The Characteristic of Optical Coherence Tomography Angiography and Retinal Arteries Angle in Familial Exudative Vitreoretinopathy with Inner Retinal Layer Persistence

PurposeTo compare the angle of retinal arteries and macular vessel density and foveal avascular zone (FAZ) in early stage familial exudative vitreoretinopathy (FEVR) patients with inner retinal layer (IRL) persistence with FEVR patients without IRL persistence and normal people.MethodsThis study enrolled 113 early stage FEVR patients and 55 age-matched normal subjects. FEVR patients were divided into IRL group and non-IRL group based on the presence or absence of IRL in fovea. The angle of superior temporal and inferior temporal branch retinal arteries on ultra-wide-field fundus images were measured. Superficial and deep vessel density of whole image, fovea and parafovea, the area and perimeter of FAZ, A-circularity index (AI, perimeter/standard circle perimeter with equal area) and vessel density around the 300-mu m width of the FAZ (FD), central macular thickness (CMT) on 3 mm x 3mm OCTA were measured.Results30 FEVR patients in IRL group, 83 FEVR patients in non-IRL group, 55 normal people in control group were evaluated. BCVA were worst in IRL group (p < .001). The angle of retinal arteries was smaller in FEVR groups (p < .001) and were smallest in IRL group (p < .001). Superficial and deep vessel density of whole and parafovea area in FEVR patients were significantly lower than that in normal people (p < .05), AI were biggest (p = .01) and FD were smallest in IRL group (p < .001). CMT in IRL group were thicker than non-IRL group and control group (p < .05).ConclusionWorse BCVA, smaller angle of retinal arteries (more vessels traction), lower macular vessel density, smaller and more irregular FAZ and thicker CMT were observed in FEVR patients with IRL persistence even in early stage.