Longitudinal Behavior of the Hyper-Reflective Ganglion Cell Layer Band and Its Association With Ellipsoid Zone Constriction Rate in RP

Purpose: To investigate the longitudinal behavior of the hyper-reflective ganglion cell layer band (HGB) and its association with ellipsoid zone (EZ) constriction over time in molecularly characterized RP, based on the hypothesis that the HGB represents a manifestation of inner retinal remodeling with potential prognostic significance. Methods: We conducted a retrospective cohort study featuring patients with typical RP caused by variants in photoreceptor-associated genes and ≥6 months follow-up. Optical coherence tomography images were reviewed for the presence of HGB, epiretinal membrane, and cystoid macular edema. Predictors of ganglion cell layer (GCL) thickness and longitudinal changes in best-corrected visual acuity were assessed with linear mixed models. Longitudinal EZ constriction was estimated using exponential mixed models with log-transformed EZ width. Results: We included 118 eyes from 61 patients (30 females; mean baseline age, 34.7 ± 13.2 years) with a mean follow-up of 4.5 ± 2.6 years. HGB was detected at baseline in 27 eyes (22.9%) from 14 patients. Over follow-up, no eyes developed or lost the HGB. Baseline GCL thickness was significantly greater in eyes with HGB compared with those without (65 ± 10 µm vs. 53 ± 8 µm). Compared with autosomal recessive RP (5.5% per year), EZ constriction was significantly slower in autosomal dominant RP (2.8% per year; P = 0.0005) and faster in X-linked RP (7.7% per year; P = 0.021). Eyes without HGB showed a decline of 6.4% per year, compared with 4.3% per year in eyes with HGB (P = 0.001). Conclusions: HGB is a relatively stable optical coherence tomography finding in RP and is associated with increased GCL thickness and a slower rate of EZ constriction.