Parafoveal cone function in choroideremia assessed with adaptive optics optoretinography. Sci Rep 2024 Apr 09;14(1):8339
Date
04/10/2024Pubmed ID
38594294Pubmed Central ID
PMC11004114DOI
10.1038/s41598-024-58059-xScopus ID
2-s2.0-85189907880 (requires institutional sign-in at Scopus site)Abstract
Choroideremia (CHM) is an X-linked retinal degeneration leading to loss of the photoreceptors, retinal pigment epithelium (RPE), and choroid. Adaptive optics optoretinography is an emerging technique for noninvasive, objective assessment of photoreceptor function. Here, we investigate parafoveal cone function in CHM using adaptive optics optoretinography and compare with cone structure and clinical assessments of vision. Parafoveal cone mosaics of 10 CHM and four normal-sighted participants were imaged with an adaptive optics scanning light ophthalmoscope. While acquiring video sequences, a 2 s 550Δ10 nm, 450 nW/deg2 stimulus was presented. Videos were registered and the intensity of each cone in each frame was extracted, normalized, standardized, and aggregated to generate the population optoretinogram (ORG) over time. A gamma-pdf was fit to the ORG and the peak was extracted as ORG amplitude. CHM ORG amplitudes were compared to normal and were correlated with bound cone density, ellipsoid zone to RPE/Bruch's membrane (EZ-to-RPE/BrM) distance, and foveal sensitivity using Pearson correlation analysis. ORG amplitude was significantly reduced in CHM compared to normal (0.22 ± 0.15 vs. 1.34 ± 0.31). In addition, CHM ORG amplitude was positively correlated with cone density, EZ-to-RPE/BrM distance, and foveal sensitivity. Our results demonstrate promise for using ORG as a biomarker of photoreceptor function.
Author List
Xu P, Cooper RF, Jiang YY, Morgan JIWAuthor
Robert F. Cooper Ph.D Assistant Professor in the Biomedical Engineering department at Marquette UniversityMESH terms used to index this publication - Major topics in bold
ChoroidChoroideremia
Humans
Ophthalmoscopy
Retinal Cone Photoreceptor Cells
Retinal Pigment Epithelium
Tomography, Optical Coherence
