Familial exudative vitreoretinopathy

Two sisters and a brother with the syndrome of familial exudative vitreoretinopathy are described (FEV). Findings associated with this condition include temporal neovascularization, vitreous hemorrhage, and retinal detachment. Familial exudative vitreoretinopathy is a genetic syndrome with nearly complete penetrance but widely variable expressivity. The differential diagnosis is fairly extensive. This condition is sometimes confused with retrolental fibroplasia, and FEV is probably a more frequently occurring disease than previously thought, because of confusion with RLF. The pathophysiologic mechanism, natural history, and optimal treatment of this disease are matters of debate. With active surgical management, three of the four eyes which presented to us for treatment have visual acuity of 20/30 or better.