Macular Microvascular Findings in Familial Exudative Vitreoretinopathy on Optical Coherence Tomography Angiography.

Background And Objective: To describe depth-resolved macular microvasculature abnormalities in patients with familial exudative vitreoretinopathy (FEVR) using optical coherence tomography angiography (OCTA).

Patients And Methods: Twenty-two eyes (11 eyes of six patients with FEVR and 11 control eyes) were imaged with OCTA. Graders qualitatively analyzed the OCTA images of the superficial and deep vascular complexes for abnormal vascular features and compared to fluorescein angiography (FA).

Results: Seven of 11 eyes with FEVR displayed abnormal macular vascular findings. Abnormalities in the superficial vascular complex included dilation, disorganization, straightening, heterogeneous vessel density, and curls/loops. In the deep vascular complex, abnormalities included areas of decreased density, disorganization, curls/loops, and "end bulbs." Except for dragging and straightening of the vessels, none of these macular features were visible on FA.

Conclusion: OCTA revealed marked macular abnormalities in eyes with FEVR that have not been previously observed with FA alone, suggesting this is more than a disease of the retinal periphery and involves macular and deep retinal vasculature abnormalities. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:322-329.].