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 treat ment for these gravely ill patients. Of the 34 patients with prune bel ly syndrome evaluated at our institutions, 6 had urethral atresia (3 m ales and 3 females). Two of these patients died as neonates as a resul t of pulmonary insufficiency and 1 was stillborn. The common feature o f the 3 surviving patients (2 males and 1 female) was the presence of a vesicocutaneous fistula. As neonates, 2 suffered from pulmonary insu fficiency due to oligohydramnios. The mean length of follow-up for the se 3 patients was 37 months (range 13-58). The urinary tract was decom pressed 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 pat ient, 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 we ll. In one of the boys, urethral atresia was initially managed by peri neal urethrostomy and then by reconstructive surgery. Progressive cath eter dilation was used on the other boy. Urethral atresia occurred in 18% of our patients with PBS; the incidence was equal in males and fem ales. Survival correlated with the development of a spontaneous vesico cutaneous fistula. Two-thirds of the survivors developed end-stage ren al failure.