Good outcome in prune-belly syndrome despite associated severe anomalies

Pediatr Nephrol. 1988 Oct;2(4):512-4. doi: 10.1007/BF00853450.

Abstract

A boy aged 4.5 years with prune-belly syndrome and associated urethral stenosis, oligohydramnios, imperforate anus and vesicosigmoid fistula is described. In contrast to the anticipated poor prognosis, vesicostomy and divided transverse colostomy performed after birth followed by prophylaxis of infection and bicarbonate supplementation have resulted in a good outcome. The vesicosigmoid fistula might have served in utero as a "natural diversion" protecting from pressure-induced renal damage. It is suggested that the main determinant of prognosis in PBS is the presence and degree of kidney dysplasia at birth as reflected by the neonatal renal function after performance of an indicated urinary diversion procedure rather than the presence of severe associated anomalies.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Anus, Imperforate / complications
  • Child, Preschool
  • Hip Dislocation, Congenital / complications
  • Humans
  • Infant, Newborn
  • Intestinal Fistula / complications
  • Male
  • Prune Belly Syndrome / complications
  • Prune Belly Syndrome / pathology*
  • Prune Belly Syndrome / surgery
  • Urethral Stricture / complications
  • Urinary Bladder Fistula / complications