Pigmented paravenous chorioretinal atrophy.

Two patients with pigmented paravenous chorioretinal atrophy are presented. Both patients had chorioretinal atrophy with pigment clusters located in the paravenous areas, and one of the patients had macular affection. This patient had exotropia and juvenile cataract, the other patient had senile cataract. Electroretinography showed decreased, but not totally extinguished potentials. Automatic and manual perimetry revealed relative and absolute scotomas corresponding to the atrophic paravenous areas. To our knowledge this is only the sixth report of pigmented paravenous chorioretinal atrophy with macular affection.