Familial Exudative Vitreoretinopathy

Familial exudative vitreoretinopathy (FEVR) is a hereditary retinal vascular anomaly characterized by incomplete peripheral retinal vascularization during development. The vascular abnormalities exhibit varying degrees of severity: from minor changes in peripheral retinal vessels to severe retinal detachments leading to blindness. FEVR has been shown to have autosomal dominant, autosomal recessive, and X-linked recessive inheritance patterns, with numerous genes implicated in the condition. Wide-field fluorescein angiography is essential for early detection, diagnosis, and management. Laser photocoagulation is used to treat sequelae from the avascular retina, and retinal detachments are managed with vitreoretinal surgery. Visual prognosis depends on the severity of the disease, and lifelong follow-up is recommended.