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Esophageal atresia

Last updated: January 7, 2021


Esophageal atresia is a congenital defect in which the upper esophagus is not connected to the lower esophagus, ending blindly instead. It is caused by the abnormal development of the tracheoesophageal septum. Esophageal atresia with a fistula connected distally to the trachea is the most common kind of esophageal malformation (classified as Gross type C). It manifests immediately after birth with cyanotic attacks, foaming at the mouth, and coughing, and prevents any attempts to pass a feeding tube into the stomach. X‑ray is mandatory for classifying the atresia and should show an air‑filled pouch situated at the level of the third thoracic vertebra. Infants with suspected esophageal atresia cannot be fed orally because of the risk of aspiration pneumonia. Curative surgery must, therefore, be performed within the first 24 hours after birth.


  • Incidence: approx. 1:4,300 live births in the US [1]

Epidemiological data refers to the US, unless otherwise specified.


Types Description Percentage
Type A
  • ∼ 8% of cases
Type B
  • ∼ 3% of cases
Type C
  • ∼ 84% of cases
Type D
  • ∼ 1% of cases
Type E
  • ∼ 4% of cases




Clinical features



Newborns usually present with symptoms directly after birth! The exception is the Gross type E fistula: The diagnosis of a small H‑type tracheoesophageal fistula may occur as late as adulthood.




Differential diagnoses

Double aortic arch


Differential diagnoses of newborn swallowing disorders
Differential diagnosis Findings
Esophageal atresia
Status post C‑section
  • Excessive secretions
  • Reversible condition, as opposed to esophageal atresia
Choanal atresia
  • Cyanotic attacks
  • Attacks normalize after crying or opening the mouth
Esophageal stenosis
  • Delayed diagnosis (after introduction of solid food)
  • Dysphagia
  • Regurgitation
  • Very rare during childhood
  • Delayed diagnosis (after introduction of solid food)
  • Dysphagia
  • Regurgitation
Defective swallowing reflex


The differential diagnoses listed here are not exhaustive.



Infants who potentially have esophageal atresia should not be fed orally under any circumstances!


Surgical treatment should be performed within the first 24 hours of birth.




Overall good prognosis, but surgical complications occur frequently:


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