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Virtual Pediatric Hospital: Paediapaedia: Meconium Ileus Paediapaedia: Gastrointestinal Diseases

Meconium Ileus

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Abdominal distension, delayed onset of bilious vomiting, and failure to pass meconium. While most cases of meconium ileus are due to cystic fibrosis, only ten to twenty percent of patients with cystic fibrosis present with meconium ileus.

Etiology/Pathophysiology:
Decreased exocrine gland function and lack of digestive enzymes lead to abnormally thick meconium which fills the terminal ileum and cannot be expelled. Fifty percent of cases are complicated by prenatal volvulus, ischemic necrosis, peritonitis, or intestinal atresia.

Pathology:
Not applicable

Imaging Findings:
The classic abdominal film triad is ileal obstruction, a lack of air fluid levels because of the sticky meconium, and a "soap bubble" appearance to the right lower quadrant because of air bubbles mixed with the viscous intraluminal meconium. The enema usually demonstrates a microcolon, because it has never been used, and inspissated meconium throughout the colon and terminal ileum. It is treated by a hypertonic water soluble contrast enema that thoroughly refluxes the terminal ileum and reaches above the level of obstruction. The patient needs to be well hydrated before and after the enema. Several enemas may have to performed over several days to completely relieve the obstruction.

DDX:
Low Bowel Obstruction

References:

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