Tracheoesophageal fistula & esophageal atresia treatment

Tracheoesophageal fistula and esophageal atresia are serious birth defects that can occur separately or together. Affecting the esophagus (throat) and the trachea (windpipe), these esophageal motility disorders look different in each baby.

Tracheoesophageal fistula may also occur in older children or adults because of a cancer that involves both the esophagus and trachea.

What is tracheoesophageal fistula (TEF)?

Tracheoesophageal fistula is an opening between the windpipe and throat.

TEF can cause choking, pneumonia and sometimes death by allowing some contents of the esophagus (milk, saliva) to be breathed into the lungs. Air may also enter the stomach, which can cause the stomach to be swollen.

TEF can’t be diagnosed until after birth.

What is esophageal atresia?

Esophageal atresia is when the esophagus doesn’t connect to the stomach. Often, there is a top part and a separate bottom part of the esophagus. The top and bottom parts either end in a pouch or end in a hole in the trachea. Your baby will be unable to get food to their stomach and may have trouble breathing.

Esophageal atresia may be diagnosed during pregnancy, which gives you and your doctors time to plan for immediate action when your baby is born. Symptoms of esophageal atresia are usually obvious from the first time your baby tries to feed.

Causes of tracheoesophageal fistula and esophageal atresia

We don’t know what causes either of these conditions. Neither condition appears to be inherited.

Related conditions

Several genes have been associated with esophageal atresia. It’s also associated with genetic disorders trisomy 13, trisomy 18 and trisomy 21. However, it’s thought that many factors may play a part.

About half of babies who have tracheoesophageal fistula and esophageal atresia also have groups of other birth defects. The other birth defects may involve the heart, kidneys, stomach, bowels, eyes, ears, muscles or bones. If your baby has some of those other defects, priority may be given to treating other defects before treating tracheoesophageal fistula or esophageal atresia.

Regardless of how your baby is affected, your doctors will explain their condition carefully and let you know what to expect at each stage of diagnosis and treatment.

Symptoms of TEF

Babies with TEF may not have symptoms right after their birth. The most common symptoms are coughing while feeding and lung infections.

Symptoms of esophageal atresia

Esophageal atresia symptoms vary depending on the details of your baby’s condition. Common symptoms include:

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

Diagnosis of tracheoesophageal fistula and esophageal atresia

Tracheoesophageal fistula and esophageal atresia are diagnosed based on symptoms and on X-rays of the chest and abdomen.

Diagnosis of tracheoesophageal fistula may also include these tests:

• 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

If your baby is born with either or both of these conditions, they’ll need to be fed artificially at first – either through an IV (total parenteral nutrition) or through a tube into the stomach (enteral nutrition).

Tracheoesophageal fistula may be treated with surgery to remove the connections between the windpipe and the throat and to repair the holes in each. Stents (tubes) in the esophagus or trachea may also be used to prevent leaking of liquids or gases into the wrong pipe.

Esophageal atresia may be treated in different ways depending on how much of the esophagus is missing and whether it’s connected to the trachea.

• Internal traction: Each part of the esophagus may be encouraged to grow using static internal traction and dynamic internal traction.
• Jejunal interposition: A piece of small intestine may be inserted and stitched in place to replace the missing part of the esophagus.
• Primary anastomosis: If the parts of the esophagus are long enough, they may be sewn together.

When the conditions occur together, a combination of treatments will be used. They may require a series of treatments over a period of weeks.