Inflammatory pigmented paravenous retinochoroidal atrophy

Purpose To describe an active inflammatory cause of pigmented paravenous retinochoroidal atrophy. Methods A 54-year-old female patient presented with complaints of worsening visual acuity and poor night vision was examined. Fundus examination was performed and color fundus photographs were taken. In addition to fluorescein angiography, visual field examinations and electroretinographic tests were performed. Macular evaluation was performed with optical coherence tomography. Results Both fundi showed circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins. She had also marked vitreous cells with snow ball opacities and cystoid macular edema in both eyes. Fluorescein angiography confirmed the presence of a hyperfluorescence due to widespread paravenous retinal pigment epithelial defect while ICG angiography disclosed hypofluorescence in all phases. The electroretinogram showed reduced responses especially in the left eye. Visual field tests showed scotomas corresponding with areas of atrophy along the retinal veins. Conclusions This is a report of the findings in pigmented paravenous retinochoroidal atrophy that is a nonspecific degenerative disease and may occur in association with systemic infections or inflammation. Ocular inflammation with cystoid macular edema is an unusual manifestation of the disease.