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Distal intestinal obstruction sydrome

Diagnosis

DIOS affects about 10% of children with CF. It usually presents with abdominal pain and a firm, sometimes tender, mass in the right iliac fossa/ The differential diagnosis for abdominal pain and/or vomiting includes adhesional obstruction, intussusception or appendicitis/appendix mass. Abdominal X-ray can help with the diagnosis (bubbly granular appearance in RIF). Most of the time the obstruction is not complete and DIOS can be managed in an outpatient setting.

Treatment

All children admitted to hospital for the management of DIOS should be discussed with the CF team in Oxford who will talk to the paediatric surgical team. This is especially important if the child has had previous abdominal surgery (as neonate or since) as adhesions may be contributing to the bowel obstruction. If there is complete obstruction (bile stained vomited, distended loops on AXR, constipation), urgent surgical opinion should be sought.

Outpatient treatment

For mild symptoms, treat as outpatient with Movicol 1-2 sachets/day - increased upto 6 sachets per days for a short period if necessary. Children not responding to movicol who have persitent pain or vomiting should be admitted to hospital.

Inpatient treatment

Initial inpatient management is likely to include oral movicol 2 sachets 2-3 times a day or oral gastrogaffin. For children with incomplete obstruction (ie not vomiting) not responding to movicol or gastrograffin, bowel lavage plus/minus gastrograffin enema should be considered.

Oral gastrograffin

Oral gastrograffin	<8 years 50ml in 150ml juice
	>8 years 100ml in 200ml juice

It is important to encourage drinking to prevent osmotic dehydration as fluid is drawn into the bowel. Children can eat normally. The dose may be repeated every 12 to 18 hours.

Bowel lavage
For bowel lavage, an iv line should be sited so that iv fluids can be given if dehydration occurs. Use Movicol, Kleen-prep or Go-LYTELY. This will nearly always require a nasogastric tube. If at any stage of the lavage there are signs of complete obstruction (increasing vomiting, abdominal distension), the lavage should be stopped and the NG tube left on drainage. Consider repeat AXR, contrast enema, surgical opinion.

Start bowel lavage early in the morning and continue until the stools are yellow, watery and free of solid matter (3 litres is usually enough, although more may be needed in larger children - MAX dose is 8 litres). Children should can have clear fluids to drink, but no solids. If success is not achieved in 12 hours, stop, give a light evening meal and resume the following morning. Electrolyte disturbance is rare and it is not necessary to check blood electrolytes. In children with CFRD close monitoring of blood glucose will be necessary.

Complete obstruction
Patients with evidence of complete obstruction (vomiting, abdominal distension) caused by DIOS should be managed with the surgical team. These children will need to stop oral fluids, have a nasogastric tube and most likely have a gastrograffin enema.