Meconium Ileus

In 1020% of infants born with cystic fibrosis, the thick mucous secretions of the small bowel
produce obstruction by inspissated meconium. This usually occurs in the terminal ileum.
Although there is no clear correlation between pancreatic insufficiency and the development of
inspissated meconium, meconium ileus also occurs in patients with pancreatic duct obstruction
and pancreatic aplasia. Meconium obstruction with no apparent cause has also been described in
newborn infants.

Clinical Findings

Symptoms and Signs

The infant typically has a normal birth weight and very distended abdomen. No meconium is
passed, and bilious emesis occurs early. Loops of thick, distended bowel may be seen and
palpated.

Imaging Studies

Plain abdominal radiographs show loops of bowel that vary greatly in diameter; the thick
meconium gives a ground-glass appearance. Air mixed with the meconium produces the "soap
bubble" sign, which is usually located in the right lower quadrant. Radiographs taken shortly
after the infant has been placed in an upright position may fail to show air-fluid levels because
the thick, viscid meconium fails to layer out rapidly. Contrast enema will show microcolon with
rare meconium flecks. Reflux of contrast medium through the ileocecal valve demonstrates a
small terminal ileum containing "pellets" of inspissated mucus; more proximally, the bowel is
progressively distended with packed meconium. Antenatal perforation may be detected by the
presence of abdominal calcifications, since the meconium becomes saponified.

Complications

Meconium ileus may be complicated by a segmental (not midgut) volvulus due to the heavy,
distended loops of distal ileum. If this occurs early in fetal life, the volvulus may progress to
gangrene of the affected bowel segment. This can heal completely, with abdominal calcifications
as the only manifestation that it occurred. Conversely, it may heal in such a way that an intestinal
atresia is formed. Perforation late in gestation may lead to meconium peritonitis or a large
meconium pseudocyst at birth.

Other common complications of meconium ileus are related to the almost universal presence of
cystic fibrosis. These infants are susceptible to repeated pulmonary infection with chronic
bronchopneumonia, bronchiectasis, atelectasis, and lung abscess. Malabsorption due to
pancreatic insufficiency requires pancreatic enzyme replacement. Rectal prolapse and
intussusception may be produced by strained passage of inspissated stools. Nasal polyps and
chronic sinusitis are frequent. Biliary cirrhosis and bleeding varices from portal hypertension are
late manifestations of bile duct obstruction by mucus.
Treatment & Prognosis

Nonoperative treatment is successful in 6070% of cases. A nasogastric tube should be inserted

and connected to suction. A contrast enema can be both diagnostic and therapeutic. It should be
performed with a slightly hypertonic water-soluble contrast agent (never barium). The addition
of N-acetylcysteine, which is mucolytic, may be necessary to disperse the meconium in
uncomplicated cases. The infant must be well hydrated, and intravenous fluids must be continued
during and after the procedure in order to prevent hypovolemia from the effects of the hypertonic
contrast solution. If this treatment fails to relieve the obstruction, laparotomy is indicated. The
ileum is opened and, if possible, flushed clear. The bowel can be reanastomosed or brought out
as a double-barrel stoma. Alternatively, a T-tube may be placed in the bowel and brought out of
the anterior abdominal wall for postoperative irrigations. Compromised intestine is resected, and
appendectomy is performed because of the high rate of appendicitis in patients with cystic
fibrosis.

All patients should be evaluated for cystic fibrosis. Pancreatic enzyme replacement may be
required. A formula low in long-chain fatty acids and high in medium-chain triglycerides may
give better absorption and growth than standard formulas. The patient must be placed in an
environment with high humidity to keep tracheobronchial secretions fluid. Postural drainage with
cupping of the chest should be taught to the parents so that they will continue to maintain
tracheobronchial toilet indefinitely. Older children and adolescents may develop a meconium
ileuslike syndrome termed distal ileal obstruction syndrome. This is ileal obstruction due to
inspissated stool. It can occur when patients are not compliant with their medications or become
dehydrated. Most often, it is successfully treated with hypertonic contrast enemas.

Mak GZ et al: T-tube ileostomy for meconium ileus: four decades of experience. J Pediatr Surg
2000;35:349. [PMID: 10693694]

Ziegler MM: Meconium ileus. Curr Probl Surg 1994;31:731. [PMID: 8062591]