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James D. Hsuan, FRCOphth; Dinesh Selva, FRANZCO; Alan A. McNab, FRANZCO; Timothy J. Sullivan, FRANZCO; Peerooz Saeed, MD; Brett A. O’Donnell, FRANZCO

Arch Ophthalmol. 2006;124:1244-1250.

Objective  To perform a multicenter review of the clinical features and treatment of 31 patients with idiopathic sclerosing orbital inflammation.

Methods  We included all patients with histologically confirmed idiopathic sclerosing orbital inflammation from 5 regional orbital centers. We reviewed the case notes to determine the clinical presentation, diagnostic features, and response to treatment. The main outcome measures were duration and nature of symptoms, anatomical location of disease, histopathological findings, treatment modalities, treatment efficacy and adverse effects, and final clinical status.

Results  We included 13 male and 18 female patients ranging in age from 7 to 83 years. The average duration of symptoms at presentation was 13.4 months. There was a predilection for the lateral and superior quadrants. Thirteen patients had apical disease, and 4 had extraorbital involvement. Histopathological findings invariably showed sclerosis associated with a sparse mixed cellular infiltrate. Twenty-seven patients were treated with oral prednisolone, response to which was good in 9 patients, partial in 11, and poor in 7. Six patients were treated with a second-line immunosuppressive agent, and 6 received radiotherapy. The response to radiotherapy was generally poor.

Conclusions  Idiopathic sclerosing orbital inflammation is a rare condition that can be difficult to diagnose and manage. Early intervention with immunosuppression in the form of corticosteroids combined with second-line agents can result in control and even regression of the disease.

Author Affiliations: Department of Ophthalmology, Walton Hospital, Liverpool, England (Dr Hsuan); Oculoplastic and Orbital Unit, Department of Ophthalmology, Royal Adelaide Hospital, Adelaide, Australia (Dr Selva); Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia (Dr McNab); Eyelid, Lacrimal, and Orbital Clinic, Department of Ophthalmology, Royal Brisbane Hospital, Brisbane, Australia (Dr Sullivan); Orbital Centre, Department of Ophthalmology, Academic Medical Centre, Amsterdam, the Netherlands (Dr Saeed); and Department of Ophthalmology, Royal North Shore Hospital, Sydney, Australia (Dr O’Donnell).

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