Gastrointestinal surgery in cystic fibrosis: a 20-year review

J Pediatr Surg. 2014 Feb;49(2):280-3. doi: 10.1016/j.jpedsurg.2013.11.038. Epub 2013 Nov 18.


Objectives: The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF).

Methods: Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental nutrition (TP), and time to full feeds (TFF). Outcome measures for DIOS were: age of onset, number of episodes, and need for laparotomy.

Results: Seventy-five of 376 neonates presented with MI. Fifty-four (92%) required laparotomy. Contrast enema decompression was attempted in nineteen. There were no post-operative deaths. Thirty-nine (72%) neonates with MI were managed with stomas. LOS was longer in those managed with stomas (p=0.001) and in complex MI (p=0.002). Thirty-five patients were treated for DIOS. Twenty-five patients were managed with gastrograffin. Ten patients underwent surgical management of DIOS. Overall, MI did not predispose to later development of DIOS. There was a significantly greater incidence of laparotomy for DIOS in children who had MI.

Conclusion: The proportion of neonates with complex meconium ileus was high (49%) and may explain the infrequent utilisation of radiological decompression. Complex MI or management with stomas both significantly increase LOS. Re-laparotomy rate is high (22%) in MI irrespective of the type of management. DIOS is not a benign condition, particularly when the child has had previous abdominal surgery. Early referral to a surgical team is essential in these children.

Keywords: Cystic fibrosis; Distal intestinal obstruction syndrome; Meconium ileus; Surgery.

MeSH terms

  • Contrast Media / therapeutic use
  • Cystic Fibrosis / complications*
  • Diatrizoate Meglumine / therapeutic use
  • Digestive System Surgical Procedures / methods*
  • Enema
  • Female
  • Humans
  • Ileus / etiology
  • Ileus / surgery*
  • Infant, Newborn
  • Intestinal Obstruction / etiology
  • Intestinal Obstruction / surgery*
  • Length of Stay / statistics & numerical data
  • Male
  • Meconium
  • Reoperation / statistics & numerical data
  • Retrospective Studies
  • Surgical Stomas
  • Treatment Outcome


  • Contrast Media
  • Diatrizoate Meglumine