Surgical outcomes of familial exudative vitreoretinopathy-associated retinal detachment: a systematic review and meta-analysis.

BACKGROUND: Familial exudative vitreoretinopathy (FEVR) is a rare hereditary vitreoretinal disorder in children, characterized by abnormal peripheral retinal vascularization leading to retinal detachment (RD) in 21% to 64% of cases. Optimal surgical management remains uncertain owing to disease rarity and heterogeneity of published evidence. PURPOSE: To evaluate anatomical and functional outcomes of surgical repair for FEVR-associated RD. METHODS: This PRISMA-adherent systematic review and meta-analysis was prospectively registered in PROSPERO (CRD420251071825). Eligible designs included retrospective and prospective cohort studies and case series reporting surgical outcomes in FEVR-associated RD with more than five patients. Pre- and postoperative best-corrected visual acuity (BCVA; logMAR) was pooled using random-effects models. Retinal reattachment rates were synthesized as proportions. Subgroup analyses were performed by detachment type (rhegmatogenous (RRD) versus tractional (TRD)) and surgical approach (pars plana vitrectomy (PPV), scleral buckling (SB), combined PPV + SB). Heterogeneity was quantified using I² and 95% prediction intervals. Formal meta-regression was not performed, given insufficient study counts per subgroup. RESULTS: Twenty studies comprising 586 eyes were analyzed. Overall surgery yielded a significant BCVA improvement (MD -0.30 logMAR; 95% CI -0.50 to -0.08; p = .009) with substantial heterogeneity (I² = 86%; prediction interval − 1.03 to 0.45). RRD demonstrated greater functional benefit (MD -0.45 logMAR; 95% CI -0.72 to -0.16) and high anatomical success (88.8%; I² = 0%). TRD showed no significant visual improvement (MD -0.15; p = .38) and lower, more variable reattachment (72.8%; I² = 63.3%). SB achieved the highest reattachment rate (93.2%), combined PPV + SB the most consistent gains (84.0%; I² = 0%), and PPV alone the lowest reattachment (63.0%), reflecting its preferential use in complex TRD. FEVR staging was inconsistently reported, precluding stage-stratified analysis. CONCLUSION: Surgical repair of FEVR-associated RD yields meaningful outcomes, particularly in RRD, while TRD goals are centered on vision preservation. Findings are limited by retrospective designs, substantial heterogeneity, and inconsistent staging data. Prospective multicenter registries with standardized reporting are urgently needed.