A 1-day-old child with trisomy 21 born after 32 weeks’ gestation presents with bilious vomiting. Prenatal ultrasound was significant for polyhydramnios.
An abdominal x-ray shows a dilated stomach, a dilated first part of the duodenum (double bubble), and absence of air beyond the second air bubble.
Duodenal atresia results from failure to recanalize the lumen of the duodenum after the solid phase of embryologic development. Between 38% and 55% of patients with duodenal atresia have another associated significant congenital anomaly: approximately 30% of cases are associated with Down syndrome, and 23%-34% of cases are associated with isolated cardiac defects. Esophageal atresia may be present in 7%-12% of patients. Other gastrointestinal anomalies include malrotation, anorectal anomalies, intestinal atresias, cloacal anomalies, annular pancreas, and renal anomalies. Duodenal atresia is associated with prematurity and low birth weight. Duodenal atresia is prenatally detected in 32%-57% of patients, and fetal ultrasound may show polyhydramnios.
Patients present with vomiting, which can be bilious or nonbilious. The abdominal x-ray shows a “double bubble.” Once diagnosed, patients are given nothing by mouth, a nasogastric tube is inserted, and intravenous fluids are started.
Decompress the stomach.
Use preoxygenation and intravenous atropine 10 μg/kg.
Use either awake intubation or rapid-sequence IV induction with endotracheal intubation.
Maintain anesthesia with sevoflurane or narcotics and muscle relaxants.
Fluid replacement for the third space and insensible fluid loss is needed in addition to the maintenance fluid requirements.
Postoperative complications are reported in 14%-18% of patients. Possible indications for reoperation include anastomotic leak, functional duodenal obstruction, adhesions, and missed atresias.
The patients are given nothing by mouth until bowel sounds are heard, stool is passed, and the gastric drainage is limited (<1 mL/kg/h of clear or pale-green fluid). This may take 7-10 days, but it can be prolonged in the premature infant with other significant anomalies and may require intravenous total parental nutrition.
The operative management of duodenal atresia is determined by the anatomic findings and associated anomalies noted upon laparotomy. Bypass procedures for duodenal atresia or stenosis include duodenoduodenostomy and duodenojejunostomy.
Current survival rates for infants with duodenal atresia or stenosis are 90%-95%. Higher mortality rates are associated with prematurity and multiple congenital abnormalities.