Distal intestinal obstruction syndrome

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Classification according to ICD-10
E84.1 Cystic fibrosis with bowel manifestations - including: distal intestinal obstruction syndrome

Meconium ileus in cystic fibrosis

P75 * Meconium ileus in cystic fibrosis
ICD-10 online (WHO version 2019)

The distal intestinal obstruction syndrome (DIOS, also meconium ileus equivalent syndrome) is a complication in patients with cystic fibrosis , in which hardened, thickened feces in the transition area from the small to the large intestine lead to constipation .

The clinical picture corresponds to the meconium ileus of the newborn, but only occurs at a later age, even in adults, with colic-like abdominal cramps and is considered an emergency .

distribution

The frequency is given as around 10 to 20% of cystic fibrosis patients. It occurs more frequently with increasing age, in around 80% the syndrome first appears in adulthood. The recurrence rate is around 50%.

Clinical manifestations

Clinical criteria are:

  • colicky abdominal cramps
  • Patients with known cystic fibrosis
  • increasingly also patients with additionally known pancreatic insufficiency

Diagnosis and treatment

In addition to an X-ray of the abdomen overview and sonography , enemas (initially colonic contrast enema ) are used both diagnostically and therapeutically. In addition, infusions and drinking an electrolyte solution , possibly through a stomach tube , are indicated. Surgical intervention may be required.

Differential diagnostics

Must be distinguished are adhesions small bowel obstruction, acute appendicitis or intussusception .

literature

  • M. Mavilia: Distal intestinal obstructive syndrome (DIOS): a gastrointestinal complication of cystic fibrosis in adults. In: Clinical journal of gastroenterology. [Electronic publication before going to press] April 2019, doi: 10.1007 / s12328-019-00979-7 , PMID 30963407 .
  • J. Green, W. Carroll, FJ Gilchrist: Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. In: The Cochrane database of systematic reviews. Volume 8, 08 2018, S. CD012798, doi: 10.1002 / 14651858.CD012798.pub2 , PMID 30075058 , PMC 6513219 (free full text) (review).

Individual evidence

  1. a b c Entry on Distales_intestinales_Obstruktionssyndrom in Flexikon , a Wiki of the DocCheck company
  2. a b c Orphanet emergency cystic fibrosis
  3. a b W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. 2nd edition, Springer 1996, ISBN 3-540-60224-0 , p. 112, 12.2.3
  4. X. Dray, T. Bienvenu, N. Desmazes-Dufeu, D. Dusser, P. Marteau, D. Hubert: Distal intestinal obstruction syndrome in adults with cystic fibrosis. In: Clinical Gastroenterology and Hepatology. Volume 2, Number 6, June 2004, pp. 498-503, PMID 15181619 .
  5. ^ A b Treatment of Distal Intestinal Obstruction Syndrome