Reticular Dystrophy of the Retinal Pigment Epithelium

Reticular dystrophy of the retinal pigment epithelium was found in a Dutch girl and her brother. The brother developed this dystrophy during infancy. No reports of this peculiar dystrophy are known to us, since H. Sjögren described it in a Swedish family for the first time in 1950. This is the first time that an extensive functional investigation of the retina, as well as fundus photography and fluorescein angiography, is performed in an affected individual. Recessive degenerative deafmutism, spherophakia, and diaphanous irides, which were found in Sjögren's family, were not discovered in the family presented here.