Multimodal Imaging Studies of Pigmented Paravenous Retinochoroidal Atrophy: A Case Report

BACKGROUND Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare and poorly understood retinopathy, characterized by retinochoroidal atrophy and pigmentation along the retinal veins. Its etiology remains unclear; proposed mechanisms include inflammatory, developmental, and genetic factors. Here, we describe a 56-year-old woman who was diagnosed with PPRCA via multimodal imaging. CASE REPORT A 56-year-old Chinese woman presented with a 3-day history of vision deterioration in her left eye. Dilated fundus examination revealed bilateral pigment clumps and retinochoroidal atrophy along the retinal veins, which was more pronounced and displayed macular involvement in the left eye. Ultra-wide fundus fluorescein angiography showed hyperfluorescence consistent with retinal pigment epithelium degeneration, whereas fundus autofluorescence revealed hypoautofluorescent patches and hyperautofluorescence at lesion borders. Optical coherence tomography angiography demonstrated areas of flow void beneath the retinal pigment epithelium-Bruch membrane layer, suggestive of choriocapillaris hypoperfusion that corresponded with fundus autofluorescence findings. Spectral-domain optical coherence tomography showed absence of the myoid zone, ellipsoid zone, and interdigitation zone in the macular region, along with partial preservation of the retinal pigment epithelium. Based on these findings, a diagnosis of bilateral PPRCA was made. The patient was advised to undergo routine follow-up. CONCLUSIONS Multimodal imaging is essential to confirm the diagnosis of PPRCA. Optical coherence tomography angiography provides valuable insight into disease pathogenesis by demonstrating primary choriocapillaris involvement. Further research into genetic factors and potential therapeutic targets, including vascular endothelial growth factor inhibitors, is warranted to improve understanding and management of this condition.