Prune-belly syndrome with anorectal malformation

Eur J Pediatr Surg. 2004 Oct;14(5):351-4. doi: 10.1055/s-2004-817961.

Abstract

Associated abnormalities of other organ systems result in considerable long-term morbidity in patients of prune-belly syndrome (PBS). The gastrointestinal anomalies commonly observed with PBS are persistence of the embryonic wide mesentery and absence of fixation to the posterior abdominal wall, whereas anorectal malformations (ARM) are rarely associated with PBS. We describe one such case of PBS associated with high ARM and urethral stenosis in a one-day-old male newborn. The neonate underwent preliminary sigmoid colostomy for ARM, but he subsequently developed necrotizing gastritis and gastric perforation in the postoperative period and had to undergo partial gastrectomy and vesicostomy. The neonate developed fatal septicemia after second surgery. A review of all the reported cases of this association is presented and the dilemmas in the management of these cases are discussed.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anal Canal / abnormalities*
  • Fatal Outcome
  • Gastritis / complications
  • Gastritis / pathology
  • Humans
  • Infant, Newborn
  • Male
  • Necrosis
  • Prune Belly Syndrome* / complications
  • Prune Belly Syndrome* / surgery
  • Rectum / abnormalities*