Eun Kyoung Lee1, Sang-Yoon Lee2, Baek-Lok Oh1, Chang Ki Yoon1, Un Chul Park1, Hyeong Gon Yu3. 1. Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea. 2. SNU Blue Eye Clinic, Seoul, Republic of Korea. 3. Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea. Electronic address: hgonyu@snu.ac.kr.
Abstract
PURPOSE: To investigate the clinical findings and natural course of patients with pigmented paravenous chorioretinal atrophy (PPCRA) using multimodal imaging. DESIGN: 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 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 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%). CONCLUSIONS: 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.
PURPOSE: To investigate the clinical findings and natural course of patients with pigmented paravenous chorioretinal atrophy (PPCRA) using multimodal imaging. DESIGN: 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 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 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%). CONCLUSIONS: 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.
Authors: Nora Siegal; Michal Gutowski; Lakshmi Akileswaran; Norman J Beauchamp; Lien-Chieh Ding; Christopher B Chambers; Russell N Van Gelder Journal: Sci Rep Date: 2021-07-28 Impact factor: 4.379