Pigmented Paravenous Chorioretinal Atrophy: Clinical Spectrum and Multimodal Imaging Characteristics

PURPOSE: To investigate the clinical findings and natu-ral course of patients with pigmented paravenous chorior-etinal atrophy (PPCRA) using multimodal imaging. DESIGN: Retrospective, observational case series. METHODS: We reviewed the records of consecutive pa-tients diagnosed with PPCRA at a single center and assessed serial fundus photographs, fundus autofluores-cence (FAF), and spectral-domain optical coherence to-mography images. Electrophysiological findings and visual field analysis were also reviewed. RESULTS: The study included 50 eyes in 25 patients. The mean age of the population was 51.6 +/- 14.6 years. Nine patients (36.0%) were asymptomatic and 9 (36.0%) complained of nyctalopia. We divided fundus appearance into one of 3 groups: paravenous (58.0%), focal (16.0%), and confluent (26.0%). Of the 50 eyes, macular involvement was present in 13 eyes (26.0%). Fifteen patients (60.0%) demonstrated a symmetric fundus appearance, whereas 10 (40.0%) had marked asymmetry. Eight eyes (16.0%) exhibited apparent changes in fundus findings, over a mean follow-up period of 8.8 years. FAF imaging was most sensitive to evaluate the extent of lesions. Sixteen eyes (44.4%) showed progressive visual field loss during the follow-up period. Most patients maintained sta-ble vision, and 36 eyes (72.0%) had a final visual acuity of 20/50 or better. Nevertheless, some eyes with macular involvement experienced severe deterioration in vision. Electrophysiological data were variable, and interocular asymmetry was common (45.8%). CONCLUSIONS: PPCRA can present with a more vari-able expressivity than previously described. Multimodal imaging can provide insights into its clinical characteris-tics to facilitate the diagnosis, classification, and follow-up of these patients. (Am J Ophthalmol 2021;224: 120-132. (c) 2020 Elsevier Inc. All rights reserved.)