Duodenal atresia is a congenital malformation in which the duodenum does has a blind end, and so is not patent. This can result in an obstruction-type picture presenting clinically.

Conditions associated with duodenal atresia

• Approximately 25% of cases are associated with Down's syndrome
• Other intestinal atresias
• VACTERL association

Presentation

• Antenatally, it may be associated with polyhydramnios as the baby cannot ingest amniotic fluid properly
• Distended abdomen
• Vomiting may be bilious or non-bilious depending on the site of the atresia

Investigations

• Abdominal X-rays have a characteristic 'double bubble'
  • one gas bubble visible in the stomach, and one gas bubble visible in the most proximal (patent) part of the duodenum prior to the atresia
  • occurs due to air from the stomach being between the pyloric sphincter and the blind end of the duodenum

Management

• Surgical repair. Duodenoduodenostomy involves reconnecting the closed proximal and distal segments of the duodenum in order to relieve the obstruction.