Surgical Management of a Neurofibromatosis Type 1 Associated Retinal Detachment

A 6-year-old boy with neurofibromatosis type 1 (NF1) presented with a total retinal detachment (RD) in the left eye (A, B). The mid-peripheral and peripheral retina were mostly avascular. Immature vessels were confined to the posterior retina and preretinal new vessels induced a posterior retinal tear (C). He underwent pars plana vitrectomy with scleral buckling and silicone oil tamponade resulting in successful reattachment of the retina. After silicone oil removal (D), visual acuity was 1/10. Severe anomalies of the peripheral retinal vascularization exist in NF1 and can result in both rhegmatogenous and tractional RD.