A 3-day-old neonate with Down syndrome presents with bilious vomiting and failure to pass meconium. Abdominal X-ray shows a 'double bubble' sign with absence of distal gas. What is the most likely diagnosis?

• A Malrotation with volvulus
• B Jejunal atresia
• C Hirschsprung disease
• D Duodenal atresia ✓

Explanation

Duodenal atresia presents with bilious vomiting and the 'double bubble' sign on X-ray, caused by a markedly dilated stomach and proximal duodenum with no distal gas. It is strongly associated with Down syndrome (trisomy 21), which is present in approximately 30 percent of cases. Malrotation with volvulus shows distal gas and is a surgical emergency.

Reference: Pediatric Surgery, 7th, Coran ed.

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