Pigmented paravenous retinochoroidal atrophy associated with unilateral cystoid macular oedema

A 65-year-old man was referred to our department with complaints of blurred vision in the left eye. Funduscopic examination revealed areas of retinochoroidal atrophy along the retinal veins bilaterally and bone spicule pigmentation along the nasal and superior temporal venous branches, as well as macular oedema in the left eye. Fluorescein angiography, visual field test, optical coherence tomography and electrophysiological examination were performed, and results were compatible with the diagnosis of pigmented paravenous retinochoroidal atrophy (PPRCA). Treatment with topical dorzolamide and intravitreal bevacizumab in the left eye resulted in poor anatomical and visual response. There is scarce documentation of macular involvement with non-inflammatory unilateral cystoid macular oedema in PPRCA in the literature. Further investigation is required to elucidate the pathogenesis of PPRCA and to properly manage these patients.