A clinical review of Stargardt's disease and/or fundus flavimaculatus with follow-up.

Out of 49 patients diagnosed as having Stargardt's disease that have been clinically reviewed, 22 of these cases have been followed for a period up to 13 years. The cases have been studied in three subgroups, according to their fundus appearance. Patients from group I were those whose lesions were confined to the macula, showing no flecks in the retina. In group II the macular lesion was surrounded by perimacular flecks and in group III fundus flavimaculatus flecks were seen, diffusely scattered in the posterior pole. It appears that the prognosis of group I is better than the other groups, where surrounding flecks are seen in addition to the macular lesion. The follow-up indicates that the disease evolves with a rather large spectrum of expressivity. Assessment of a predictive factor for the evolution of the disease is not yet possible. However, the observation of a dark choroid appears to be strongly suggestive for a further centrifugal progression of the disease.