Pigmented Paravenous Chorioretinal Atrophy: Clinical Spectrum and Multimodal Imaging Characteristics

Author: Eun Kyoung Lee (Korea, Democratic People's Republic of)

Co-authors: Sang-Yoon Lee, Baek-Lok Oh, Chang Ki Yoon, Un Chul Park, Hyeong Gon Yu

Purpose

To investigate the clinical findings and natural course of patients with pigmented paravenous chorioretinal atrophy (PPCRA) using multimodal imaging.

Setting/Venue

Retrospective, observational case series.

Methods

We reviewed the records of consecutive patients diagnosed with PPCRA at a single center and assessed serial fundus photographs, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography 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 nine (36.0%) complained of nyctalopia. We divided fundus appearance into one of three 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, while ten (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 stable 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%).

Conlusions

PPCRA can present with a more variable expressivity than previously described. Multimodal imaging can provide insights into its clinical characteristics to facilitate the diagnosis, classification, and follow-up of these patients.

Financial Disclosure

No financial relationship to disclose.