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

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.

Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype

“…In addition, the bowel tracts showing an increased wall thickness, differently from previous reports, were mainly located in the terminal ileum and right colon.Bowel motility impairment was seen in less than 10% of CF patients and in none of the controls. This finding may be partly related to the presence of increased luminal hyperechogenic and corpusculated contents, likely to be the expression of the abundant dense compact secretions within the intestinal lumen, which are likely to affect the intestine peristalsis of these patients: this is indirectly confirmed by the presence of bowel distension with corpusculated contents in 41% of our patients.Bowel distension with thick corpusculated content and a "mass" in the left lower quadrant has been reported in association with distal intestinal obstruction syndrome, which is a common complication of CF 2,4,35. In our patients we found no associationbetween increased corpusculated contents and intestinal tracts with thickened bowel wall.…”

“…2 CFTR is expressed throughout the intestine and over the last few years there has been an increasing interest in the possible consequences of CFTR dysfunction in terms of obstruction, infection and inflammation, as it occurs in the airways. 4,5 Patients with CF may develop constipation and frank obstruction, mucus accumulation, disturbed motility, dysbiosis with small-bowel bacterial overgrowth and chronic intestinal inflammation, which may predispose to malignancy. Many of these aspects have been explored in animal models.…”

Dental utilization for Medicaid‐enrolled children with cystic fibrosis

“…As the individual with CF gets older, viscid fecal material may continue to potentially obstruct the terminal ileus and proximal colon in a condition known as distal intestinal obstruction syndrome (DIOS). Treatment includes oral rehydration in combination with laxatives (22).…”

Ivacaftor therapy for cystic fibrosis

“…Both the US Cystic Fibrosis Foundation and the European Cystic Fibrosis Society have published guidelines on a range of treatment issues [4,5]. Current treatment is targeted towards organ system manifestations that result from CFTR dysfunction and include providing pancreatic enzyme replacement therapy and a nutrient-dense diet to achieve normal growth in children and maintain normal weight in adults; to clear the lungs of dehydrated, thick secretions that promote infection and bronchiectasis; and to eradicate or suppress bacterial pulmonary infection.…”