Management and surgical outcomes of pediatric retinal detachment associated with familial exudative vitreoretinopathy – Our experience at a tertiary care ophthalmic center in North India

PURPOSE: To report the clinical profile, management, and long-term anatomical and visual acuity (VA) outcomes of pediatric macula-off rhegmatogenous retinal detachment (RRD) secondary to familial exudative vitreoretinopathy (FEVR). METHODS: This was a prospective, interventional study of 14 eyes of 13 children aged ≤18 years with macula-off FEVR-RRD. The primary outcomes were anatomical reattachment and VA changes. RESULTS: The mean (±SD) age of the study population was 12.14 (±3.23) years (range 6–18 years) with a male preponderance (M:F – 10:3). Of the 14 eyes, 10 underwent vitrectomy with silicone oil injection, while four underwent scleral buckling surgery. Significant improvement in VA was noted at a mean (±SD) follow-up duration of 3.32 (±1.34) years, with the mean (±SD) LogMAR VA improving from 1.42 (±0.48) (Snellen equivalent 2/60; range from 6/36 to counting finger close to face [CFCF]) to 0.6 (±0.31) (Snellen equivalent 6/24; range 6/9–6/36) (P < 0.00001) at the final visit. Successful anatomical reattachment was achieved in 13/14 eyes (92.85%). Screening of the other eye and family members was performed for FEVR and treated with laser photocoagulation when deemed necessary (7/10 contralateral eye; 12/20 siblings; 0/24 parents). CONCLUSION: To conclude, RRD may arise in eyes with FEVR at a young age and with a male predilection in Indian population. Timely surgical intervention by scleral buckling procedure or vitrectomy, based on the patient profile, can achieve excellent anatomical and VA outcomes. Careful clinical and angiographic screening of the other eye and family members is vital.