Tracheoesophageal fistula (TEF)
Find a fetal care specialistTracheoesophageal fistula (TEF) is a rare but serious condition that affects newborns. It happens when there’s an abnormal connection between the esophagus and trachea, causing feeding and breathing challenges.
While hearing your baby has tracheoesophageal fistula or any birth defect can be overwhelming, our team of fetal care experts are here to provide expert care and compassionate support every step of the way.
A newborn with esophageal atresia and tracheoesophageal fistula has an abnormal connection between the esophagus and trachea.
What is tracheoesophageal fistula?
Tracheoesophageal fistula is a birth defect where the esophagus (the tube that carries food from the mouth to the stomach) is connected to the trachea (the windpipe that carries air to the lungs).
This abnormal connection can cause serious issues with feeding and breathing, as food or liquid may enter the lungs instead of the stomach, leading to breathing problems.
Tracheoesophageal fistula often occurs with esophageal atresia, where the upper part of the esophagus doesn’t connect to the lower esophagus and stomach. This combination makes it impossible for newborns to swallow and digest food properly.
What is the difference between TEF and EA?
While tracheoesophageal fistula and esophageal atresia (EA) are closely related, they’re not the same. TEF involves an abnormal connection between the esophagus and trachea, while EA refers to a blockage or discontinuity in the esophagus.
Many newborns with TEF also have EA, which complicates feeding and breathing further. Together, these conditions are called tracheoesophageal fistula with esophageal atresia.
How common is tracheoesophageal fistula?
Tracheoesophageal fistula is one of the most common congenital anomalies, affecting about 1 in 4,000 births. It’s slightly more common in males than females and often occurs with other birth defects such as heart abnormalities, gastrointestinal anomalies or a septal defect (a hole in the heart’s wall).
Tracheoesophageal fistula types
Tracheoesophageal fistula types are classified into types based on the anatomy of the esophagus and trachea:
- Type A (Isolated esophageal atresia): The esophagus is not connected to the trachea, and the upper and lower segments of the esophagus each end in a pouch (esophageal atresia).
- Type B: The upper esophagus connects to the trachea, but the lower esophagus is not connected.
- Type C: The upper esophagus ends in a pouch, and the lower esophagus connects to the trachea. This is the most common type.
- Type D: Both the upper and lower parts of the esophagus connect to the trachea.
- Type E (H-type fistula): The esophagus and trachea are connected by a single abnormal channel, and there’s no esophageal atresia (pouch).
Tracheoesophageal fistula symptoms
Signs and symptoms of tracheoesophageal fistula in newborns can appear shortly after birth and may include:
- Difficulty feeding or choking during feedings
- Excessive drooling
- Coughing or gagging, especially when eating
- Cyanosis (bluish skin tone) during feedings
- Recurrent respiratory infections or pneumonia
- Abdominal distension from swallowed air
If your baby exhibits these symptoms, it’s important to seek medical attention immediately.
What causes tracheoesophageal fistula in newborns?
The exact cause of tracheoesophageal fistula isn’t fully understood, but it’s believed to result from abnormal development during pregnancy. Genetic factors and environmental influences may play a role.
Tracheoesophageal fistula often occurs alongside other congenital conditions, including heart defects, gastrointestinal anomalies and chromosomal abnormalities.
Diagnosing tracheoesophageal fistula
Doctors typically diagnose tracheoesophageal fistula shortly after birth based on symptoms and digital imaging.
Common diagnostic tests include:
- Chest X-rays: To identify abnormalities in the esophagus and trachea.
- Contrast studies: Using a special dye to highlight the esophagus and trachea on X-rays.
- Endoscopy: A thin, flexible tube with a camera is inserted through the throat to visualize the esophagus and trachea.
Tracheoesophageal fistula treatment
The primary treatment for tracheoesophageal fistula is surgery to repair the abnormal connection and restore normal function. The timing and approach depend on your baby’s overall health and the type of tracheoesophageal fistula.
Your baby may need a feeding tube to bypass the esophagus and prevent aspiration (food entering the lungs) prior to surgery. Respiratory support may also be required.
During surgery, the surgeon separates the esophagus and trachea, repairs the fistula and reconnects the esophageal segments if necessary.
After surgery, your baby may need a stay in the neonatal intensive care unit (NICU) for monitoring and recovery. Follow-up care often includes feeding therapy and regular check-ups to monitor growth and development.
