Tracheoesophageal Fistula

Overview

What is tracheoesophageal fistula?

Typically, your esophagus (the hollow tube that connects your throat to your stomach) and trachea (windpipe) are separate. People with tracheoesophageal fistula (TEF) have an abnormal connection between these two structures. This results in aspiration (inhalation) of food and liquids into your lungs. Tracheoesophageal fistula commonly occurs with esophageal atresia (EA) — a condition that’s characterized by an abnormal connection between your esophagus and stomach.

Who does tracheoesophageal fistula affect?

Tracheoesophageal fistula is a congenital condition. In other words, people are born with it. Most cases of TEF are diagnosed and treated in infancy.

It’s also possible for adults to develop tracheoesophageal fistula as the result of esophageal cancer or lung cancer, infections (such as tuberculosis) or trauma from a medical procedure. This is called acquired tracheoesophageal fistula.

How common is tracheoesophageal fistula?

It’s estimated that tracheoesophageal fistula affects 1 in every 3,000 to 5,000 births in the United States. Approximately 50% of babies with TEF or EA have another congenital condition.

Acquired tracheoesophageal fistula, which occurs in adulthood, isn’t common. Very rarely, adults can have congenital tracheoesophageal fistula. This means that they were born with the condition but didn’t develop symptoms until later in life.

What are the five types of tracheoesophageal fistula?

Tracheoesophageal fistula is generally categorized into five different types:

  • Type A: There’s no TEF, only EA. Your esophagus is divided into two parts, with both portions ending in blind pouches (cavities that are closed at one end). This is also commonly referred to as pure esophageal atresia. It makes up about 8% of all cases.
  • Type B: This rare form of TEF affects about 2% of all cases. The lower portion of your esophagus ends in a blind pouch, and the upper portion is connected to your windpipe by a tracheoesophageal fistula.
  • Type C: The most common form of TEF, type C is when the upper portion of your esophagus ends in a blind pouch, and the lower portion is connected to your trachea by a tracheoesophageal fistula. About 85% of babies born with TEF have this type.
  • Type D: In this rarest form of TEF, a tracheoesophageal fistula connects both the upper and lower portions of your esophagus to your trachea. Less than 1% of babies born with TEF have this type.
  • Type E: Your esophagus connects to your stomach normally and is fully intact. However, a tracheoesophageal fistula connects your esophagus and trachea. Type E affects about 4% of TEF cases.

Symptoms and Causes

What are the symptoms of tracheoesophageal fistula?

Tracheoesophageal symptoms depend on whether EA is present, as well as TEF. Babies who are born with TEF but not EA usually don’t show symptoms at birth. Over time, however, they may:

  • Have frequent lung infections.
  • Cough when feeding.

Babies who have esophageal atresia with tracheoesophageal fistula usually exhibit symptoms immediately after delivery. The most common symptoms include:

  • Coughing.
  • Choking when trying to swallow.
  • Breathing problems.

What causes tracheoesophageal fistula?

During fetal development, your esophagus and trachea form as one single tube. Typically, about four to eight weeks after conception, a wall develops between these structures, making them two distinct tubes. If this wall doesn’t form properly, it can result in tracheoesophageal fistula.

Diagnosis and Tests

How do you diagnose a TEF?

In rare instances, your healthcare provider may suspect a tracheoesophageal fistula before your baby is born due to abnormalities on an ultrasound. Most of the time, however, your medical team may suspect TEF within a few hours of delivery if your baby has excessive mucous, breathing difficulties or is unable to swallow.

To confirm a TEF or EA diagnosis, your healthcare provider will likely take X-rays of their chest and abdomen. In addition, they may recommend an endoscopy or bronchoscopy. These tests allow them to look inside of the airways using a thin tube and a lighted camera. If your baby is diagnosed with TEF or EA, your healthcare provider will probably order additional tests to identify or rule out other congenital conditions.

Management and Treatment

How is tracheoesophageal fistula treated?

Tracheoesophageal fistula treatment involves corrective surgery. During this procedure, the connection between your baby’s esophagus and trachea is repaired. This may be done using traditional or minimally invasive techniques.

Are there risks involved with tracheoesophageal fistula repair?

As with any surgical procedure, tracheoesophageal fistula repair may be accompanied by complications. Possible risks include anastomotic leaks (when fluid leaks out from the area where the esophagus and trachea were joined), esophageal strictures (abnormal tightening of the esophagus) and damage to the laryngeal nerve.

There’s also a chance that TEF could come back later. Approximately 3% to 14% of TEF repairs result in fistula recurrence (return).

How long does it take to recover from tracheoesophageal fistula treatment?

Recovery time depends on several factors, including the severity of your baby’s condition and how well they respond to treatment. In most cases, full healing takes up to 12 weeks.

Prevention

Can I prevent tracheoesophageal fistula?

Because people are born with congenital TEF, there’s no way to prevent it from happening. And since most cases of acquired TEF are caused by cancer and infections, there’s no known way to reduce your risk for the condition.

Outlook / Prognosis

What can I expect if my baby has tracheoesophageal fistula?

If your baby has TEF, your healthcare provider will recommend surgery to address the problem. The extent of surgery depends on the type of TEF. If your baby has postoperative complications, your medical team will keep your baby in the hospital for a few days to monitor their progress.

Babies who have complications after their initial surgery are more likely to experience recurrence. Therefore, if your baby has postoperative complications, your healthcare provider will perform periodic follow-ups.

Is tracheoesophageal fistula curable?

Yes. Tracheoesophageal fistula is curable with surgical intervention. Because it’s a life-threatening condition, it should be treated immediately.

Living With

What can I expect if my baby has tracheoesophageal fistula?

If your baby has TEF, your healthcare provider will recommend surgery to address the problem. The extent of surgery depends on the type of TEF. If your baby has postoperative complications, your medical team will keep your baby in the hospital for a few days to monitor their progress.

Babies who have complications after their initial surgery are more likely to experience recurrence. Therefore, if your baby has postoperative complications, your healthcare provider will perform periodic follow-ups.

Is tracheoesophageal fistula curable?

Yes. Tracheoesophageal fistula is curable with surgical intervention. Because it’s a life-threatening condition, it should be treated immediately.

A note from Cleveland Clinic

Left untreated, tracheoesophageal fistula is a life-threatening condition. Prompt care ensures that the abnormal connection between your baby’s esophagus and windpipe is repaired. This greatly reduces the risk of aspiration. There are also several organizations that provide support and resources, including EA/TEF Child and Family Support Connection, Inc., the Fetal Hope Foundation and the Genetic and Rare Diseases (GARD) Information Center.

Last reviewed by a Cleveland Clinic medical professional on 06/28/2022.

References

  • National Center for Biotechnology Information. Tracheoesophageal Fistula. (https://www.ncbi.nlm.nih.gov/books/NBK535376/) Accessed 6/28/2022.
  • National Organization for Rare Disorders. Esophageal Atresia and/or Tracheoesophageal Fistula. (https://rarediseases.org/rare-diseases/esophageal-atresia-andor-tracheoesophageal-fistula/) Accessed 6/28/2022.
  • U.S. National Library of Medicine. Esophageal atresia/tracheoesophageal fistula. (https://medlineplus.gov/genetics/condition/esophageal-atresia-tracheoesophageal-fistula/) Accessed 6/28/2022.
  • Yang S, Li S, Yang Z, et al. Risk Factors for Recurrent Tracheoesophageal Fistula After Gross Type C Esophageal Atresia Repair. (https://www.frontiersin.org/articles/10.3389/fped.2021.645511/full) Frontiers in Pediatrics. 2021;9:454. Accessed 6/28/2022.

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