Familial Exsudative Vitreoretinopathy

Familial Exudative Vitreoretinopathy (FEVR) is a disease affecting the retinal blood vessels, characterized by the failure of retinal capillaries to develop during the last few months of intrauterine life. It is a hereditary disease, with a high level of penetrance, but it varies in its expression. There are minor, asymptomatic forms corresponding to discrete peripheral vascular abnormalities, and more evolved forms with vitreo-retinal fibrovascular proliferation. The most frequent complication is retinal detachment. In the serious forms, there is complete disorganization of the eyeball, with neovascular glaucoma or even phthisis of the eyeball (reduction in the volume of the eye). The diagnosis is based on an examination of the fundus and fluorescein angiography. Treatment of minor forms is based on laser photocoagulation. The most severe forms of the condition require surgical treatment.