Atrophy Expansion Rates in Stargardt Disease Using Ultra-Widefield Fundus Autofluorescence

• DOI: 10.1016/j.xops.2021.100005
• Publication Date: 2021-03-07T21:08:47Z
• Authors: Rachael C. Heath ...; Jennifer A. Thompson; Johnny Lo; Tina M. Lamey; Terri L. McLaren; Ian L. McAllister; David A. Mackey; Ian J. Constable; John N. De Roach; Fred K. Chen
• Keywords: Stargardt disease; ABCA4; Autofluorescence

PurposeTo investigate atrophy expansion rate (ER) using ultra-widefield (UWF) fundus autofluorescence (FAF) in Stargardt disease (STGD1).DesignRetrospective, longitudinal study.ParticipantsPatients with biallelic ABCA4 mutations who were evaluated UWF FAF and Heidelberg 30° × 55° imaging.MethodsPatients secondary to STGD1 classified into genotype groups: group A, severe or null-like variants early-onset disease; B, 1 intermediate variant trans variant; C, mild late-onset disease. The boundaries of definitely decreased (DDAF) outlined manually areas (in square millimeters) recorded at baseline follow-up. Bland-Altman analysis was conducted examine agreement between observers devices. Linear mixed modeling used evaluate predictors ER DDAF area root (SRA).Main Outcome MeasuresPatient ocular SRA included age onset, duration symptoms, group, visual acuity, size.ResultsA total 138 eyes from 69 patients (33 men [47%]; mean ± standard deviation, 41 20 years; range, 10–83 years) carrying 61 unique recruited. Ultra-widefield measurements equivalent imaging. Baseline the only significant predictor (P < 0.001). Age for ER. Definitely ranged 4.65 mm2/year (group A) 0.62 C).ConclusionsUltra-widefield is a feasible reliable method assessing STGD1. value mutation severity predicting warrants further investigation. To (STGD1). Retrospective, study. Patients (SRA). Patient size. A C).