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Cystoid Macular Edema in Retinitis Pigmentosa: Pathophysiologic Insight Using Swept-Source Optical Coherence Tomography and Angiography
Journal article   Open access   Peer reviewed

Cystoid Macular Edema in Retinitis Pigmentosa: Pathophysiologic Insight Using Swept-Source Optical Coherence Tomography and Angiography

Taariq K. Mohammed, Kyle M. Green, D. Brice Critser, S. Scott Whitmore, Lauryn J. Renze, Robert F. Mullins, Edwin M. Stone and Ian C. Han
Ophthalmology science (Online), 101193
04/2026
DOI: 10.1016/j.xops.2026.101193
url
https://doi.org/10.1016/j.xops.2026.101193View
Published (Version of record) Open Access

Abstract

Objective Cystoid macular edema (CME) is relatively common in patients with retinitis pigmentosa (RP), but its pathophysiology is poorly understood. This study aims to provide insight into pathophysiologic mechanisms of CME formation using swept source optical coherence tomography angiography (SS-OCTA). Design Retrospective, cross-sectional study at a tertiary referral center. Participants 70 consecutive patients with molecularly-confirmed RP (associated with RHO , USH2A , or RPGR ), with and without CME. Methods All patients underwent complete ophthalmic examination with multimodal imaging, including SS-OCT with SS-OCTA (PLEX Elite 9000; Carl-Zeiss Meditec Inc, Dublin, California). Images were then analyzed in a semi-automated fashion using ImageJ (National Institutes of Health, Bethesda, Maryland). Main Outcome Measures Structural and vascular abnormalities seen on SS-OCT and SS-OCTA in patients with RP and spatial overlap between CME areas, vascular flow loss in the deep capillary plexus (DCP), and preserved external limiting membrane (ELM) and ellipsoid zone (EZ). Results 129 eyes from 70 patients (mean age 37 years, range 6 to 78) with molecularly-confirmed RP were included. Median best-corrected visual acuity measured 0.31 logMAR (range-0.12 to 2.7). Overall, 31% of patients had CME (10% in RPGR, 36% in USH2A , 48% in RHO ). There was a strong correlation between regions of CME and ELM preservation (97% spatial overlap overall) as well as DCP flow loss (83% spatial overlap). There was moderate correlation between regions of CME and EZ preservation (66% spatial overlap). Conclusions CME is relatively common in RP, with different prevalence depending on genotype. There is strong spatial overlap between areas of DCP loss and ELM preservation regardless of genotype, suggesting a shared mechanism of impaired fluid clearance at the level of the DCP.

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