Characteristics of Retinal Breaks and Surgical Outcomes in Rhegmatogenous Retinal Detachment in Familial Exudative Vitreoretinopathy

Abstract

To determine the characteristics of retinal breaks and surgical outcomes in eyes with a rhegmatogenous retinal detachment (RRD) with familial exudative vitreoretinopathy (FEVR). Retrospective, noncomparative case series. Thirty-seven patients (46 eyes) with a RRD in FEVR. The medical records were reviewed and the types, directions, and positions of the retinal breaks and surgical outcomes were analyzed. Fundus examinations, including ophthalmoscopy, fluorescein angiography, and RetCam imaging (Natus Medical Incorporated, Pleasanton, CA). The retinal breaks were identified as tears in 12 eyes, atrophic holes in 24 eyes, tears and atrophic holes in 2 eyes, dialysis-related in 1 eye, a retinal break in the ora serrata in 1 eye, and unidentified in 6 eyes. Most retinal breaks (86.1%) were identified only in the temporal retina. Most tears (85.7%) were observed on the demarcation line, whereas atrophic holes were identified both on the demarcation line (53.8%) and the avascular retina (42.3%). The representative tears were almond-shaped, which differs from the typical horseshoe-shaped tears. Scleral buckling was performed as the initial surgery in 37 eyes and resulted in reattachment in 35 eyes (94.6%). Vitrectomy with or without scleral buckle was performed for eyes with more complex RRD in FEVR and resulted in reattachment in 5 of 9 eyes (55.6%). In total, reattachment was achieved in 40 of 46 eyes (87.0%). There was a history of stage 1A or 2A FEVR in 45 eyes and a history of stage 2B FEVR in 1 eye. Our data clarified the types, directions, and positions of the retinal breaks and the effectiveness of scleral buckling as the first surgical choice for treating RRDs in FEVR.