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 appearance of retinal pigment epithelial atrophy and clumping in a paravenous distribution. Pigmented paravenous retinochoroidal atrophy is usually asymptomatic and seldom causes marked decrease in visual acuity or significant impairment of electrophysiologic functions. Optic nerve head drusen, which are thought to be inherited as an autosomal dominant trait, rare in blacks, and known to be associated with retinitis pigmentosa, have not been previously reported with PPRCA. The authors present a case of PPRCA which is classic except for an additional finding of optic nerve head drusen. The heritability of PPRCA remains controversial but the authors' reported association of PPRCA and disc drusen seems to suggest some genetic influence.