Improved surgical treatment of familial exudative vitreoretinopathy in children.

Purpose: To evaluate current surgical results in patients with familial exudative vitreoretinopathy and study the histologic findings of epiretinal membranes obtained at the time of surgery.
Methods: We performed phakic pars plana vitrectomy and membrane peeling on five patients (six eyes) with familial exudative vitreoretinopathy, who ranged in age from 18 months to 9 years and had traction retinal detachment. Membranes from two eyes (two patients) were analyzed by light and electron microscopy.
Results: The macula was reattached in all six eyes. Visual acuity improved after surgery in five of the six eyes, with two eyes improving from a preoperative visual acuity of 20/400 to a postoperative visual acuity of 20/25 and 20/60. Electron microscopic analysis of the membranes from two patients disclosed thick fibrocellular fragments with vascular elements and astrocytes.
Conclusion: With current surgical techniques, there appears to be improvement in the anatomic reattachment rate and visual outcome in patients with familial exudative vitreoretinopathy. Amblyopia, reproliferation, and vitreous hemorrhage may limit long-term improvement in vision.