Journal of Cystic Fibrosis
Volume 10, Supplement 2 , Pages S24-S28, June 2011

Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients

  • Carla Colombo

      Affiliations

    • Cystic Fibrosis Center, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinica, University of Milan, Italy
    • Corresponding Author InformationCorresponding author: Carla Colombo, MD, Associate Professor of Pediatrics, University of Milan, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Cystic Fibrosis Center, Via Commenda 9, 20122 Milano, Italy. Tel.: +39 02 5503 2456; Fax: +39 02 5503 2814
    • ,
  • Helmut Ellemunter

      Affiliations

    • Cystic Fibrosis Center, Department of Pediatrics, Medical University Innsbruck, Innsbruck, Austria
    • ,
  • Roderick Houwen

      Affiliations

    • Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands
    • ,
  • Anne Munck

      Affiliations

    • CF Center, Department of Pediatric Gastroenterology and Nutrition, University Hospital Robert Debre, AP-HP, Paris, France
    • ,
  • Chris Taylor

      Affiliations

    • Academic Unit Child Health, Sheffield Children's Hospital, Sheffield, UK
    • ,
  • Michael Wilschanski

      Affiliations

    • Pediatric Gastroenterology, Hadassah University Hospitals, Hebrew University, Jerusalem, Israel
    • ,
  • on behalf of the ECFS

Abstract 

Complete or incomplete intestinal obstruction by viscid faecal material in the terminal ileum and proximal colon – distal intestinal obstruction syndrome (DIOS) – is a common complication in cystic fibrosis. Estimates of prevalence range from 5 to 12 episodes per 1000 patients per year in children, with higher rates reported in adults. DIOS is mainly seen in patients with pancreatic insufficiency, positive history of meconium ileus and previous episodes of DIOS. DIOS is being described with increasing frequency following organ transplantation. Diagnosis is based on suggestive symptoms with a right lower quadrant mass confirmed on X-ray. The main differential is chronic constipation. Treatment consists of rehydration combined with stool softening laxatives or gut lavage with balanced electrolyte solutions. Rapid fluid shifts have been described following osmotic agents. Avoiding dehydration and optimizing pancreatic enzyme dosage may reduce the chance of further episodes. Prophylactic laxative therapy is widely used, but is not evidence-based.

Keywords:  Cystic fibrosis , Intestinal obstruction , Diagnosis , Treatment

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PII: S1569-1993(11)60005-2

doi:10.1016/S1569-1993(11)60005-2

Journal of Cystic Fibrosis
Volume 10, Supplement 2 , Pages S24-S28, June 2011

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