Updated: 12/25/2021

Duodenal Atresia

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  • Snapshot
    • A 45-year-old woman with polyhydramnios delivers a male infant with Down syndrome. Shortly after birth, the infant vomits greenish-yellow fluid. Imaging reveals two distinct pockets of air in the abdominal region.
  • Introduction
    • Congenital failure of the duodenal lumen to completely recanalize
    • Results in stenosis of the duodenal passageway between the stomach and intestinal tract
      • leads to gastric outlet syndrome
  • Epidemiology
    • > 1:10,000 births
  • ETIOLOGY
    • Pathogenesis
      • Unknown but thought to occur during weeks 8-10 of development
    • Associated with Down syndrome (20-30% of DS cases) and other birth defects
      • as with biliary atresia
  • Presentation
    • Symptoms
      • bilious vomiting that continues despite no feedings
        • as opposed to nonbilious emesis in pyloric stenosis
      • urinary infrequency
    • Physical exam
      • abdominal distention may or may not be present
      • absent meconium
  • imaging
    • Radiograph
      • reveals "double bubble" sign
      • Differential diagnosis for "dobule bubble" includes other causes of duodenal obstruction
        • duodenal atresia, duodenal web or stenosis, annular pancreas, malrotation of the bowel with a midgut volvulus , or Ladd's bands
    • Fetal ultrasound
      • may show polyhydramnios
  • Differential
    • Pyloric stenosis, tracheoesophageal fistula
  • diagnosis
    • Diagnosis based primarily on imaging
  • Treatment
    • Prevention
      • no preventive measures are available at this time
    • Medical management
      • gastric decompression
        • place G-tube to deflate stomach
      • IV hydration and electrolyte control
        • indicated in all patients
    • Surgical intervention
      • surgical correction
        • not an emergency
        • approach depends on anatomic variant
  • Complications
    • Without treatment, condition is lethal
  • Prognosis
    • Full recovery is expected after surgical correction

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