Retinal vascular changes in congenital hypertrophy of the retinal pigment epithelium.

Abstract

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a well-defined clinical entity with usually well-delineated, pigmented oval or round lesion with smooth or scalloped margins. Occasional retinal vascular changes have been reported previously. To assess the prevalence of these changes, the authors performed a retrospective analysis of 12 patients with CHRPE, for whom fluorescein angiography allowed visualization of the entire lesion and of the retinal vascular capillary bed. Retinal vascular changes were found in 11 (91%) of these 12 patients. The changes consisted of capillary rarefaction in all 11 patients, with areas of capillary nonperfusion exceeding 1 disc diameter (DD) in three patients (25%), micro-aneurysmal capillary dilatations in three (25%), and chorioretinal anastomosis in one. These results suggest that the above changes could constitute clinical and angiographic characteristics of CHRPE and allow easy corroboration of its diagnosis, thus avoiding the need for further clinical investigations.