Correlating Changes in the Macular Microvasculature and Capillary Network to Peripheral Vascular Pathology in Familial Exudative Vitreoretinopathy

Abstract Purpose To evaluate the macular microvasculature in patients with familial exudative vitreoretinopathy (FEVR) using optical coherence tomography angiography (OCTA) and assess for peripheral vascular changes using widefield fluorescein angiography (WFA). Design Multicenter, retrospective, comparative, observational case series. Participants We identified 411 patients with FEVR, examined between September 2014 and June 2018. Fifty-seven patients with FEVR and 60 healthy controls had OCTA images of sufficient quality for analysis. Methods Custom software was used to assess for layer-specific, quantitative changes in vascular density and morphology on OCTA by way of: vessel density (VD), skeletal density (SD), fractal dimension (FD), vessel diameter index (VDI), and foveal avascular zone (FAZ). WFA images were reviewed for peripheral vascular changes including capillary dropout, late-phase angiographic posterior and peripheral vascular leakage (LAPPEL), vascular dragging, venous-venous shunts, and arterio-venous shunts. Main Outcome Measures Macular microvascular parameters on OCTA and peripheral angiographic findings on WFA. Results OCTA analysis of 117 patients (187 eyes; 92 FEVR, 95 controls) demonstrated significantly reduced VD, SD and FD, and greater VDI in patients with FEVR compared to controls, in the non-segmented retina, superficial retinal layer (SRL), and deep retinal layer (DRL). The FAZ was larger compared to control eyes in the DRL (p Conclusion Patients with FEVR demonstrate abnormalities in the macular microvasculature and capillary network, in addition to the peripheral retina. The macular microvascular parameters on OCTA may serve as biomarkers of changes in the retinal periphery on WFA.