Pathology of Hereditary Juvenile Retinoschisis

The clinicopathologic data are presented in one familial case and in two other cases of juvenile retinoschisis in which the family history was not available. The principal feature in all three cases is a large retinal schisis cavity, the anterior wall of which has become a retrolental membrane that is responsible for the clinical picture of leukokoria. The schisis is seen histologically as a splitting within the nerve fiber layer. It appears impossible to state whether the changes found in the pigment epithelium of the posterior pole are secondary to the changes in the sensory retina.