Ophthalmologic and electro-oculographic findings in Gardner's syndrome.
We examined six patients with Gardner's syndrome, eight first-degree relatives, and 31 age- and sex-matched controls to document the presence, distribution, and morphologic features of congenital hypertrophy of the retinal pigment epithelium. Patients with Gardner's syndrome had multiple, bilateral lesions, with 288 of 346 foci (83%) located posterior to the equator. Linear-shaped congenital hypertrophy of the retinal pigment epithelium, a distinctive finding in these patients, accounted for 44 of 140 large lesions (31%). Despite multifocal fundus involvement, results of electro-oculography were normal in all eyes tested.
Stein
E A
EA
Department of Ophthalmology, University of Colorado School of Medicine, Denver.
Brady
K D
KD
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004585:Electrooculography; D005736:Gardner Syndrome / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D006984:Hypertrophy; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology / Q000503:physiopathology; D010858:Pigmentation