Distal intestinal obstruction syndrome

Gastrointestinal involvement may present initially as small bowel obstruction shortly after birth (known as meconium ileus) due to abnormally thick meconium that cannot be passed. Older CF patients may develop distal intestinal obstruction syndrome (DIOS), formerly called meconium ileus equivalent, which occurs due to fecal impaction in the terminal ileum and cecum. 

Intestinal obstruction may be manifested as meconium ileus, distal intestinal obstruction syndrome, or intussusception on abdominal x-ray or computed tomography scan. Rectal prolapse may be noted on physical exam. 

Treatment of distal intestinal obstruction syndrome (DIOS) consists of oral or nasogastric administration of polyethylene glycol electrolyte (PEG) solutions. Enemas may also be used to facilitate stool clearance. IV fluids are often required to correct dehydration due to vomiting or decreased oral intake. Re-evaluation of enzyme compliance and dosing is essential to prevent further episodes. Patients with recurrent symptoms may require daily PEG administration (Miralax ).5 Severe presentations of DIOS or initial meconium ileus may require surgical resection. 

Treating Meconium Ileus and Distal Intestinal Obstruction Syndrome... 

The treatment of meconium ileus or distal intestinal obstruction syndrome sometimes can be limited to the use of enemas with iso-osmolar... 

Bowel obstruction may be present at birth due to meconium ileus. Older children might present with distal intestinal obstruction syndrome (DIOS), colonic stricture(s) and, less commonly, intussusception, fecal impaction of the appendix, gastroesophageal reflux or recurrent rectal prolapse (Fig. 5.24). 

Fig. 5.27. A boy with cystic fibrosis complicated with distal intestinal obstruction syndrome. Note the dilated intestinal loops and huge fecal impaction in the terminal ileum and caecum...

Functional intestinal obstruction of the premature newborn is also called meconium ileus-like syndrome because, as in the case of meconium ileus, an obstruction exists at the distal ileum and caecum but meconium has passed. The obstruction is caused not by meconium but by stools. 

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