Characterisation of Vascular Changes in Different Stages of Stargardt Disease Using Double Swept-source Optical Coherence Tomography Angiography

Abstract: Objective: To describe vascular changes in different stages of Stargardt disease (STGD) via double swept-source optical coherence tomography angiography. Methods and analysis: Prospective, cross-sectional case-control study. Twenty-three patients (45 eyes) with ABCA4 mutations graded according to the Fishman STGD classification and 23 controls (23 eyes) were included. Two independent investigators quantified the foveal avascular zone (FAZ) in the superficial and deep capillary plexus (SCP/DCP) and the areas presenting rarefied flow and complete vascular atrophy in the outer retina to choriocapillaris (ORCC) and choriocapillaris (CC) slab. Results: The mean age at first diagnosis of STGD was 24.0 years (range 9-50) and 37.9 years (range 18-74) at the time of examination. Eleven patients were assigned to the Fishman STGD classification stage (S) 1, three to S2, eight to S3 and one to S4. The FAZ in SCP and DCP was increased in all stages compared with controls (p0.01). Areas with rarefied flow signal and vascular atrophy were detected in the ORCC and the CC layer and grew with increasing stage of disease (p0.01). The duration of disease correlated with the extent of the enlarged FAZ in the SCP/DCP and with the area of reduced flow in the ORCC and CC layer (p