Distal Intestinal Obstruction Syndrome and Severe Constipation, Children with Cystic Fibrosis

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Scope

Management of Distal Intestinal Obstruction Syndrome (DIOS) and severe constipation in children with cystic fibrosis RHC CF Unit, Glasgow.

Introduction

Patients with Cystic Fibrosis usually require to take pancreatic enzyme supplements with all foods in order to digest fat and protein in their diet. Even with optimal pancreatic enzyme replacement therapy, only up to 85% of all ingested fat may be successfully absorbed. Occasionally, stool fat content will increase over time, resulting in stool accumulation –in the ascending colon/ caecum region presenting as DIOS, or throughout the colon presenting as severe constipation.  

DIOS or severe constipation?

“In DIOS, there is an acute onset of symptoms versus a gradual onset of symptoms in constipation. In clinical practice, the differential diagnosis between impending DIOS and severe constipation may not always be possible, although an abdominal radiograph may clarify the diagnosis by showing faecal accumulation throughout the colon in constipation. However, this might not be an important issue, as the initial approach to treatment, .. is similar.” 1

Symptoms and signs

Patients may present with abdominal pain and, with DIOS, may have a palpable right sided faecal mass on abdominal examination. With severe constipation, faecal loading may be palpable throughout the colon. 

Following abdominal X-Ray a diagnosis of Distal Intestinal Obstruction Syndrome (DIOS) may be made where faecal loading is predominantly in the right colon area. Widespread faecal loading with less acute onset of symptoms is associated with chronic / severe constipation. 

  • If impending or acute DIOS, or severe constipation, is diagnosed the patient should be admitted to hospital for management
  • Symptoms of acute intestinal obstruction are rare however if the patient has severe abdominal pain or vomiting a surgical opinion should be sought at time of presentation.
Investigations

Abdominal X-Ray

  • This may reveal faecal loading in all areas or predominantly in the right colon.
  • Total intestinal obstruction is associated with the presence of fluid levels.

Depending on abdominal X-Ray findings, the following treatment plan will apply where DIOS / severe constipation is diagnosed.

Further management

CF Dietitians may arrange for stool to be sent for Faecal Fat analysis and will also review fibre and fluid intake. 

On discharge, Movicol Paediatric may be prescribed:

 

Child 1–11 months

0.5–1 sachet daily.

Child 1 year

1 sachet daily, adjust dose to produce regular soft stools; maximum 4 sachets per day.

Child 2–5 years

  • sachet daily, adjust dose to produce regular soft stools; maximum 4 sachets per day.

Child 6–11 years

  • sachets daily, adjust dose to produce regular soft stools; maximum 4 sachets per day.

 

References

1. Carla Colombo, Helmut Ellemunter, Roderick Houwen, Anne Munck, Chris Taylor, Michael Wilschanski on behalf of the ECFS. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. Journal of Cystic Fibrosis, Volume 10 Suppl 2 (2011) S24–S28. 

 

Editorial Information

Last reviewed: 21 August 2018

Next review: 21 August 2020

Author(s): Dr Jane Wilkinson, Dr Rachel Tayler, Julie Crocker, Ms Nicola Brindley

Version: 1

Approved By: Paediatric Drugs & Therapeutics Committee