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Change in Cone Structure Over 24 Months in USH2A-Related Retinal Degeneration

Duncan, JL; Liang, W; Maguire, MG; Porco, TC; Wong, J; Audo, I; Cava, JA; ... Carroll, J; + view all (2023) Change in Cone Structure Over 24 Months in USH2A-Related Retinal Degeneration. American Journal of Ophthalmology , 252 pp. 77-93. 10.1016/j.ajo.2023.03.006. Green open access

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Abstract

Purpose: To describe cone structure changes using adaptive optics scanning laser ophthalmoscopy (AOSLO) in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) study. Design: Multicenter, longitudinal natural history study. Methods: AOSLO images were acquired at 4 centers, twice at baseline and annually for 24 months in this natural history study. For each eye, at least 10 regions of interest (ROIs) with ≥50 contiguous cones were analyzed by masked, independent graders. Cone spacing Z-scores, standard deviations from the normal mean at the measured location, were compared between graders and tests at baseline. The association of cone spacing with clinical characteristics was assessed using linear mixed effects regression models weighted by image quality score. Annual rates of change were calculated based on differences between visits. Results: Fourteen eyes of 14 participants were imaged, with 192 ROIs selected at baseline. There was variability among graders, which was greater in images with lower image quality score (P < .001). Cone spacing was significantly correlated with eccentricity, quality score, and disease duration (P < .02). On average, the cone spacing Z-score increased 0.14 annually (about 9%, P < .001). We observed no significant differences in rate of change between disease type (Usher syndrome or retinitis pigmentosa), imaging site, or grader. Conclusions: Using current methods, the analysis of quantitative measures of cone structure showed some challenges, yet showed promise that AOSLO images can be used to characterize progressive change over 24 months. Additional multicenter studies using AOSLO are needed to advance cone mosaic metrics as sensitive outcome measures for clinical trials.

Type: Article
Title: Change in Cone Structure Over 24 Months in USH2A-Related Retinal Degeneration
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ajo.2023.03.006
Publisher version: https://doi.org/10.1016/j.ajo.2023.03.006
Language: English
Additional information: THIS IS AN OPEN ACCESS ARTICLE UNDER THE CC BY-NC-ND LICENSE ( HTTP://CREATIVECOMMONS.ORG/LICENSES/BY-NC-ND/4.0/ )
Keywords: Foundation Fighting Blindness Consortium Investigator Group
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10169903
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