Orbital Pseudotumor: Case Report and Literature Review

Abstract

The term orbital pseudotumor refers to a broad category of non-specific idiopathic inflammations of the orbit. This disease, which may affect any orbital structure, is one of the commonest causes of exophtalmus, occurring with a similar incidence in both sexes. The diagnosis is based on a combination of clinical, radiological and histopathological findings, after careful exclusion of specific systemic and local diseases. Many classification systems have been proposed and a range of therapeutic modalities, including surgery, steroids, immunosuppressive agents, and radiation therapy, have been employed by various authors in heterogeneous series of patients. This slowly proliferating disease, which usually presents with a long clinical history and high variability in clinical manifestations and prognosis, is difficult to manage with any of the available therapeutic options. The difficulties and controversies regarding the diagnostic and therapeutic management of these patients are addressed in an updated review of the literature and exemplified in our case report.

Orbital Pseudotumour

“…This descriptive term was first used in 1905 by Birch-Hirschfield to describe an inflammatory condition of the orbit of unknown aetiology. It therefore excludes specific infections, granulomatous diseases and collagen-vascular disorders such as polyarteritis nodosa and Wegener's granulomatosis [2]. The clinical picture of pseudotumour varies widely with single or combined signs of mass effect, inflammation and infiltration.…”