Urethral atresia and the prune belly syndrome. Report of 6 cases.

An association between urethral atresia and the prune belly syndrome (PBS) has been recognised, but few reports discuss the outcome of treatment for these gravely ill patients. Of the 34 patients with prune belly syndrome evaluated at our institutions, 6 had urethral atresia (3 males and 3 females). Two of these patients died as neonates as a result of pulmonary insufficiency and 1 was stillborn. The common feature of the 3 surviving patients (2 males and 1 female) was the presence of a vesicocutaneous fistula. As neonates, 2 suffered from pulmonary insufficiency due to oligohydramnios. The mean length of follow-up for these 3 patients was 37 months (range 13-58). The urinary tract was decompressed in 2 patients by a formal vesicostomy performed early in life. The surviving girl has normal renal function following reconstructive surgery. Because both the bladder and urethra were absent in this patient, an ileocaecal bladder substitute and an appendiceal urethra were constructed. The 2 surviving boys both have renal insufficiency. One has received a renal transplant from a living relative and is doing well. In one of the boys, urethral atresia was initially managed by perineal urethrostomy and then by reconstructive surgery. Progressive catheter dilation was used on the other boy. Urethral atresia occurred in 18% of our patients with PBS; the incidence was equal in males and females. Survival correlated with the development of a spontaneous vesicocutaneous fistula. Two-thirds of the survivors developed end-stage renal failure.