Macular capillary dropout in familial exudative vitreoretinopathy and its relationship with visual acuity and disease progression.

PURPOSE:To quantitatively detect the macular microvascular alterations of eyes with familial exudative vitreoretinopathy (FEVR) and analyze their associations with the severity and visual acuity of FEVR. METHODS:A case-control study comprising 62 patients (62 eyes) with FEVR and 21 age-matched healthy individuals (21 eyes) with normal vision was conducted. Parafoveal vascular density (VD) was measured using optical coherence tomography angiography. Visual acuity, intraocular pressure, and axial length were recorded. RESULTS:Parafoveal VD of eyes with FEVR was lower than that of the controls (P < 0.05). Parafoveal VD decreased with increasing FEVR stages (P < 0.05), and decreased VD in superficial capillary plexus (SCP) was independently correlated with FEVR severity (odds ratio: 1.558, P < 0.001) after controlling for other confounding variables. Vascular density in eyes with FEVR and decreased visual acuity was lower than eyes with FEVR and normal visual acuity (SCP, P < 0.001; deep capillary plexus, P = 0.001). Moreover, VD loss had independent association with visual loss in FEVR (SCP: odds ratio: 0.817, P = 0.019; deep capillary plexus: odds ratio: 0.763, P = 0.016). CONCLUSION:There may be parafoveal microvascular defects in FEVR and that VD loss in SCP may be correlated with the severity of FEVR. In addition, VD loss in SCP and deep capillary plexus may be associated with the visual loss in FEVR.