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.
Epidemiological data refers to the US, unless otherwise specified.
|Type A|| |
|Type B|| |
|Type C|| |
|Type D|| |
|Type E|| |
- 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) 
- Polyhydramnios: the fetus is unable to swallow amniotic fluid ; associated with an increased risk of premature birth 
- 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 
- Further diagnostics concerning VACTERL anomalies
Double aortic arch
- Definition: : an embryonic malformation resulting in a double aortic arch (vascular ring anomaly; ) with subsequent constriction of the trachea and esophagus
- Pathophysiology: the right and left pharyngeal arch arteries persist postnatally → formation of a vascular ring (double aortic arch) → constriction of the trachea and esophagus
Clinical features 
- Typically manifests within the first weeks of life, especially in cases of tracheal compression
- Tracheal constriction: inspiratory or expiratory stridor, dyspnea, respiratory arrest
- Esophageal constriction: dysphagia, choking, retching, vomiting
- Treatment: surgical division of the minor arch 
|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 
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 
- Anastomosis under tension: postoperative ventilation (for approx. 5 days)
- Radiological examination with a contrast agent (esophagram) one week after surgery to identify complications: e.g., esophageal stricture or anastomotic leak 
- Approx. 4 weeks after the procedure: Gastroscopy (EGD) and dilation of the anastomosis may be necessary.
Overall good prognosis, but surgical complications occur frequently: