Background: 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. Occa
sional retinal vascular changes have been reported previously. Purpose
and Method. To assess the prevalence of these changes, the authors pe
rformed a retrospective analysis of 12 patients with CHRPE, for whom f
luorescein angiography allowed visualization of the entire lesion and
of the retinal vascular capillary bed. Results: Retinal vascular chang
es were found in 11 (91%) of these 12 patients. The changes consisted
of capillary rarefaction in all 11 patients, with areas of capillary n
onperfusion exceeding 1 disc diameter (DD) in three patients (25%), mi
croaneurysmal capillary dilatations in three (25%), and chorioretinal
anastomosis in one. Conclusion: These results suggest that the above c
hanges could constitute clinical and angiographic characteristics of C
HRPE and allow easy corroboration of its diagnosis, thus avoiding the
need for further clinical investigations.