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BOOK TITLE: Retina: Medical and Surgical Management
Familial exudative vitreoretinopathy (FEVR) is a rare hereditary disorder characterized by incomplete and aberrant retinal angiogenesis. The most prominent feature of FEVR is the peripheral retinal avascularity as explained previously by the defective retinal angiogenesis. The severity of the disease due to the phenotypic variability may be mild to advance. Norries disease is one of the differential diagnoses of FEVR. It is considered as a severe form of FEVR and may be associated with systemic features. Treatment is strategized based on the stage of FEVR. Stage 1 requires regular monitoring and follow-up. In stage 2 (neovascularization), after confirming the leakage with fluorescein angiography (FA), laser photocoagulation of the capillary nonperfusion areas is done. Cryotherapy is useful in treating neovascularization, when the media is hazy or in cases with non-dilating pupil. Screening of asymptomatic family members helps in early detection and to provide genetic counseling. The ultrawide-field fluorescein angiography is a fast growing screening tool, which guides in identifying peripheral vascular changes at an earlier stage.