Fundus flavimaculatus with severely reduced cone electroretinogram.

An 11-year-old girl complaining of progressive visual loss in both eyes showed atrophic macular degeneration with yellowish white flecks around the fovea in both eyegrounds. Decreased vision, central scotoma with normal peripheral visual field, and angiographic dark choroid except for transmitted hyperfluorescence in the macula and at the flecks confirmed the diagnosis of fundus flavimaculatus with atrophic macula. Electroretinograms, however, revealed severely reduced cone responses associated with almost normal rod response, which led to the diagnosis of cone dystrophy. The rare combination of angiographic evidence of fundus flavimaculatus and electroretinographically diagnosed cone dystrophy was discussed.