Analysis of choroidal and retinal vasculature in inherited retinal degenerations using optical coherence tomography angiography

BACKGROUND AND OBJECTIVE: To describe the retinal vasculature and choriocapillaris, as well as the transition zone between the diseased and healthy tissue, in eyes with inherited retinal degenerations using optical coherence tomography angiography (OCTA). PATIENTS AND METHODS: Patients with inherited retinal degenerations were recruited for OCTA imaging. Retinal vasculature was assessed for increased intercapillary space and foveal avascular zone abnormalities. Choriocapillaris, retinal pigment epithelium (RPE), and photoreceptor disruption were noted, and the borders were evaluated to speculate which layers become affected first. RESULTS: Fourteen eyes of seven subjects with inherited retinal degenerations were included. All eyes (100%) demonstrated retinal thinning and increased intercapillary spaces overlying focal outer retinal changes. In all eyes, the region of choriocapillaris changes was smaller than the region of overlying RPE and photoreceptor alteration, suggesting the vascular loss was secondary. CONCLUSION: OCTA is able to provide highly detailed vascular information in eyes with inherited retinal degenerations and may be useful to better understand the pathogenesis of these diseases.