Esophageal Atresia and Tracheoesophageal Fistula

What is esophageal atresia and tracheoesophageal fistula?

An esophageal atresia (EA) and a tracheoesophageal fistula (TEF) are 2 disorders of the digestive system. They happen in babies before the babies are born. This is called a congenital defect. The 2 disorders often happen together.

There are several types of EA. In most cases of babies who have it, the esophagus doesn’t connect to the stomach. It just ends in a pouch. So nothing the baby swallows gets into the stomach.

A fistula is when 2 of the body’s tubes are connected. A tracheoesophageal fistula is when the trachea (the windpipe) and the esophagus (the swallowing tube) are connected. These tubes aren’t normally connected. When they are connected, food or milk can get into your baby’s lungs when he or she swallows. This can cause breathing problems and even pneumonia.

Are these problems common?

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

Symptoms of EA/TEF

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

  • feeding problems right away
  • drooling or spitting up a lot
  • bubbly mucus in the mouth
  • coughing, gagging, or choking when feeding
  • bluish color to the skin when feeding
  • difficulty breathing.

For most babies with EA, symptoms usually occur 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 yet sure exactly what causes these problems. In the womb, your child’s esophagus and trachea started to grow from the same bit of tissue. Sometimes the tubes don’t develop correctly. It’s not thought that these problems are inherited.

How is EA/TEF diagnosed?

The mother’s ultrasound could show too much amniotic fluid. This is a clue that the baby may have a blockage in their digestive system. The blockage could be EA.

Symptoms of EA/TEF usually happen shortly after birth. If your doctor suspects EA, he or she can try inserting 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 help confirm the diagnosis.

An X-ray can also help confirm TEF.

Can EA/TEF be prevented or avoided?

There is nothing a pregnant mother can do to prevent her baby from developing EA/TEF in the womb. 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. Then, if a fistula is connecting the esophagus to the trachea, it must be closed. Your child’s doctor will decide when to do the surgery. Healthy, full-term babies can have the surgery when they are just a few days old. If the baby has any other problems (such as pneumonia or other birth defects), surgery may have to wait.

How long will my baby be sick?

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 premature or the operation was complicated.

Living with EA/TEF

Does my baby have any other problems?

Infants with EA/TEF often have a weakness and floppiness to the walls of their windpipe. This is called tracheomalacia. 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. He or she may order more X-rays or ultrasound pictures. 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.

Will my baby have other problems in the future?

Babies born with esophageal atresia sometimes have long-term problems. The most common of these is gastroesophageal reflux disease, or GERD. GERD is similar to heartburn, and can usually be treated with medicine.

Another problem is scar tissue. Sometimes scar tissue grows where the esophagus connects to the stomach. This scar tissue can make swallowing hard or painful because the food can’t easily get past. Sometimes another surgery is needed to open the scar tissue. Your child may need more X-rays or an endoscopy. In this procedure, a narrow tube holding a tiny camera is put into your child’s esophagus. The pictures help your doctor see inside the esophagus and stomach.

Questions to ask your doctor

  • Are there other options besides surgery?
  • How long will my baby be in the hospital, and how long will it take her to recover?
  • What health condition is my baby at risk of, and how can I help prevent them?
  • What caused this condition? Was it inherited?
  • Will my baby be in pain after surgery?
  • What medicine will help my baby recover? What are possible side effects?