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Dominant exudative vitreoretinopathy (DEVR) is a rare ocular condition involving the vitreous and retina. Syneresis and liquefaction of the vitreous, preretinal membranes, and delicate white opacities represent the most common changes in the vitreous. Alterations in the retina include heterotopia of the macula, retinal neovascularization, subretinal and intraretinal exudation, and localized retinal detachment. Abrupt termination of the retinal vasculature in the temporal equatorial zone and nonperfusion of the peripheral retina are the most common findings and can be demonstrated by fundus photography and fluorescein angiography. Although DEVR bears a resemblance to the retinopathy of prematurity, it has an autosomal dominant pattern of inheritance and is not associated with premature birth or postnatal oxygen treatment. Myopia is common in both diseases.
This monograph is divided into three parts. Part 1 includes a brief description of the normal development and the anatomy of the peripheral retinal vasculature. Disturbances in the development of