June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Visualization of the Photoreceptor / RPE / Choriocapillaris Complex in Choroideremia Using Adaptive Optics Enhanced Indocyanine Green Imaging
Author Affiliations & Notes
  • Nancy Aguilera
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Tao Liu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Joanne Li
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Andrew J. Bower
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Rongwen Lu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • John Giannini
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Brian Patrick Brooks
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Wadih M Zein
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Laryssa Huryn
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Johnny Tam
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Nancy Aguilera None; Tao Liu None; Joanne Li None; Andrew Bower None; Rongwen Lu None; John Giannini None; Brian Brooks None; Wadih Zein None; Laryssa Huryn None; Johnny Tam None
  • Footnotes
    Support  Intramural Research Program of the National Eye Institute, National Institutes of Health; Alcon Research Institute
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 393 – F0431. doi:
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      Nancy Aguilera, Tao Liu, Joanne Li, Andrew J. Bower, Rongwen Lu, John Giannini, Brian Patrick Brooks, Wadih M Zein, Laryssa Huryn, Johnny Tam; Visualization of the Photoreceptor / RPE / Choriocapillaris Complex in Choroideremia Using Adaptive Optics Enhanced Indocyanine Green Imaging. Invest. Ophthalmol. Vis. Sci. 2022;63(7):393 – F0431.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Adaptive optics enhanced indocyanine green (AO-ICG) imaging can be used to image the photoreceptor (PR) - retinal pigment epithelium (RPE) - choriocapillaris (CC) complex in the living human eye (PMID 30456310). In this study, we applied this technique to evaluate the PR-RPE-CC complex in choroideremia, an X-linked retinal degeneration.

Methods : AO-ICG imaging was performed at the fovea in 6 female carriers and 3 affected male patients (n=16 eyes) with confirmed mutations in the CHM gene. A subset of patients (3 carriers, 1 affected male, n=7 eyes) were imaged a second time, approximately 1-2 years after the initial visit. The transit phase immediately after ICG dye injection was used to obtain an image of the CC, and the late phase (at least 30 minutes afterward) was used to image the RPE. Images of the cone PRs were simultaneously obtained. Images were co-registered and quantified for cell-to-cell spacing (PR, RPE) or average flow void diameter (CC) were quantified using custom software.

Results : The PR-RPE-CC complex was intact in all patients imaged. Quantitative analysis of the initial visit of each eye revealed that the RPE was the most disrupted layer (average cone spacing increase compared to the expected normal value, 7%; RPE spacing increase, 156%; CC flow void diameter increase, 14%). Longitudinal follow-up imaging further corroborated this result, with the largest visit-to-visit changes occurring in the RPE when compared to the cone PR and CC layers (average increase from visit 1 to visit 2: cone PR, 1%; RPE, 24%; CC, 4%). Together, these results suggest that the PR, RPE and CC degenerate at different rates, with the largest changes occurring in the RPE.

Conclusions : We demonstrate, for the first time, longitudinal assessment of the PR-RPE-CC complex using AO-ICG. In choroideremia, the RPE appears to be affected to a greater degree than the surrounding cone PR and CC layers and shows the largest change over time.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

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