Esophageal Atresia and Tracheoesophageal Fistula

Instructor: Lisa Cauthen

Lisa is a Registered Nurse with a 14 years of experience and a Masters Degree in Nursing Education. She has certifications in CPN, ACLS, PALS, and NRP.

Esophageal Atresia and Tracheoesophageal Fistulas are congenital defects of the esophagus and trachea affecting eating and breathing in the newborn. In this lesson we will review the types, diagnosis, treatment, and prognosis for the infant with this condition.

Esophageal Atresia (EA) and Tracheoesophageal Fistula (TEF)

It's your fist night in the neonatal intensive care unit and the nurses are preparing for admission of an infant. On the admission papers you see 'EA/TEF-type D.' What does that mean? You remember it has something to do with the esophagus. Or was it the trachea? Could it have been both? The Admission team heads to the delivery room and you dive into your notes so you'll be prepared for this baby's arrival.

Esophageal Atresia, or Type A

Esophageal Atresia (EA) occurs when the esophagus (the passage between the mouth and stomach) is not developed correctly. There is an incomplete passageway or an inability for the food to pass from the mouth to the stomach. Food that is swallowed is not able to reach the stomach. Instead, the infant's saliva and food is regurgitated, putting the infant at risk for choking and aspirating. A simple EA with no Tracheoesphageal Fistula is type A and is represented by figure b in the image below.

Tracheoesophageal Fistula, or Type E

Tracheoesphageal Fistula (TEF) is when the trachea and esophagus share a 'passageway'. With TEF, or type E, a simple (uncomplicated) fistula joins an otherwise normal esophagus and trachea. This is represented by figure c in the image below.

Esophageal Atresia with Tracheoesophageal Fistula

When both abnormalities exist there can be variations in the location and degree of esophageal atresia and the tracheoesophageal fistula. There can be a fistula at the proximal or distal portion of the esophagus and trachea, or both. The esophagus can be fully or partially separated. These variations are classified the following way:

Type B - Complete EA with a proximal (nearer to the head) TEF

Type C - Complete EA with a distal (farther from the head) TEF (portrayed by figure a in the image below)

Type D - Complete or incomplete EA with both proximal and distal TEFs

The image below represents three types of EA and TEF, however the lettering of the figures differs from the type.

Esophageal Atresia, Tracheoesophageal Fistula
Esophageal Atresia, Tracheoesophageal Fistula

Diagnosis of EA and TEF

Diagnosis of EA and TEF is is often made using ultrasound at a prenatal visit. However, not all pregnant women receive prenatal care and not all prenatal ultrasounds reveal all conditions, so some infants are not diagnosed until after birth.

EA should be suspected when an infant cannot swallow their secretions and has drooling (drooling is not typical in a newborn). If not discovered right away, EA will exhibit with feeding difficulties because the food that is swallowed cannot pass to the stomach. The infant may pool the milk in it's mouth, appear to spit up or vomit the milk, or cough and choke on the milk. This infant is at risk for aspiration and development of pneumonia. Oral feedings should be stopped when EA is suspected.

An infant with TEF may present with abnormal respiratory sounds and/or respiratory distress. As the infant takes its first breaths the full amount of air that should be passing into the lungs is instead distributed in the lungs and the stomach, the upper esophagus (and out of the mouth), or both, depending on the location of the TEF.

Diagnosis of EA and/or TEF is made with imaging studies such as ultrasound or x-ray.

X-ray of Esophageal Atresia
Xray of Esophageal Atresia

Treatment and Prognosis

Upon diagnosis of EA and TEF, the infant is admitted to the neonatal intensive care unit (NICU). Because of the risk of an infant choking or aspirating its saliva, a tube will be placed into the upper pouch of the EA or stomach (in the case of type E) to remove the secretions and prevent choking. The infant will also be nourished through IV fluids and breathing assistance can be given as needed.

Both EA and TEF require surgical repair soon after birth. For type A, or simple esophageal atresia, the surgery is also rather simple. The two closed ends of the esophagus are opened and joined together. The surgical repair of type E is also rather uncomplicated as it requires the removal of the fistula and has little to no involvement with the trachea or esophagus. Surgical repair for types B, C, and D are more involved and the details of repair depend upon the location and severity of the condition.

Below is an image of surgical repair of a Type C EA and TEF.

Repair of EA with TEF, Type C
Repair of EA with TEF

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