International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome

J Cyst Fibros. 2016 Jul;15(4):531-9. doi: 10.1016/j.jcf.2016.02.002. Epub 2016 Feb 23.


Background: Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis.

Methods: A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults.

Results: 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs. 2%, p=0.02). Complete DIOS entailed longer hospitalisation (4 [3; 7] days vs. 3 [1; 4], p=0.002). Delayed arrival at hospital and prior weight loss had a significant impact on the time needed for DIOS resolution. Associated CF co-morbidities for DIOS included meconium ileus (40% vs. 18%, p<0.0001), exocrine pancreatic insufficiency (92% vs. 84%, p=0.03), liver disease (22% vs. 12%, p=0.004), diabetes mellitus (49% vs. 25%, p=0.0003), and Pseudomonas aeruginosa (68% vs. 52%, p=0.01); low fibre intake and insufficient hydration were frequently observed. Female gender was associated with recurrent DIOS (75% vs. 52%, p=0.04), constipation with incomplete episodes (39% vs. 11%, p=0.03), and poor patient compliance in taking pancreatic enzyme therapy during complete episodes (25% vs. 3%, p=0.02).

Conclusion: DIOS is a multifactorial condition having a similar incidence in children and adults. We show that delayed arrival at hospital after the initial symptoms causes significant morbidity. Early recognition and treatment would improve the prognosis.

Keywords: Abdominal pain; Cystic fibrosis; Distal intestinal obstruction syndrome; Incidence.

Publication types

  • Multicenter Study
  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Causality
  • Child, Preschool
  • Conservative Treatment* / methods
  • Conservative Treatment* / statistics & numerical data
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / epidemiology
  • Digestive System Surgical Procedures* / methods
  • Digestive System Surgical Procedures* / statistics & numerical data
  • Europe / epidemiology
  • Female
  • Hospitalization / statistics & numerical data
  • Humans
  • Incidence
  • Infant, Newborn
  • Intestinal Obstruction* / diagnosis
  • Intestinal Obstruction* / epidemiology
  • Intestinal Obstruction* / etiology
  • Intestinal Obstruction* / therapy
  • Longitudinal Studies
  • Male
  • Prognosis
  • Prospective Studies
  • Risk Factors
  • Severity of Illness Index