Idiopathic orbital inflammatory disease | |
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Other names | Orbital pseudotumor |
In this area there is a predominance of large histiocytes admixed with plasma cells. | |
Specialty | Ophthalmology |
Idiopathic orbital inflammatory (IOI) disease 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[1] in 1903 and by Busse and Hochheim.[2] It was then characterized as a distinct entity in 1905 by Birch-Hirschfeld.[3][4] It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause.[5] 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.[6]
Idiopathic orbital inflammation 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.[7] 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.[8]
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