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idiopathic orbital inflammatory disease
Idiopathic Orbital Inflammatory Disease, or orbital pseudotumor, refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve palsy (Tolosa-Hunt syndrome), uveitis, and retinal detachment. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was first described by Gleason〔Gleason JE. Idipathic myositis involving the extraocular muscles. Ophthalmol Rec.12:471–478, 1903〕 in 1903 and by Busse and Hochhmein.〔Busse O, Hochheim W. cited by Dunnington JH, Berke RN. Exophthalmos due to chronic orbital myositic. Arch Ophthal . 30:446–466, 1943〕 It was then characterized as a distinct entity in 1905 by Birch-Hirschfeld.〔Birch-Hirschfeld A. Zur diagnostic and pathologic der orbital tumoren. Ber Dtsch Ophthalmol Ges. 32: 127–135, 1905〕〔Birch-Hirschfeld A. Handbuch der gesamten augenheilkunde, vol. 9. Berlin: Julius Springer. p. 251–253, 1930〕 It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause.〔 Its diagnosis is of exclusion once neoplasm, primary infection and systemic disorders have been ruled-out. Once diagnosed, it is characterized by its chronicity, anatomic location or histologic subtype.〔
Idiopathic orbital inflammation (IOI) has a varied clinical presentation depending on the involved tissue. It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat.〔 Its former name, orbital pseudotumor, is derived due to resemblance to a neoplasm. However, histologically it is characterized by inflammation. Although a benign condition, it may present with an aggressive clinical course with severe vision loss and oculomotor dysfunction.〔Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491–9, 2003〕
==Signs and symptoms==
Affected individuals typically present with sudden painful proptosis, redness, and edema. Proptosis will vary according to the degree of inflammation, fibrosis, and mass effect. Occasionally, ptosis, chemosis, motility dysfunction (ophthalmoplegia), and optic neuropathy are seen.〔〔Narla LD et al: Inflammatory Pseudotumor. RadioGraphics. 23(3):719–729, 2003〕 In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. Symptoms usually develop acutely (hours to days), but have also been seen to develop over several weeks or even months.Malaise, headaches, and nausea may accompany these symptoms. Other unusual presentations described include cystoid macular edema, temporal arteritis, and cluster headaches.〔
Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation.〔〔 The most common sign is proptosis, but redness and pain are also experienced. Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. The presence of uveitis generally implies a poor outcome for pediatric IOI. Bilateral presentation may have a higher incidence of systemic disease.〔
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