desmoplasia
recommended 8. Effective immunosuppression needs causes compressive optic neuropathy and dysfunction
to be in sufficient dose and maintained for the duration of ocular motor nerves 1. Obstruction of venous drainage
of active disease 11. Intravenous pulse steroids are results in orbital congestion 1. IOIS has the tendency to
reserved for patients with rapid progression of spread intracranially, paranasal sinuses, into infratemporal
symptoms 5. Failures in corticosteroid treatment may and pterygopalatine fossa through the major openings
be termed primary, if there is no improvement despite in the posterior orbit; optic canal, superior and inferior
adequate steroid dosage; recalcitrant, if there is orbital fissure 1,2,11,16. Hence in cases with persistent or
breakthrough inflammation during tapering steroid recurrent or progressive clinical symptoms contrast
dosage; recurrent, if the pseudotumor recurs after a enhanced computed tomography and magnetic
period of remission. The systemic side effects due to resonance imaging is indicated 14. In the presence of
prolonged steroid therapy includes cushingoid extraorbital extension, biopsy should be performed to
symptoms and signs, growth retardation, weight gain, exclude other conditions mimicking ISOI 16. IOIS causes
June 2009 Renuka Srinivasan et al. - Orbital Pseudotumor 131
open angle glaucoma secondary to raised episcleral 7. Chirapapaisan N, Chuenkongkaew W, Pornpanich K and
Vangveeravong S. Orbital pseudotumor: clinical features
pressure. Engorged ciliary processes/ posterior scleritis
and outcomes. Asia Pacific Journal of Allergy and
with choroidal effusions/ relative obstruction of vortex Immunology 2007;25:215-18.
veins with swelling of uvea pushing iris-lens diaphragm 8. Snebold NG. Orbital pseudotumor syndromes. Curr
are the mechanism put forward for secondary angle opinion in Ophthalmol 1997;8:41-44.
closure glaucoma caused by pseudotumor 23. 9. Suchi ST, Gupta A, Sengupta S, Srinivasan R. Chronic
bilateral lacrimal gland pseudotumor in an adult. Indian
J Ophthalmol 2008;56:86-87.
Conclusion 10. Boonman ZFHM, de-Keizer RJW, Grsniewski-Wijnands
HS, Watson PG. Orbital myositis in scleritis. Br J
Orbital pseudotumor comprises a significant proportion Ophthalmol 2003;87:38-42.
of cases of orbital lesions. It should be considered in 11. Hsuan JD, Selva D, McNab AA, Sullivan TJ, Saeed P,
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Orbital pseudotumor is typically characterized by the Arch Ophthalmol. 2006;124:1244-50.
rapid development of pain, proptosis, and swelling 12. Ugale J, Rongkavilit C. An 11-year-old male with
recurrent orbital swelling. Paediatric annals
around the eye and orbit. Ultrasound and computed
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poorly defined margins. Patients with classic findings
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of orbital pseudotumor may be treated without a biopsy.
15. Atlas SW, Grossman RI, Savino PJ, Sergott RC, Schatz
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(which helps confirm the diagnosis). Atypical cases of pseudotumor. AJR 1987;148:803-8.
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17. Dhir SP, Zafarullah KM, Jain IS, Mohan K, Raina A.
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