Pigmented paravenous retinochoroidal atrophy

Purpose: V/e evaluated clinical features, diagnosis and characteristic findings in a cases of pigmented pa­ ravenous retinochoroidal atrophy. Case: Best corrected visual acuity of a 58 year old vvoman who was diagnosed on routine examination as pigmented paravenous retinochoroidal atrophy was 20/30 in the right eye and 20/50 in the left eye. No other family members are knovvn to have eye disease. Anterior segments and intraocular pres- sures were normal. İn ali qudrants, bilaterally, there was striking paravenous pigmentary retinal pigment epithelial atrophy vvith dense black bone-spicule pigmentation. The optic discs, maculas and retinal vessels outside of the pigmentary regions appeared normal. The fluorescein angiogram revealed areas of hyperfluorescence in- terspreaded vvith pigment clumps. There was no leakage from the retinal vessels. Conclusions: Pigmented pa­ ravenous retinochoroidal atrophy is a rare disorder that is not we understood or classified. The diagnosis of pig­ mented paravenous retinochoroidal atrophy is established on the opthalmoscopic appearence of a striking disturbance of the retinal pigment epithelium in a distinctly perivascular distribution vvith or vvithout bone spicule pigmentation.