Cone Photoreceptor Morphology in Choroideremia Assessed Using Non-Confocal Split-Detection Adaptive Optics Scanning Light Ophthalmoscopy

Choroideremia (CHM) is an X-linked inherited retinal degeneration causing loss of the photoreceptors, retinal pigment epithelium, and choriocapillaris, although patients typically retain a central island of relatively preserved, functioning retina until late-stage disease. Here, we investigate cone...

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Veröffentlicht in:Investigative ophthalmology & visual science Jg. 64; H. 10; S. 36
Hauptverfasser: Xu, Peiluo, Jiang, Yu You, Morgan, Jessica I. W.
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States The Association for Research in Vision and Ophthalmology 28.07.2023
Schlagworte:
Choroideremia - diagnosis
Choroideremia - genetics
Humans
Ophthalmoscopy - methods
Retina
Retina - anatomy & histology
Retinal Cone Photoreceptor Cells
Retinal Pigment Epithelium
Tomography, Optical Coherence - methods
ISSN:1552-5783, 0146-0404, 1552-5783
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Zusammenfassung:Choroideremia (CHM) is an X-linked inherited retinal degeneration causing loss of the photoreceptors, retinal pigment epithelium, and choriocapillaris, although patients typically retain a central island of relatively preserved, functioning retina until late-stage disease. Here, we investigate cone photoreceptor morphology within the retained retinal island by examining cone inner segment area, density, circularity, and intercone space. Using a custom-built, multimodal adaptive optics scanning light ophthalmoscope, nonconfocal split-detection images of the photoreceptor mosaic were collected at 1°, 2°, and 4° temporal to the fovea from 13 CHM and 12 control subjects. Cone centers were manually identified, and cone borders were segmented. A custom MATLAB script was used to extract area and circularity for each cone and calculate the percentage of intercone space in each region of interest. Bound cone density was also calculated. An unbalanced two-way ANOVA and Bonferroni post hoc tests were used to assess statistical differences between the CHM and control groups and along retinal eccentricity. Cone density was lower in the CHM group than in the control group (P < 0.001) and decreased with eccentricity from the fovea (P < 0.001). CHM cone inner segments were larger in area (P < 0.001) and more circular (P = 0.042) than those of the controls. Intercone space in CHM was also higher than in the controls (P < 0.001). Cone morphology is altered in CHM compared to control, even within the centrally retained, functioning retinal area. Further studies are required to determine whether such morphology is a precursor to cone degeneration.
Bibliographie:ObjectType-Article-1
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content type line 23
ISSN:1552-5783
0146-0404
1552-5783
DOI:10.1167/iovs.64.10.36