Characterisation of vascular changes in different stages of Stargardt disease using double swept-source optical coherence tomography angiography

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 (p Conclusions Patients with STGD reveal vascular changes in the retina and CC in all disease stages. The avascular zone in the SCP/DCP and areas with rarefied flow signal in the ORCC/CC increase with the duration and stage of disease, indicating progressive vascular decay most likely secondary to retinal pigment epithelium and neuronal loss. Furthermore, increased vascular damage is associated with decreased vision.