Tracheoesophageal fistula & esophageal atresia treatment

Tracheoesophageal fistula and esophageal atresia are birth defects that involve the esophagus (throat) and the trachea (windpipe). These esophageal motility disorders may occur separately or together. They affect every baby differently.

What is tracheoesophageal fistula (TEF)?

Tracheoesophageal fistula is a condition where the esophagus and trachea have an opening between them.

With TEF, air may enter the stomach and cause it to be distended. Or contents of the esophagus (milk and secretions) may be inhaled. This can cause choking, pneumonia and even death in serious cases.

TEF can’t be diagnosed until after your baby is born.

Older children or adults with cancer could develop TEF when a tumor invades both the trachea and esophagus.

What is esophageal atresia?

Atresia occurs when a body part fails to develop normally and closes off or narrows when it shouldn’t. When your baby is born with esophageal atresia, it means their esophagus doesn’t reach the stomach correctly.

The most common way this happens is when the top part of the esophagus has a closed end and the bottom part of the esophagus is connected to the trachea (tracheoesophageal fistula). However, esophageal atresia can also occur without a fistula. In most types of atresia, your baby won’t be able to swallow food into their stomach. They may also have trouble breathing.

It’s possible to diagnose esophageal atresia during pregnancy. When this happens, your doctors will plan for treatment as soon as your baby is born. If it’s not diagnosed before birth, esophageal atresia symptoms are usually obvious as soon as your baby tries to feed.

Causes of tracheoesophageal fistula and esophageal atresia

Causes of either of these conditions are unknown and they don’t appear to be inherited. Researchers believe that many factors may be involved.

Related conditions

Esophageal atresia has been associated with several genes and with trisomy 13, trisomy 18 and trisomy 21 genetic disorders.

About 50% of the time, having TEF and esophageal atresia together is related to VACTERL or CHARGE syndromes, which are groups of birth defects that are often found together. Birth defects of the eyes, ears, kidneys, intestines, heart, stomach, muscles or bones may be involved in these syndromes.

When your baby has more than one birth defect, priority will be given to treating the one that is most dangerous. Treatment for TEF and esophageal atresia may be delayed until other things are under control.

Your doctors will explain your baby’s individual condition completely and keep you informed at every stage of their treatment.

Symptoms of TEF

Symptoms of TEF may not appear right away, so a diagnosis may not come until the early weeks of your baby’s life. Coughing while feeding and lung infections are the most common symptoms.

Symptoms of esophageal atresia

Your baby’s symptoms of esophageal atresia will vary depending on how much of their esophagus is missing and whether they also have TEF.

Symptoms may include:

• Bluish skin color
• Coughing or choking during feeding
• Difficulty breathing
• Frothy white bubbles in your baby’s mouth

Diagnosis of tracheoesophageal fistula and esophageal atresia

To diagnose TEF and esophageal atresia, your baby’s doctor needs to observe their symptoms and perform tests that show the location of the defects. Some of the tests they may use are:

• X-rays of the chest and abdomen.
• Endoscopy of esophagus: Inserting a scope (clear, thin tube with a camera at the end) into the esophagus to see how far it goes and whether there’s a connection to the trachea.
• Bronchoscopy of trachea and lungs: Inserting a scope into the trachea to see if there are connections to the esophagus.

Treatment for tracheoesophageal fistula and esophageal atresia

Until your baby’s defects can be corrected, they’ll need to be fed artificially through an IV with total parenteral nutrition or directly into the stomach using a tube.

Surgery will be needed to disconnect the trachea and esophagus or to repair any holes. Depending on your baby’s condition, several surgeries may need to be done over a period of weeks.

Esophageal atresia treatment makes the esophagus long enough to reach the stomach.

If the gap between the top and bottom sections of the esophagus is small enough, they’ll be stitched together using primary anastomosis.

When jejunal interposition is used, a piece of the small intestine may be used to bridge the gap between the esophagus and the stomach.

Sometimes internal traction is used to encourage the parts of the esophagus to grow toward each other until they’re long enough to be stitched together.

When tracheoesophageal fistula and esophageal atresia are both present, we’ll use a combination of treatments. Treatments may occur over a period of weeks.