Gyrate atrophy of choroid and retina complicated by vitreous hemorrhage.

We describe two patients with gyrate atrophy of the choroid and retina who suffered from vitreous hemorrhages in adolescence. The diagnosis of gyrate atrophy was confirmed biochemically and clinically; hyperornithinemia and a deficiency of ornithine ketoacid transaminase were confirmed biochemically. Typical fundus changes of scalloped chorioretinal atrophies with sharp margins, deteriorated dark adaptation, non-recordable electroretinogram, flat electrooculogram, and constricted visual fields were noted. We believe that the vitreous hemorrhage is an ocular complication in this disorder.