[Exudative retinopathy with dominant transmission. Report of a new pedigree]

A family with the syndrome of familial exudative vitreoretinopathy is described. A 7-year-old presented a non-perfused, avascular periphery in the temporal retina. The father is blind and his fundus was described in childhood as showing vitreoretinal traction, retinal telangectasia and temporal fibrotic changes. A cousin of our patient, a 5-year-old girl, was seen with vitreoretinal traction of the posterior pole. Familial exudative vitreoretinopathy is sometimes confused with retrolental fibroplasia. The family history and a fluorescein angiographic study of non-affected members are useful for confirmation of this diagnosis: pathophysiologic mechanisms and optimal treatment of the disease still remain about discussion.