Abstract

Pigmented Paravenous Retinochoroidal atrophy (PPRCA) is a rare retinal pathology characterized by pigment clumping around retinal veins with underlying retino-choroidal atrophy which occurs due to retinal pigment epithelium damage. We present a case of 21-yr-old male having diminished vision in left eye from past 5 years. BCVA OD:6/6 OS: 6/60. Anterior segment examination -normal. Fundus examination of left eye showed pigment clumping around retinal vessels and chorioretinal atrophy extending from optic disc to macula and full thickness macular hole. SD-OCT shows outer retinal thinning along with full thickness macular hole. Fundus autofluorescence of left eye shows area of chorioretinal atrophy starting from optic disc and extending along superior macular arcades with hyperfluorescent borders along it. ERG showed decreased scotopic and photopic responses. It's a unique case as macular findings reported with PPRCA are lamellar macular hole, cystoid macular edema, and macular coloboma