A tracheoesophageal fistula (TEF) occurs when there’s a defective connection between the trachea (windpipe) and esophagus (the tube that connects the throat to the stomach).

The condition primarily affects infants. Congenital birth defects typically cause it. Rarely, adults can develop a TEF, usually as a result of cancer treatment, infections, or injuries.

Most cases of TEF in infants occur alongside esophageal atresia (EA), which is when the esophagus doesn’t connect properly to the stomach. EA and TEF occur in about 1 in every 4,000 births in the United States.

This article takes a closer look at TEFs, including the different types, symptoms, diagnosis, treatment, and what the outlook is like for people who have the condition.

Medical experts usually classify TEFs into five main types. The vast majority of cases are type C.

Besides these five types, TEFs are also categorized by when they are formed. Most are congenital in nature, meaning they formed during fetal development.

However, in rare cases, TEFs form later in life, usually from cancer treatment, an infection, or an injury or trauma.

An illustration showing examples of tracheoesophageal fistulas (TEF) and esophageal atresia (EA) and how these issues compare to normal anatomy. Share on Pinterest
Illustration by Jason Hoffman

Type A

In this type, only an esophageal atresia (EA) is present; there is no TEF. This occurs in about 8% of cases.

Type B

In this rare type, the bottom portion of the esophagus ends in a blind pouch, while the upper portion of the esophagus is connected to your windpipe by a TEF. Type B occurs about 2% of the time.

Type C

This is the most common type. It occurs in about 85% of infants born with a TEF. In this type, the upper part of the esophagus ends in a blind pouch, while the lower part is connected to the windpipe by a TEF.

Type D

This is the rarest type. It occurs in under 1% of cases. In this type, the TEF links both portions of the esophagus (upper and lower) to the windpipe.

Type E

Known as an H-type fistula, in this type, the esophagus connects to the stomach in a standard manner. At the same time, a TEF links the esophagus and windpipe together. This type occurs in 4% of cases.

The overwhelming majority of TEFs occur in newborns. Symptoms are usually present at birth or soon after. The condition can cause severe breathing and eating issues. Here’s what you might notice in your baby:

  • inability to swallow normally
  • breathing issues
  • large amount of drool or mucus
  • white, frothy bubbles in the mouth
  • aspiration of gastric juices, saliva, mucous, or other secretions into the lungs, causing breathing issues
  • gagging, coughing, choking
  • signs of low oxygen levels, which may include skin turning blue, labored breathing, and flared nostrils

Again, in rare cases, adults may develop a TEF. Symptoms in adults may include:

  • coughing
  • aspiration of fluids
  • fever
  • pneumonia
  • difficulty swallowing
  • coughing up blood
  • coughing that includes swallowing solid or liquid matter

Sometimes TEFs — and more commonly EAs — are seen during a routine ultrasound during pregnancy. But most TEFs are diagnosed soon after birth, based on an infant’s symptoms.

If your doctor believes your infant has a TEF, EA, or both, they will perform a few diagnostic tests. These include X-rays of the chest and stomach, and esophageal endoscopy or bronchoscopy.

Infants with a TEF or EA are often born with other developmental irregularities, most commonly cardiac issues. A doctor may also conduct diagnostic tests, such as an echocardiogram, to see whether a cardiac irregularity is present.

TEFs must be treated with surgery. The surgery repairs the connection between the trachea and esophagus. Surgery usually happens soon after birth (usually within 24 to 48 hours) unless your baby has other complications, such as a cardiac issue or an infection.

A team of specialists manage surgeries for TEFs. This team includes pediatricians, intensive care unit (ICU) nurses, surgeons, and cardiologists. Your baby will get general anesthesia during surgery and will need to recover in the neonatal ICU (NICU).

Surgeons have been performing TEF repairs for many years. The survival rate for this surgery is over 90%. Infants with cardiac defects or who were born at low birth weights are less likely to survive the surgery.

Is a tracheoesophageal fistula life threatening?

Yes. If a TEF isn’t addressed promptly with surgical repair, it can become life threatening for an infant.

This is because babies with a TEF have trouble feeding and breathing. If not corrected, the lungs can also become damaged from repeated exposure to gastric fluids.

To avoid these issues, it’s important that TEFs be treated as soon as possible after birth.

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With surgery, most infants have a good outlook and will be able to live a normal life.

Recovery from surgery can take several weeks. Infants will need to be watched carefully and cared for in the NICU.

Complications that may happen in the weeks following surgery include leakage at the area of reconnection or narrowing of tissues at the reconnection site. TEFs can come back in about 3% of cases.

Children and adults who had TEF and EA surgery as infants may have some long-term side effects. The most common side effect is a condition called esophageal dysmotility. It causes the smooth muscles of the esophagus to contract abnormally. This causes swallowing issues and gastroesophageal reflux.

Another side effect among children is that they may be more likely to experience repeated chest infections when they are young.

Tracheoesophageal fistula (TEF) is a serious condition that mostly affects newborn babies. It’s characterized by a faulty connection between the windpipe and esophagus, resulting in breathing and feeding issues.

Most of the time, infants also have esophageal atresia (EA), where the esophagus doesn’t connect properly to the stomach.

Because a TEF is life threatening if not treated promptly, a baby diagnosed with this condition will usually need to have surgery soon after birth.

If you have any further questions about TEFs, don’t hesitate to reach out to your pediatrician or other members of your baby’s medical team.