Case Report: Pigmented paravenous retinochoroidal atrophy: a case report

Abstract

This article, to the best of our knowledge, reports the youngest typical case of pigmented paravenous retinochoroidal atrophy (PPRCA) reported to date. A 27-month-old girl presented with exodeviation in her right eye. She had normal birth and development with unremarkable family history. There were no inflammatory signs. In funduscopy, typical bilateral radial paravenous pigmentary changes and retinochoroidal atrophy were noticed in both eyes. The pigmentations consisted of coarse black pigmentations and fine subretinal yellowish round flecks. They arborized into the peripheral retina along the veins. Unaffected areas between the lesions seemed to be normal. Electroretinogram (ERG) responses showed mild to moderate reductions in both scotopic and photopic tests. Based on retinal examination and ERG findings PPRCA was diagnosed. On 16-month follow up, clinical and ERG findings were the same as the initial presentation. This case showed no progression during 16 months of follow up, which may indicate that primary congenital PPRCA with no inflammatory association may be a non-progressive disease.