Duodenal atresia

Background

  • During weeks 6 and 7 of gestation, the GI tract becomes occluded then recanalizes during weeks 8 to 10
  • Duodenal atresia is thought to result from failure of recanalization
  • Often associated with other malformations such as biliary atresia or gallbladder agenesis
  • May also be associated with cardiac, renal, or vertebral abnormalities
  • About a quarter of patients born with duodenal atresia have Downs syndrome

Clinical Features

Presentation is very early in the postnatal period

  • Abdominal distention and bilious emesis within first 24 hours of birth
    • Abdomen often markedly distended, with visible or palpable loops of bowel
    • NG tube aspirate >20 mL
  • Signs of dehydration (e.g. dry mucous membranes, poor skin turgor, and sunken fontanelle)
  • +/- Signs of other congenital anomalies

Differential Diagnosis

Nausea and vomiting (newborn)

Newborn '
Obstructive intestinal anomalies Esophageal stenosis/atresia, pyloric stenosis, intestinal stenosis/atresia, malrotation ± volvulus, incarcerated hernia, meconium ileus/plug, Hirschsprung disease, imperforate anus, enteric duplications, duodenal atresia
Neurologic Intracranial bleed/mass, hydrocephalus, cerebral edema, kernicterus
Renal Urinary tract infection, obstructive uropathy, renal insufficiency
Infectious Viral illness, gastroenteritis, meningitis, sepsis
Metabolic/endocrine Inborn errors of metabolism (urea cycle, amino/organic acid, carbohydrate), congenital adrenal hyperplasia
Miscellaneous Ileus, gastroesophageal reflux, necrotizing enterocolitis, milk allergy, GI perforation

Evaluation

  • Imaging
    • AP, lateral, and cross table XR should be obtained on all infants with concern for obstruction
    • Classic double bubble sign due to dilation of the stomach and proximal duodenum
    • Absent distal gas

Management

Disposition

  • Admission

References