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  • September 10, 2021
  • 2021 Abstracts

Characterization of choroidal and choriocapillaris abnormalities in Alport Syndrome

Author: Maria Vittoria Cicinelli (Italy)

Co-authors: Maria Vittoria Cicinelli, Markus Ritter, Hassan Tausif, Lee M. Jampol, Manjot Gill

Purpose

To analyze the characteristics of the choriocapillaris and the choroid in patients with Alport syndrome (AS), in particular choriocapillaris flow deficits (FD) density and choroidal vascularity index (CVI), and to investigate their clinical and demographic associations.

Setting/Venue

Multi-center, cross-sectional study held in the Department of Ophthalmology at Northwestern University (Chicago, Illinois) and the Department of Ophthalmology of the Medical University of Vienna (Vienna, Austria).

Methods

Twenty-one patients with AS and 18 healthy controls consecutively enrolled. Demographics and past medical history data were collected for each participant. Each eye underwent a complete ophthalmic examination, 3x3 swept-source optical coherence tomography angiography (PLEX® Elite 9000 2.0, Carl Zeiss Meditec, Dublin, CA), and macular spectral-domain OCT (Spectralis HRA2; Heidelberg Engineering, Heidelberg, Germany). FD were segmented with the Phansalkar method using a radius of 4 pixels. The CVI was calculated as the ratio between the luminal choroidal area and the total choroidal area. Factors influencing FD density and CVI in AS patients were explored with multivariable linear mixed models.

Results

Choriocapillaris FD were more numerous (p=0.02) and smaller in size (p=0.01) in patients with AS compared to controls. Patients with history of kidney transplant had larger FD mean area (p=0.04), larger total FD area (p=0.008) and higher FD density (p=0.005) than AS patients never transplanted. The CVI in AS eyes was similar to controls, irrespective of history of kidney transplant. The FD density was higher with older age (estimate= 0.41 for each year) and in patients with history of kidney transplant (estimate= 8.57 with positive history). The CVI was lower in eyes with dot maculopathy (estimate= -3.62 if present) and anterior lenticonus (estimate= -6.69 if present).

Conlusions

The quantitative evaluation of the choriocapillaris may be used as a clinical biomarker of kidney involvement in Alport syndrome. Lower choroidal vascularity was found in concomitance of other ocular structural abnormalities.

Financial Disclosure

No financial disclosure

Comments

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