Duodenal atresia

Duodenal atresia is a congenital intestinal obstruction in which the duodenum (the first part of the small intestine, just beyond the stomach) is completely blocked or absent, preventing the passage of food from the stomach into the rest of the intestinal tract. It results from a failure of the duodenal lumen to recanalize during embryonic development, typically between the 8th and 10th weeks of gestation. The condition is also referred to as congenital duodenal obstruction or duodenal stenosis when the blockage is partial. Duodenal atresia primarily affects the gastrointestinal system and presents in the newborn period with symptoms including bilious or non-bilious vomiting (depending on whether the obstruction is above or below the ampulla of Vater), abdominal distension of the upper abdomen, and failure to pass meconium. Prenatal ultrasound often reveals polyhydramnios (excess amniotic fluid) and the characteristic 'double bubble' sign, which represents a dilated stomach and proximal duodenum. Duodenal atresia is strongly associated with Down syndrome (trisomy 21), with approximately 20–30% of affected infants having this chromosomal abnormality. Other associated anomalies may include congenital heart defects, malrotation of the intestine, annular pancreas, esophageal atresia, and renal anomalies. The condition may occur as an isolated finding or as part of the VACTERL association (vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb anomalies). Treatment for duodenal atresia is surgical and is typically performed shortly after birth. The standard procedure is a duodenoduodenostomy, in which the surgeon connects the two ends of the duodenum to bypass the obstruction and restore intestinal continuity. Preoperative management includes nasogastric decompression and intravenous fluid resuscitation. With modern surgical techniques and neonatal intensive care, the prognosis is generally excellent, with survival rates exceeding 90%. Long-term outcomes depend largely on the presence and severity of associated anomalies, particularly cardiac defects and chromosomal abnormalities.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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