Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 May;90(5):681-686.
doi: 10.1111/ans.15357. Epub 2019 Jul 30.

Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis

Amy Hort  1 , Ahmer Hameed  1   2 , Peter G Middleton  3   4 , Henry C Pleass  1   2   3
Affiliations
Review

Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis

Amy Hort et al. ANZ J Surg. 2020 May.

Abstract

As life expectancy for those with cystic fibrosis (CF) now exceeds 40 years of age, adult hospitals away from specialized CF services are being exposed more frequently to people with acute complications of CF. Well-known manifestations of CF include pulmonary disease and pancreatic insufficiency with malabsorption. However, a less well-known entity is distal intestinal obstruction syndrome (DIOS), which is an important cause of obstructive symptoms in people with CF that must be differentiated from other causes of bowel obstruction. However, one confounding factor is that adults with CF may have elements of both DIOS and mechanical bowel obstruction due to adhesions from previous operations. A recent tragic outcome in a young adult with CF highlights the need for all doctors, both junior and senior, especially those who are not directly involved in day-to-day CF care, to understand the features of DIOS and the appropriate management, which differs from that of a mechanical bowel obstruction. This review aims to highlight the clinical and pathophysiological features of DIOS, differentiate it from other causes of bowel obstruction and contrast management strategies. Improved knowledge of DIOS will help to facilitate appropriate recognition and permit optimal, multidisciplinary management of this CF complication.

Keywords: cystic fibrosis complication; distal intestinal obstruction syndrome; meconium ileus equivalent.

PubMed Disclaimer

References

    1. Jennings MT, Riekert KA, Boyle MP. Update on key emerging challenges in cystic fibrosis. Med. Princ. Pract. 2014; 23: 393-402.
    1. Reid DW, Blizzard CL, Shugg DM, Flowers C, Cash C, Greville HM. Changes in cystic fibrosis mortality in Australia, 1979-2005. Med. J. Aust. 2011; 195: 392-5.
    1. Munck A, Alberti C, Colombo C et al. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome. J. Cyst. Fibros. 2016; 15: 531-9.
    1. Bell SC, Bye PT, Cooper PJ et al. Cystic fibrosis in Australia, 2009: results from a data registry. Med. J. Aust. 2011; 195: 396-400.
    1. Houwen RH, van der Doef HP, Sermet I et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J. Pediatr. Gastroenterol. Nutr. 2010; 50: 38-42.