NORRIES DISEASE IN NORTH AMERICA

Norrie's disease is a congenital or infantile, bilateral, hemorrhagic retinal detachment which is transmitted as a sex-linked recessive. Many of the affected children are poor of hearing, some are mentally retarded. Two affected families are reported from North America. They encompass three and four generations respectively. Nineteen affected males are described clinically and six eyes could be studied histologically. The salient features of the histologie picture are: A complete retinal detachment, retinal hemorrhages and edema, a vascularized preretinal membrane and secondary degenerative change. These features are identical with those found in the usual Coats' disease. © 1969 Dr. W. Junk N.V.