Updated: 12/17/2019

Duodenal Atresia

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Topic
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Evidence
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Snapshot
  • AXRA 45-year-old women 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
  • Etiology unknown but thought to occur during weeks 8-10 of development
  • Results in stenosis of the duodenal passageway between the stomach and intestinal tract
    • leads to gastric outlet syndrome
  • Associated with Down syndrome (20-30% of DS cases) and other birth defects
    • as with biliary atresia
  • Epidemiology
    • > 1:10,000 births
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
Evaluation
  • Diagnosis based primarily on
  • 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
Treatment
  • 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
Prognosis, Prevention, and Complications
  • Prognosis
    • full recovery is expected after surgical correction
  • Prevention
    • no preventive measures are available at this time
  • Complications
    • without treatment, condition is lethal
 

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