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Introduction

Duodenal atresia is a congenital defect in which a segment of the duodenal lumen is completely obstructed.  Patients have feeding intolerance and bilious (most common) or nonbilious emesis in the first 1-2 days of life.

Pathophysiology and risk factors

Normally, during weeks 6-7 of gestation, there is a brief period in which portions of the fetal intestinal lumen are occluded due to epithelial proliferation.  This is followed by recanalization during weeks 8-10.  Failure of recanalization can cause intestinal atresia anywhere along the gastrointestinal (GI) tract.  In the duodenum, the atretic segment is typically distal to the second part of the duodenum ( Figure 1), where the common bile duct enters the intestine at the major duodenal papilla ( Figure 2).

Duodenal atresia can occur in isolation in up to 50% of all cases.  It may also occur in association with other conditions, including:

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