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Prognostication in Stargardt Disease Using Fundus Autofluorescence

Malena Daich VarelaYannik LaichShaima Awadh HashemOmar A. MahrooAndrew R. WebsterMichel Michaelides

出版	Universität, 2023
URL	http://books.google.com.hk/books?id=oppg0AEACAAJ&hl=&source=gbs_api

註釋Abstract: Purpose
To explore fundus autofluorescence (FAF) imaging as an alternative to electroretinography as a noninvasive, quick, and readily interpretable method to predict disease progression in Stargardt disease (STGD).

Design
Retrospective case series of patients who attended Moorfields Eye Hospital (London, United Kingdom).

Participants
Patients with STGD who met the following criteria were included: (1) biallelic disease-causing variants in ABCA4, (2) electroretinography testing performed in house with an unequivocal electroretinography group classification, and (3) ultrawidefield (UWF) FAF imaging performed up to 2 years before or after the electroretinography.

Methods
Patients were divided into 3 electroretinography groups based on retinal function and 3 FAF groups according to the extent of hypoautofluorescence and retinal background appearance. Fundus autofluorescence images of 30° and 55° were reviewed subsequently.

Main Outcome Measures
Electroretinography and FAF concordance and its association with baseline visual acuity (VA) and genetics.

Results
Two hundred thirty-four patients were included in the cohort. One hundred seventy patients (73%) were in electroretinography and FAF groups of the same severity, 33 (14%) were in a milder FAF than electroretinography group, and 31 (13%) were in a more severe FAF than electroretinography group. Children 10 years of age (n = 23) showed the lowest electroretinography and FAF concordance at 57% (9 of the 10 with discordant electroretinography and FAF showed milder FAF than electroretinography), and adults with adult onset showed the highest (80%). In 97% and 98% of patients, 30° and 55° FAF imaging, respectively, matched with the group defined by UWF FAF.brbrConclusions