[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.
Suzuki
R
R
Department of Ophthalmology, Yamaguchi University School of Medicine, Japan.
Hirose
T
T
D002648:Child; D002675:Child, Preschool; D002829:Choroid / Q000473:pathology; D005192:Family Health; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008266:Macula Lutea / Q000473:pathology; D008268:Macular Degeneration / Q000175:diagnosis* / Q000235:genetics; D008297:Male