Idiopathic Sclerosing Orbital Inflammation – A Case Series Study

Abstract

Aims: It was the aim of this study to perform a review of the clinical features, treatments and outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) in Taiwan. Methods: All patients with histologically confirmed ISOI from 1995 to 2007 at the National Taiwan University Hospital were included. We reviewed the medical charts to see the clinical presentation, treatment modalities, treatment efficacy and final clinical status of these patients. Results: Six male and 3 female patients ranging in age from 33 to 82 years were included. The most common symptoms were pain and blurred vision (both 77.8%). Reduced visual acuity and proptosis were the most common signs (both 89%). Five patients received oral steroid treatment with/without pulse therapy combined with tissue removal, the response to which was good in 1 patient and partially effective in the other 4 patients. One patient received oral steroids and surgery, followed by radiotherapy, with only partial response. Patients with oral prednisolone or surgery only all had poor responses. Conclusion: ISOI is rare and also difficult to diagnose and manage. Early intervention with steroids/immunosuppressants combined with surgical debulking procedures may result in control or regression of this recalcitrant disease.

Suspected idiopathic sclerosing orbital inflammation presenting as immunoglobulin G4-related disease: a case report

“…In addition, ISOI is an ill-defined heterogeneous entity. There are many reports on ISOI [1-4]. Early intervention with immunosuppression in the form of corticosteroids can result in the control and even regression of the disease.…”

Myeloid Sarcoma: A Rare Case of an Orbital Mass Mimicking Orbital Pseudotumor Requiring Neurosurgical Intervention

“…Our patient was unique in that the initial imaging demonstrated involvement of several of the EOMs and then progressed to involve all of the EOMs but with no well-defined mass. Such imaging findings are frequently seen in patients with endocrine orbitopathy or orbital pseudotumor [ 18 – 22 ].…”

Orbital inflammatory disease management

“…The current mainstays for treatment of SOI include corticosteroids and surgical debulking. Corticosteroid administration is the most common initial treatment for SOI [29,[31][32][33]. The use of steroids alone has produced varying results in the literature.…”