Tracheoesophageal Fistula and Esophageal Atresia

What are tracheoesophageal fistula and esophageal atresia?

Tracheoesophageal fistula

Tracheoesophageal fistula is a connection between the esophagus and the trachea. The esophagus is the tube that connects the throat to the stomach. The trachea is the tube that connects the throat to the windpipe and lungs. Normally, the esophagus and trachea are 2 tubes that are not connected. This problem is also called TE fistula or TEF. It can happen in 1 or more places.

TE fistula is a birth defect. This means it’s a problem you are born with. It happened when your baby was forming during pregnancy.

When a baby with a TE fistula swallows, liquid can pass through the connection between the esophagus and trachea. When this happens, liquid gets into your baby's lungs. This can cause pneumonia and other problems.

Esophageal atresia

TE fistula often happens with another birth defect called esophageal atresia. This means your baby’s esophagus doesn’t form well during pregnancy. It forms in 2 parts instead of 1. One part connects to the throat. The other part connects to the stomach. But the 2 parts don’t connect to each other.

Since the esophagus is in 2 parts, liquid that your baby swallows doesn’t pass as it should through the esophagus and reach the stomach. This means your baby can't digest milk or other fluids.

What causes TE fistula and esophageal atresia?

As a baby is growing during pregnancy, organ systems are forming. The trachea and the esophagus start forming as 1 tube. At 4 to 8 weeks of pregnancy, a wall forms between the esophagus and trachea. This separates them into 2 tubes. TE fistula and esophageal atresia happen when this wall doesn’t form as it should.

Who is at risk for TE fistula or esophageal atresia?

Having the following issues can raise your baby’s risk for these conditions:

• Trisomy 13, 18, or 21. —These are genetic conditions that happen when there is an extra copy of a chromosome

• Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia, or imperforate anus

• Heart problems, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus

• Kidney and urinary tract problems, such as a horseshoe or polycystic kidney, absent kidney, or hypospadias

• Muscular or skeletal problems

• VACTERL syndrome, which involves spinal, anal, heart (cardiac), kidney (renal), and limb issues

Up to half of babies with TE fistula or esophageal atresia also have another birth defect. 

What are the symptoms of TE fistula or esophageal atresia?

Your child’s healthcare provider will often spot symptoms soon after your baby is born. Symptoms can happen a bit differently in each child. They can include:

• Frothy, white bubbles in the mouth

• Coughing or choking when feeding

• Vomiting

• Blue color of the skin, especially when the baby is feeding

• Trouble breathing

• Very round, full stomach

Your baby may also have other birth defects.

The symptoms of these conditions may look like symptoms of other health problems. Your child must be diagnosed with this condition by their healthcare provider.

How are TE fistula or esophageal atresia diagnosed?

Your child’s healthcare provider will ask about your child’s health history. They will also give your child an exam.

Your child may need an X-ray of their chest and belly. For this test, the healthcare provider will put a small tube into your child's mouth or nose. It will be guided into the esophagus. With esophageal atresia, the tube often can't go very far into the esophagus. The tube's position can be seen with the X-ray. This can help your child’s healthcare provider diagnose the condition.

How are TE fistula or esophageal atresia treated?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. If your child has 1 or both of these issues, they will need surgery.

With a TE fistula, the connection between the esophagus and trachea is closed in surgery.

Sometimes children with esophageal atresia need more than 1 surgery. This depends on how close the 2 tubes are to each other. Your baby's surgeon and healthcare team will decide when your baby should have the surgeries.

What are possible complications of TE fistula or esophageal atresia?

When a baby with a TE fistula swallows, liquid can pass through the connection between the esophagus and trachea. This can cause liquid to get into your baby's lungs. This can lead to pneumonia and other problems.

With esophageal atresia, the esophagus is in 2 parts. Liquid that your baby swallows doesn’t pass normally through the esophagus and reach the stomach. Your baby can't digest milk or other fluids.

Living with TE fistula or esophageal atresia

TE fistula

Children who had surgery for a TE fistula may develop scar tissue. This can make swallowing tricky. Your child may need another surgery to open the scar tissue.

Esophageal atresia

Some children with esophageal atresia have long-term problems. It may be hard for them to swallow foods and liquids. This may be because of the following:

• Trouble with the movement of foods and liquids down the esophagus (peristalsis)

• Scarring that can happen after surgery as the wounds heal and can partially block the passage of foods

Some children may need a special test to widen a narrowed esophagus. Your child’s healthcare provider may do this while your child is under general anesthesia. Other children may need another surgery. Your child’s surgeon will open up the esophagus so food can pass to the stomach.

About half of children who had surgery for esophageal atresia will develop gastrointestinal reflux disease (GERD). GERD causes acid to move up into the esophagus from the stomach. This causes a burning or painful feeling called heartburn. GERD can often be managed with medicines.

Work with your child’s healthcare team to create a care plan for your child. This will help your child grow and develop well.

When should I call my child's healthcare provider?

Before surgery. Call your child’s healthcare provider right away if your child breathes liquid into their lungs. Call 911 if your child has trouble breathing.

After surgery. Call your child’s healthcare provider if your child has trouble swallowing, persistent vomiting, or isn't gaining weight.

Key points about TE fistula and esophageal atresia

• TE fistula is an abnormal connection between the esophagus and the trachea. Esophageal atresia happens when the esophagus has 2 segments. These parts don’t connect to each other.

• Your child’s healthcare provider will often spot symptoms of these issues soon after your baby is born.

• Both of these problems can happen together. If your child has 1 or both issues, they will need surgery.

• Even after surgery, children with these conditions may have trouble swallowing well.

• It’s important to work with your child’s healthcare team to create a care plan for your child.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.