Good long-term results with comprehensive surgical management of prune belly syndrome

Last Updated: 2015-07-03

By Will Boggs MD

NEW YORK (Reuters Health) - Comprehensive surgical management yields good long-term results in children with prune belly syndrome, though reoperations are common, researchers from Brazil report.

"Despite the somewhat increased rate of reoperations for complications, the overall result concerning renal function in our group of operated patients is better than in most series with conservative treatment," Dr. Francisco Tibor Dénes from University of São Paulo Medical School told Reuters Health by email.

Prune belly syndrome (PBS), which has an incidence between 1:35,000 and 1:50,000 live births, usually presents with abdominal wall flaccidity, urological abnormalities and cryptorchidism.

While management of these children is controversial, Dr. Dénes's team prefers comprehensive surgical management due to the difficulty of long-term continuous surveillance resulting from social and geographical conditions in Brazil.

They present long-term results of this approach in 46 children with PBS online May 6 in the Journal of Pediatric Urology.

The median age at surgery was 16 months (range, 25 days to 10 years), and the children were followed for a median 143 months (range, 15 months to 26 years).

Treatment included 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions.

About a quarter of the children had surgical-related complications, and 17 of 46 (37%) required reoperations. There were no deaths.

All children showed improvements in abdominal appearance, though four required secondary abdominoplasties to achieve it, and 24 of 40 children who required upper urinary tract reconstruction showed significant anatomical improvement in most renal units. Thirty-five of these children had normal renal function after surgery, two developed clinically managed chronic renal failure, and three ultimately were submitted for renal transplantation due to end-stage renal disease.

More than three-quarters of the 42 children who required lower urinary tract reconstruction were continent after surgery and adequate bladder emptying was possible for 72% of those who were tested.

Ultimately, 85% of testes were normal in size and adequately located in the scrotum after orchiopexy, and all pubertal children developed normal secondary sexual characteristics.

"PBS is a condition that requires urologic care," Dr. Dénes said. "Orchiopexy is required in all patients. Whenever urinary tract reconstruction and/or abdominoplasty is also necessary, we think that these three procedures can be performed concomitantly, and as our data shows, with good results."

Dr. Dénes added, "We vehemently object to pregnancy termination when a diagnosis of PBS is made during fetal life, as most patients who have the syndrome, particularly those who undergo the comprehensive surgery, are able to live normal lives with stable renal function and voiding habits, improved body image, and normal sexual function. We are now in the process of evaluating and trying to improve their fertility in adulthood."

SOURCE: http://bit.ly/1f4Va0s

J Pediatr Urol 2015.