Tracheo-esophageal Fistula (TEF) or Esophageal Atresia (EA)

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What is a tracheo-esophageal fistula (TEF) or esophageal atresia (EA)?

Esophageal atresia and tracheoesophageal fistula is a congenital disorder of the esophagus and /or trachea that may result both in an obstruction of the esophagus and communication of the esophagus with the airway (trachea). Since the development of the esophagus occurs early in gestation (4th week), abnormalities (anomalies) can occur in other organs undergoing development during the same time (cardiovascular, urinary, skeletal systems).

What are the causes and incidence?

Early in gestation the trachea and esophagus develop from the primitive foregut and undergoes separation into the esophagus (swallowing tube) and trachea (windpipe). Failure of separation results in the upper esophagus ending blindly and without a connection to the lower esophagus and stomach. In these cases, the blind ending upper esophagus is often connected with the trachea (fistula). If a connection to the airway is absent then the defect is categorized as esophageal atresia. Approximately 1 in every 5,000 births is affected and usually occurs within families sporadically. Additional abnormalities are observed in up to 40% of babies.

What are the symptoms?

  • Excessive amniotic fluid in pregnancy (polyhydramnios) due to inability to swallow amniotic fluid
  • Excessive salivation, drooling, poor feeding due to the inability to swallow milk or saliva
  • Coughing and choking with feeding due to the passage of milk into the airway

 

A fetal ultrasound demonstrating excessive amniotic fluid may suggest esophageal atresia or other obstruction of the gastrointestinal tract. After birth, typically a small feeding tube is passed into the stomach. The failure of the feeding tube to pass into the stomach suggests blockage of the esophagus (esophageal atresia) and an X-ray is obtained. If the X-ray shows the feeding tube coiled in upper esophagus and air is observed in the stomach, then esophageal atresia with tracheesophageal fistula is likely.

 

What is the treatment?

Surgery (incision in the chest) to restore the connection of the esophagus to the stomach and eliminate the abnormal connection to lungs is necessary. Prior to surgery, a prompt evaluation (ultrasound) for other abnormalities, particularly heart problems is conducted. Generally within 24 – 36 hours after birth, the infant proceeds to surgery. In the interim, the infant is not fed by mouth and a suction tube is kept in the upper esophagus to prevent the infant from swallowing saliva into the lungs. Following surgery, your baby will be fed by vein and a tube will drain the chest until a contrast X-ray study is performed (5-7 days) to ensure that there is no leak.

 

What is the expected outcome?

In babies without significant other abnormalities, survival is greater than 95%. However, your baby might require additional procedures, like esophageal dilation if there is persistent narrowing of the esophagus.

 

What are the complications?

  • Respiratory problems (pneumonia, aspiration, tracheomalacia)
  • Leak or stricture of the esophagus
  • Gastroesophageal reflux is common after surgery and can generally be treated with medicine, but some infants may require an anti-reflux operation (fundoplication).

 

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