Renal anomalies and agenesis associated with total intestinal aganglionosis

Four patients with total aganglionosis of the intestine had renal abnormalities. Two patients had cortical subcapsular cysts, one had unilateral renal agenesis and one other had unilateral renal dysplasia. The consistency of renal anomalies with total aganglionosis suggests the presence of a common genetic origin.