Pigmented Paravenous Retinochoroidal Atrophy

Abstract

Pigmented paravenous retinochoroidal atrophy appears much like retinitis pigmentosa to the uninitiated. Indeed there are many patients who have this disorder and spend a great deal of their life expecting to become blind. Funduscopically, these patients demonstrate retinal pigment spicules clustered around the retinal veins. Clinically, some have a mild nyctalopia and mild decrease in acuity; however, the optic atrophy, arteriolar narrowing, and progression of retinitis pigmentosa are not present. Electrophysiologically, we have seen normal photopic and scotopic electroretinograms. The fluorescein angiogram demonstrates a hypofluorescence around the veins that corresponds to the perivascular location of the spicules. There is also a decrease in the retinal pigment epithelium around the fovea. Macular fields show decreased perifoveal values, consistent with the hypopigmented perifoveal region. It seems that this disorder remains stable, without progression, over several years. It is unclear whether the entity is congenital or acquired from another illness. Genetic studies suggest that inheritance is sporadic.