Ophthalmic manifestations of fibrous dysplasia: a disease of children and adults.
OBJECTIVE
To determine whether adults constitute a significant proportion of patients presenting with ophthalmic complications of fibrous dysplasia.


METHODS
A retrospective case series.


METHODS
Twenty patients with biopsy-proven fibrous dysplasia participated.


METHODS
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.
Katz
B J
BJ
Department of Ophthalmology and Visual Sciences, University of Iowa Hospitals and Clinics, Iowa City 52242-1091, USA.
Nerad
J A
JA
D000293:Adolescent; D000328:Adult; D000368:Aged; D001766:Blindness / Q000209:etiology; D002648:Child; D005147:Facial Bones / Q000000981:diagnostic imaging; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D008297:Male; D009916:Orbital Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D012189:Retrospective Studies; D012886:Skull / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed