Self-reported visual function and in-depth swept-source optical coherence tomography features of cystoid macular edema in retinitis pigmentosa

Abstract

Purpose To evaluate self-reported visual function in retinitis pigmentosa (RP) patients with and without cystoid macular edema (CME) and to explore associations between cystoid spaces (CS), retinal morphometric parameters, and clinical data using swept-source optical coherence tomography (SS-OCT). Methods In this cross-sectional case-control study (1:3) conducted at an Inherited Retinal Degenerations referral center in Portugal, RP patients with and without CME (matched for age and gender) completed the Michigan Retinal Degeneration Questionnaire (MRDQ) and underwent SS-OCT. Morphometric analysis, including ellipsoid zone area (EZA), was performed by two independent graders. In the CME group, detailed CS analysis was conducted. Correlations between clinical data - age, gender, best-corrected visual acuity (BCVA) - and MRDQ domains were evaluated. Results The study included 23 RP patients with CME (60.87% male, mean age 44.65 ± 13.58 years) and 69 without CME (49.28% male, mean age 47.94 ± 14.39 years). No significant differences were found between groups in almost all MRDQ domains, BCVA, or EZA. Age positively correlated with 4 MRDQ domains in both groups. BCVA negatively correlated with nearly all MRDQ domains. While EZA showed a negative correlation in both groups, it was significant only in RP without CME. In the CME group, centrally located, outer nuclear layer-involving and large CS were associated with worse BCVA but better EZA. Conclusion MRDQ responses strongly correlated with clinical parameters. CME does not seem to affect self-reported visual function in RP patients, and CS may not worsen visual function. Thus, aggressive treatment of CME in RP may not be necessary.