Kovacs et al. (1997) described 2 consecutive mid-trimester fetuses of different sexes with identical anomalies of the upper limbs and the kidneys in association with severe dilatation of the lateral cerebral ventricles. On the parents' decision, both pregnancies ... Kovacs et al. (1997) described 2 consecutive mid-trimester fetuses of different sexes with identical anomalies of the upper limbs and the kidneys in association with severe dilatation of the lateral cerebral ventricles. On the parents' decision, both pregnancies were terminated. In the first fetus, an ectopic horseshoe kidney fused at the upper poles was found in the pelvis. In the second fetus, no kidney was found on the right side; a normal kidney on the left side had two ureters, one of which coursed to the right, crossed the midline at the level of the upper sacrum, and reached the bladder at the usual site on the right. Microscopy showed that a significant proportion of the glomeruli had a dilated Bowman capsule, and there were also tubuli with cystic dilatation in the cortex and the medulla. There was absence of the left umbilical artery. In both fetuses, autopsies confirmed a marked ventriculomegaly. Both fetuses had anomalies of the upper limbs: the first had forearms that were short and bent anteriorly with only 4 fingers on each hand, and absence of the radius and thumb bilaterally by x-ray. The second had curved forearms with flexing of the hands medially in an acute angle. Radiographs showed bilateral absence of the radius and thumb. See also 219730 for a disorder characterized by ventriculomegaly and cystic kidneys without limb anomalies.