Abstract
Purpose :
To study the poorly understood natural history of recessive retinitis pigmentosa related to EYS variants, the Foundation Fighting Blindness Consortium is conducting a 4-year multicenter, international natural history study: Rate of Progression in EYS-Related Retinal Degeneration (Pro-EYS). We evaluated baseline mesopic microperimetry (MP) and spectral domain optical coherence tomography (OCT) metrics.
Methods :
MP was performed with a MAIA system (Figure 1) and OCT was captured with Heidelberg Spectralis. Intra-class correlation coefficients (ICC) were used to assess the agreement between left and right eyes; useful information for early stage clinical trials. Bland-Altman plots were used to assess variability of repeated MP tests. The association between MP mean sensitivity, OCT ellipsoid zone (EZ) area, and central subfield thickness (CST within the center 1mm) was assessed using Spearman correlation coefficients (r).
Results :
Participants in ProEYS had a median age of 45 years (range 19-84) with median duration of disease of 19 years (range 2-54) and included 96 unique EYS variants. All study participants (N=103) had OCT, while MP was obtained at selected sites (N=71). MP mean (SD) sensitivity was 3.9 (4.8) dB. The MP mean sensitivity for both study eyes was highly correlated (Mean (SD) left vs right: 3.90 (4.79) vs 3.94 (4.77) dB, ICC = 0.96). Bland-Altman analysis showed mean difference between MP tests 1 and 2 near 0 (Figure 2, mean difference (SD): -0.02 (1.13) dB). The mean (SD) OCT EZ area was 2.12 (3.77) mm2. EZ area values remain similar after axial length correction (Mean (SD): 2.19 (3.78)). The mean CST in eyes without cystoid macular edema (CME, N=81) was 221 (50) microns. The EZ area (Mean (SD) left vs right: 2.27 (4.19) vs 1.96 (3.31), ICC =0.78) and CST without CME (219.8 (48.4) vs 222.3 (52.4), ICC = 0.96)) were symmetrical between eyes. Mean MP sensitivity was more highly correlated with OCT EZ area (r = 0.71, p <0.001) than with CST (r = 0.48, p <0.001). We found a strong correlation between EZ area and CST (r = 0.78, p <0.001).
Conclusions :
MP mean sensitivity, EZ area, and CST each show high inter-eye correlation. The structural measures of EZ area and CST were correlated with the functional measure of MP mean sensitivity.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.