PIGMENTED PARAVENOUS RETINOCHOROIDAL ATROPHY (PPRCA) WITH OPTIC DISC DRUSEN

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare retinal disorder which is diagnosed primarily on its typical funduscopic appe arance of retinal pigment epithelial atrophy and clumping in a paraven ous distribution. Pigmented paravenous retinochoroidal atrophy is usua lly asymptomatic and seldom causes marked decrease in visual acuity or significant impairment of electrophysiologic functions. Optic nerve h ead drusen, which are thought to be inherited as an autosomal dominant trait, rare in blacks, and known to be associated with retinitis pigm entosa, have not been previously reported with PPRCA. The authors pres ent a case of PPRCA which is classic except for an additional finding of optic nerve head drusen. The heritability of PPRCA remains controve rsial but the authors' reported association of PPRCA and disc drusen s eems to suggest some genetic influence.