Pigmented paravenous retinochoroidal atrophy

A patient with paravenous pigmented chorioretinal atrophy is presented. A differential diagnosis and review of the literature are discussed. Fluorescein angiography confirms the presence of a widespread paravenous retinal pigment epithelial defect with minimally affected choroidal vasculature. The visual fields are constricted in proportion to the extent of the pigmentary abnormality. The electro-oculogram and electroretinogram show reduced responses indicative of a more extensive abnormality than is visible ophthalmoscopically. The etiology and natural course of this entity are unknown.