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 Down 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

• Malrotation with volvulus
• Hirschsprung's disease
• Meconium ileus
• Other intestinal atresia

Nausea and vomiting (newborn)

Newborn	'
Obstructive intestinal anomalies	Esophageal stenosis/atresia Pyloric stenosis Intestinal stenosis/atresia Duodenal atresia Malrotation ± volvulus Incarcerated hernia Meconium ileus/plug Hirschsprung's disease Imperforate anus Enteric duplications
Neurologic	Intracranial bleed/mass Hydrocephalus Cerebral edema Kernicterus
Renal	Urinary tract infection Obstructive uropathy Renal insufficiency
Infectious	Viral illness Acute gastroenteritis Meningitis Sepsis
Metabolic/endocrine	Inborn errors of metabolism Adrenal insufficiency Congenital adrenal hyperplasia
Miscellaneous	Ileus Gastroesophageal reflux disease Necrotizing enterocolitis Milk allergy, FPIES GI perforation

Evaluation

Double Bubble sign

• 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

• NPO
• NG tube to suction
• Correct fluid and electrolyte abnormalities
• Ampicillin and gentamicin (to prevent post-op infection)
• Surgery

Disposition

• Admission