Bilateral Retinal Vascular Hypoplasia Associated With Persistence of the Primary Vitreous: A New Clinical Entity?

Three infant boys with a bilateral congenital retinopathy characterized by retinal vascular hypoplasia and persistence of the primary vitreous are described. The infants were healthy and had no non-ocular physical abnormalities. None had histories of prematurity or low birth weight. Family histories were negative to similar retinopathy, and the fundi of all examined blood relatives were normal. The described congenital retinopathy is compared and contrasted with persistent hyperplastic primary vitreous, Norrie's disease, idiopathic retinal dysplasia, familial exudative vitreoretinopathy, retinopathy of prematurity, and incontinentia pigmenti.