Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis. Issue 5 (30th July 2019)

• Record Type: Journal Article
• Authors: Hort, Amy; Hameed, Ahmer; Middleton, Peter G.; Pleass, Henry C.
• Is Part Of: ANZ journal of surgery. Volume 90:Issue 5(2020)
• Page Start: 681
• Page End: 686
• Publication Date: 2019-07-30
• Subjects: cystic fibrosis complication -- distal intestinal obstruction syndrome -- meconium ileus equivalent; Surgery -- Periodicals; 617.005
• DOI: 10.1111/ans.15357
• Languages: English
• ISSNs: 1445-1433

Abstract

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. Abstract : Distal intestinal obstruction syndrome (DIOS) is an important cause of obstructive symptoms in people with cystic fibrosis that must be differentiated from other causes of 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 cystic fibrosis complication.