Angiographic features of pediatric stage 4 familial exudative vitreoretinopathy with radial retinal folds.

Purpose: To describe vascular anomalies and nonperfusion areas (NPAs) of stage 4 familial exudative vitreoretinopathy (FEVR) with radial retinal folds (RFs) and analyze their potential clinical significance.

Methods: Retinal detachment (RD) could exceed the RFs due to exudative, rhegmatogenous, or tractional factors, which we could call secondary RD. Fluorescein fundus angiography (FFA) and risk factors for progression to secondary RD of pediatric stage 4 FEVR patients with radial RFs were respectively explored.

Results: Fifty-eight eyes with RFs from 49 stage 4 pediatric FEVR patients were studied. Various angiographic changes were noted, including peripheral NPAs (93.1%), thinning retinal arteries (48.3%), straightened retinal vessels (34.5%), supernumerary vascular branching (32.8%), arteriovenous shunt (20.7%), aberrant circumferential vessels (13.8%), bulbous vascular endings (10.3%) and peripheral vascular dilation (6.9%). Nineteen (35.2%), 21(38.2%), and 14 (25.9%) of 58 eyes showed severe, moderate, and mild peripheral NPAs, respectively. The rate of secondary RDwas higher in eyes with severe peripheral NPAs (p = 0.004). The severity of the nonperfusion area (p = 0.040) was higher in eyes with exudation. Eight eyes (42.1%) with exudation and 12 eyes (30.8%) without exudation on color fundus pictures exhibited fluorescein leakage (p = 0.394). The rate of secondary RD was 26.3% in eyes with exudation, and 2.6% in eyes without exudation (p = 0.012).

Conclusions: The majority of stage 4 FEVR eyes with RFs have severe peripheral NPAs. Exudation is related to more severe peripheral NPAs. Exudation and NPAs are predictive factors for secondary RD.

Key Messages: What is known • Radial retinal folds are a typical clinical sign of FEVR, observed in 50.9% of FEVR patients, but little is known about the angiographic characteristics of these individuals. What is new • The severity of the nonperfusion area was graded into 3 levels. The severity of the nonperfusion area and hard exudation are risk factors for progression to total retinal detachment.