- Clinical science
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 presents 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 
Epidemiological data refers to the US, unless otherwise specified.
|Type A||Esophageal atresia without tracheoesophageal fistula||∼ 8% of cases|
|Type B||Esophageal atresia with tracheoesophageal fistula to the proximal esophageal segment||∼ 3% of cases|
|Type C||Esophageal atresia with tracheoesophageal fistula to the distal esophageal segment||∼ 84% of cases|
|Type D||Esophageal atresia with tracheoesophageal fistula to the proximal and distal esophageal segments||∼ 1% of cases|
|Type E||H‑type tracheoesophageal fistula without atresia||∼ 4% of cases|
- A wedge of mesoderm called the tracheoesophageal septum separates the developing foregut (esophagus) from the trachea.
- Esophageal atresia and tracheoesophageal fistula are caused by a defect in mesodermal differentiation
About 50% of cases are associated with other mesodermal defects (VACTERL association)
- Vertebral anomaly
- Anal atresia
- Cardiac anomaly
- Tracheoesophageal fistula
- Esophageal atresia
- Renal anomaly
- Limb malformation
- Esophageal atresia → pooling of secretions → excessive secretions/foaming at the mouth
- Tracheoesophageal fistula: an abnormal connection between the trachea and esophagus
- Placement of a feeding tube: the feeding tube cannot pass through the esophagus in the case of esophageal atresia
X-ray of the thorax/abdomen
- Esophageal pouch (except with an H‑type fistula)
- Gross types A and B present with a gasless abdomen
Further diagnostics concerning VACTERL anomalies
- Ultrasound of the abdomen
|Differential diagnoses of newborn swallowing disorders|
|Status post C‑section|| |
|Choanal atresia|| |
|Esophageal stenosis|| |
|Defective swallowing reflex|| |
The differential diagnoses listed here are not exhaustive.
- Placement of an oroesophageal or nasoesophageal tube for continuous suction of secretions to prevent aspiration and facilitate breathing
- Upper body elevated, left lateral decubitus position
- Antibiotics in case of aspiration pneumonia
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.
- The goal is to reconnect the upper esophageal pouch and the lower esophagus.
- A long gap between both ends of the esophagus may not allow primary repair.[
- Uncomplicated surgery → transition to a normal diet after 2–3 days
- Radiological examination with a contrast agent (esophagram) one week after surgery to identify complications: e.g., esophageal stricture or anastomotic leak