Tracheoesophageal Fistula

A tracheoesophageal fistula (TEF) is a rare congenital condition that affects your baby’s ability to feed and breathe. “Congenital” means the condition is present at birth. A fistula is an abnormal connection between body parts.

In this case, your baby has a fistula, or abnormal connection, between their esophagus (food tube) and trachea (windpipe). Normally, these parts are separate. In TEF, they connect, so liquid goes into your baby’s trachea instead of their esophagus. Babies with this condition often have esophageal atresia (EA). This condition affects your baby’s esophagus, so liquid can’t move to their stomach.

About 1 in 4,000 babies in the U.S. have TEF. Rarely, adults may develop this condition. This is an acquired tracheoesophageal (pronounced “tray-kee-oh-es-uh-jee-ul”) fistula.

Types of tracheoesophageal fistula

The five types of this condition are:

• Type A: Your baby has esophageal atresia. Their trachea works as it should. Type A accounts for 8% of all TEF cases.
• Type B: In this rare TEF type, the upper part of your baby’s esophagus connects to their trachea. The lower part is closed. Type B accounts for about 2% of all cases.
• Type C: This is the most common TEF type. In this case, the lower part of your baby’s esophagus connects to their trachea. The upper part of their esophagus is closed. About 85% of babies with TEF have this type.
• Type D: Here, both parts of your baby’s esophagus connect to their trachea. Type D affects about 1% of babies with TEF.
• Type E: There’s an abnormal connection between your baby’s esophagus and trachea, but their esophagus works as it should. This type affects 4% of babies with TEF.

Tracheoesophageal fistula symptoms

Babies with TEF may have symptoms at birth or within a few hours after they’re born. Symptoms may include:

• Blue tint to their skin from cyanosis
• Choking when they try to swallow
• Difficulty breathing
• Drool or vomit that looks like foam on your baby’s mouth
• Sudden and severe coughing when your baby nurses or feeds

Some babies develop symptoms within the first few weeks of life. Your baby may have:

• Difficulty feeding
• Frequent lung infections

Tracheoesophageal fistula causes

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 a tracheoesophageal fistula.

TEF may happen if your baby has a genetic condition. Examples include:

• CHARGE syndrome
• Chromosomal disorders, like Down syndrome
• Fanconi anemia

Very rarely, adults can have acquired TEF. This can happen if they have:

• Esophageal cancer or lung cancer
• Infections, including tuberculosis
• Trauma, if they have surgery or treatment, when they need a breathing tube for a long time

Complications

Babies with a tracheoesophageal fistula often have other congenital conditions. For example, some babies have congenital heart issues or issues with their digestive system or urinary tract.

Other TEF complications include malnutrition and tracheomalacia, which happens when your baby’s trachea collapses.

How doctors diagnose this condition

In rare instances, a prenatal ultrasound may detect signs of a tracheoesophageal fistula. Most of the time, your baby’s care team will diagnose TEF very soon after your baby is born. They may do tests, including:

• X-rays to check your baby’s chest and belly
• Bronchoscopy or endoscopy to look at the inside of your baby’s trachea

How is TEF treated?

Your baby may have surgery to repair the abnormal connection. They may have minimally invasive surgery or open surgery. Before surgery, their care team may:

• Give them humidified oxygen
• Give them antibiotics and fluids through an IV line (a flexible tube that delivers fluids and medication into a vein)

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.

When should I seek care for my baby?

TEF symptoms may develop immediately or several days or weeks after your baby is born. Talk to their healthcare provider if your baby has frequent lung infections or feeding issues.

What can I expect if my baby has TEF?

Surgery often cures it. But some babies experience complications after surgery, including:

• Fluid leaking from the area where your baby’s trachea and esophagus were connected
• Abnormal tightening of their esophagus
• Damage to their laryngeal nerve, which controls their voice box

Your baby may have regular checkups so their provider can watch for signs that TEF has come back. Every baby’s situation is different. Their provider will explain how often your baby should have checkups.

How do you feed a baby with a tracheoesophageal fistula?

Your baby will receive nutrients through an IV or a tube into their stomach. This is a short-term solution to make sure your baby gets enough nutrients until they have surgery to fix the fistula.