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Esophageal Atresia and Tracheoesophageal Fistula

Last Updated May 2022 | This article was created by familydoctor.org editorial staff and reviewed by Deepak S. Patel, MD, FAAFP, FACSM

What is esophageal atresia (EA) and a tracheoesophageal fistula (TEF)?

An esophageal atresia (EA) and a tracheoesophageal fistula (TEF) are 2 disorders of the digestive system. The condition affects babies. It starts while the mother is pregnant. This makes it a congenital defect. The 2 disorders often happen together.

An EA is when the esophagus doesn’t connect to the stomach. TEF is when the esophagus and windpipe (trachea) have an incorrect connection. The poor connection is called a fistula. There are several types of EA. An EA sends the baby’s nutrients to a pouch. So, nothing the baby swallows enters the stomach. With a TEF, food or milk can get into your baby’s lungs when they swallow. This can cause breathing problems and even pneumonia.

About 1 of 4,000 babies has one or both of these problems. Sometimes a baby is born with one and not the other.

Babies with this condition can breathe saliva and other fluids into their lungs. This can cause pneumonia, choking, and even death. Being born prematurely may make the condition worse.

Symptoms of EA/TEF

The main problems EA causes are with the digestive system. TEF usually causes breathing problems. Symptoms of EA/TEF include:

  • Feeding problems upon birth
  • Frequent drooling or spitting up
  • Bubbly mucus in the mouth
  • Coughing, gagging, or choking when feeding
  • Bluish skin color when feeding
  • Difficulty breathing

Most babies with EA develop symptoms right after birth. For babies who have TEF but not EA, symptoms are mild and could take weeks to diagnose.

What causes EA/TEF?

Doctors are not sure exactly what causes these problems. While pregnant, your child’s esophagus and trachea start to grow from the same bit of tissue. Sometimes the tubes don’t develop correctly. Doctors don’t think it’s inherited. In some cases, an EA/TEF have a heart defect.

How is EA/TEF diagnosed?

An ultrasound during pregnancy is the first clue. If there is too much amniotic fluid (the fluid that surrounds the baby), the baby may have a blockage in his or her digestive system. The blockage could be an EA.

If your doctor suspects EA after the baby is born, they can insert a feeding tube through your baby’s mouth or nose. The tube normally goes down to the stomach. If it can’t reach the stomach, your doctor will probably diagnose your baby with EA. An X-ray can detect air in the esophagus, stomach, and intestine. It can help confirm the diagnosis. An X-ray can also help confirm TEF.

Can EA/TEF be prevented or avoided?

There is nothing a woman who is pregnant can do to prevent her baby from developing EA/TEF before birth. The best thing she can do is to take good care of herself while pregnant. This includes:

 

  • Eating a healthy diet
  • Exercising
  • Getting plenty of rest
  • Attending all scheduled doctor appointments

EA/TEF treatment

EA/TEF is an emergency situation. Your baby will need surgery to fix the problem. If your baby has EA, the swallowing tube must be connected to the stomach. If a fistula is connecting the esophagus and windpipe, it must be closed. Your child’s doctor will decide when to do the surgery. Healthy, full-term babies can have the surgery at just a few days old. If the baby has any other problems (such as pneumonia or other birth defects), surgery may have to wait.

In uncomplicated cases, your baby may start eating as soon as one week after surgery. Until your baby can swallow milk or formula, they will be fed through an IV or a stomach tube. Your baby will stay in the hospital during this time. Recovery time could be longer if your baby was born premature or the operation was complicated.

Living with EA/TEF

Infants with EA/TEF often have another problem called tracheomalacia. This is a weakness and floppiness to the walls of the windpipe. It can cause their breathing to be noisy or sound high-pitched.

Some babies who have esophageal atresia also have other problems. These include:

  • Heart problems
  • Kidney problems
  • Stomach and bowel problems
  • Muscle and bone problems

Your doctor will do a physical exam of your baby. They may order more X-rays or ultrasound tests. These usually show if your baby has other problems. Babies with other problems might have to get those fixed first. Then they can have the surgery to fix the swallowing tube.

Babies born with esophageal atresia sometimes have long-term problems, including:

  • Gastroesophageal reflux disease, or GERD. This is similar to heartburn. It can usually be treated with medicine.
  • Scar tissue. Sometimes scar tissue grows where the esophagus connects to the stomach. This can make swallowing hard or painful. Food can’t easily get past it. Surgery may be necessary to open the scar tissue. This might require more tests or a procedure to see inside the esophagus and stomach.

Questions to ask your doctor

  • What tests and procedures are needed to make this diagnosis?
  • Are there other options besides surgery?
  • How long will my baby be in the hospital, and how long will it take them to recover?
  • What health condition is my baby at risk of, and how can I help prevent them?
  • Will my baby be in pain after surgery?
  • What medicine will help my baby recover? What are possible side effects?
  • How will I know if the baby has a heart defect?

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