Vitreochorioretinal degeneration associated with trichomegaly

A 15-year-old white boy had bilateral vitreochorioretinal degeneration associated with trichomegaly. Findings on electroretinography and fluorescein angiography provided additional information on the retinal changes not noted in 3 previously reported cases having these associated features. Although similar on ophthalmoscopy, fluourescein angiography, and by electroretinography, the retinal disease in our patient differed from cases of gyrate atrophy of the choroid and retina in having normal plasma ornithine levels.