[A special form of Stargardt's disease/fundus flavimaculatus]

Stargardt's disease is a bull's-eye macular dystrophy with the characteristic appearance of a perifoveal lesion. We have observed four cases of an autosomal recessive disease with fundus features undistinguishable from Stargardt's disease, but with a different nature and much poorer prognosis. One case typical of Stargardt's disease characterized with macular degeneration showed a bull's-eye with dark choroid. Sixteen years later, the same eye developed bone corpuscle pigmentation in the periphery with non-recordable electroretinogram. The other three cases were members of a single family. They showed typical macular degeneration consistent with Stargardt's disease but had peripheral degeneration as well. Taking into account that there is a great diversity of retinal functions in Stargardt's disease, these features are different from those reported previously. Stargardt's disease should not be considered as a single clinical entity with a fair prognosis.