Review Question - QID 105427

Cystic fibrosis (CF) commonly presents with meconium ileus, characterized by bilious vomiting, distended loops of bowel on radiography, and failure to pass meconium.

CF is an autosomal recessive disease and is common in Caucasians. Neonates with CF commonly present with failure to thrive, respiratory compromise, and meconium ileus. In meconium ileus, abnormally thick meconium results in bowel obstruction, perforation, or volvulus. CF is diagnosed by the sweat chloride test or genetic testing. Radiography in ileus can demonstrate distended loops of bowel from the obstruction.

Figure A shows dilated loops of bowel on abdominal radiograph characteristic of meconium ileus.

Incorrect Answers:
Answer 2: Hirschsprung disease would present with abdominal pain with chronic constipation. There would be an absence of stool in the rectal vault on rectal exam and a positive squirt sign on physical exam (expulsion of stool/flatus on rectal exam).

Answer 3: Jejunal atresia is characterized by the "triple bubble" sign on abdominal radiographs as well as bilious vomiting.

Answer 4: Pyloric stenosis presents with non-bilious and projectile vomiting with a palpable mass on abdominal exam. This diagnosis can be confirmed with an ultrasound. The patient should first be rehydrated and have their electrolytes repleted prior to imaging.

Answer 3: Tracheoesophageal fistula presents with coughing and choking with feeding. This breastfeeding and asymptomatic child in terms of his respiratory status suggests against this.

Bullet Summary:
Cystic fibrosis presents with meconium ileus and distended bowel on radiography.