Ophthalmic manifestations of fibrous dysplasia: a disease of children and adults

Abstract

Objective: To determine whether adults constitute a significant proportion of patients presenting with ophthalmic complications of fibrous dysplasia.

Design: A retrospective case series.

Participants: Twenty patients with biopsy-proven fibrous dysplasia participated.

Main outcome measures: Patient demographics, major signs and symptoms, occurrence of visual loss, pattern of bone involvement, and radiographic appearance of fibrous dysplasia were studied.

Results: In contrast to observations made in the past, the authors found that adults constitute a significant proportion of patients suffering from the ophthalmic complications of fibrous dysplasia. Of 20 patients, 9 were younger than 18 years of age (children) and 11 were 18 years of age or older (adults) at the time of presentation. Changes in facial contour and symmetry were the most common presenting signs and symptoms. Five patients, four of whom were adults, presented because of acute visual loss. Most patients had monostotic lesions that crossed suture lines to involve multiple craniofacial bones. On computed tomographic scan, most fibrous dysplasia lesions had a characteristic, pagetoid appearance, with alternating areas of radiolucency and radiodensity.

Conclusions: Because fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and disfigurement, as well as a treatable cause of blindness in both children and adults, the diagnosis of fibrous dysplasia should not be ruled out based solely on the age of the patient. The characteristic radiologic appearance of this disease allows one to differentiate fibrous dysplasia from other tumors associated with bony expansion or density changes, specifically meningioma.