[Retinal dysplasia with hydrocephalus (author's transl)]

A Study is described of a family in which four children of the same generation died during early childhood; three of them had an obvious hydrocephalus. Two of the latter could be examined clinically, and their eyes removed for pathology. One of them presented clinically bilateral leucocoria; histology showed a total detachment of a dysplastic retina, with absence of development of the vitreous. The other child had apparently normal eyes. Yet on microscopical examination there was a discrete retinal dysplasia without retinal detachment in both eyes, and a persistent hyaloid artery, in one. This association of hydrocephalus with retinal dysplasia is clinically and genetically different from other types of retinal dysplasia.