Reuss et al. (1989) described a family in which in 2 consanguineous relationships a Cape Verdean man fathered 2 normal children and 6 infants or fetuses with hydrocephaly and cystic disease of the corticomedullary areas of the kidneys. ... Reuss et al. (1989) described a family in which in 2 consanguineous relationships a Cape Verdean man fathered 2 normal children and 6 infants or fetuses with hydrocephaly and cystic disease of the corticomedullary areas of the kidneys. All 6 affected infants were detected prenatally. The first was found at 32 weeks' gestation to have ventriculomegaly, normal-sized but echodense kidneys, and increased amniotic fluid, and was born stillborn at 32 weeks' gestation. The 5 other fetuses were found to be affected at 18 to 20 weeks' gestation, and were terminated during pregnancy. Amniocentesis during pregnancy in 5 of the 6 fetuses showed increased alpha-fetoprotein and acetylcholinesterase (AChE) levels. All had ventriculomegaly and normal-sized but echodense kidneys. Histologic studies of the kidneys showed cystic tubular dilatation in the corticomedullary area and renal medulla; the cysts contained eosinophilic amorphous proteinaceous material. One fetus had postaxial polydactyly, but no other limb anomalies were noted in any of the fetuses. The families lived in the Netherlands (ten Kate, 1991). See also 602200 for a disorder characterized by ventriculomegaly and defects of the radius and kidney.