Purpose: To evaluate the outcome for vitreoretinal surgery in children with familial exudative vitreoretinopathy (FEVR) and to evaluate the risk factors associated with failure.
Methods: This is a retrospective interventional case series of 43 consecutive eyes (34 patients) with vitreoretinal surgery for FEVR. Ocular status prior to intervention and at last follow-up and all surgical steps were recorded. Follow-up time was at least 6 months. Main outcome measure was surgical failure (defined as one of the following: (1) deterioration of visual acuity and stage, (2) persistence or development of total retinal detachment, (3) phthisis).
Results: After a mean follow-up of 3.3 ± 3.4 years (median 2.3; 0.5-15.7 years), surgery was successful in 30 eyes (70%) and failed in 13 eyes (30%). Twenty-one eyes (49%) improved, 13 (30%) remained stable, and 9 (21%) deteriorated. Postoperatively, stages and VA improved significantly (p = 0.001; p = 0.04, respectively). Surgical failure was only observed on patients with stages 4 and 5. Mean macular thickness decreased significantly in eyes (stages 2 and 3) with tractional epiretinal membrane.
Conclusion: Eyes with tractional epiretinal membrane in stages 2 and 3 seem to benefit from vitrectomy and membrane peeling with a positive risk-benefit profile. Advanced stages have a low success rate and limited functional improvement, but in selected cases, surgery seems beneficial.
Keywords: Familial exudative vitreoretinopathy; Pediatric vitreoretinal surgery; Retinal detachment; Vitreoretinal surgery.