Distal intestinal obstruction syndrome (DIOS) involves obstruction of the distal part of the small intestines by thickened intestinal content and occurs in about 20% of mainly adult individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to constipation.
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Signs and symptoms
Signs and symptoms of DIOS include crampy abdominal pain, vomiting, and a palpable mass in the abdomen. X-rays of the abdomen may reveal stool in the colon and air-fluid levels in the small intestines.
Management
Differentiation of DIOS from constipation is generally performed by unit specialising in the treatment of cystic fibrosis.
Optimisation of enzyme supplementation, diet and adequate hydration and laxatives are essential for treatment and prevention of DIOS. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement, hydration and laxatives.
Oral contrast instillation into the colon/ileum under radiological control has been found to reduce the need for surgical intervention.