Skip to main content.

Tracheoesophageal Fistula (TEF) and Esophageal Atresia (EA)

What are tracheoesophageal fistula and esophageal atresia?

The word “fistula” means “abnormal connection.” In tracheoesophageal fistula (TEF), a channel connects the windpipe (trachea) to the tube from the mouth to the stomach (esophagus). Food and saliva can get into the trachea and the lungs through this channel. This can cause coughing or choking or lead to lung infections or pneumonia.

Esophageal atresia (EA) is when the esophagus does not completely form. Usually this means the tube leading down from the mouth and the tube leading up from the stomach do not meet. Instead, each section of tube has a closed end. A baby with EA cannot eat or drink by mouth because there is no way for food and liquid to travel from the mouth to the stomach.

There are several types of tracheoesophageal fistula and esophageal atresia. In the most common form, the upper part of the esophagus has a blind ending, and the lower part of the esophagus connects to the trachea. About 85% of children with TEF/EA have this form.

People often use the terms TEF and EA interchangeably. But each name indicates a specific problem. TEF and EA usually occur together, but sometimes a child has just one and not the other.

Who gets TEF and EA?

Both conditions are present at birth (congenital). About 1 in 4,000 children are born each year with TEF or EA or both. About one-third of these children are born early.

What are the symptoms?

The most common signs of TEF/EA are breathing problems or coughing or choking when your baby tries to swallow. Babies who have only TEF, not EA, usually have only mild coughing or respiratory symptoms with feeding. Many things can cause these symptoms, so it may take doctors some time to diagnose the TEF.

How do you diagnose TEF and EA?

Sometimes doctors are able to diagnose TEF/EA before birth based on a prenatal ultrasound. After birth, doctors can diagnose TEF/EA by trying to insert a nasogastric, or NG, tube. This is a tube that passes through the baby’s nose, down the esophagus and into the stomach.

  • In children who have EA, the NG tube hits a blind end, usually in the mid chest. This can be seen on a plain x-ray. Usually no other diagnostic test is needed.
  • In children who have both EA and TEF, the plain X-ray will also show gas in the stomach. Because the esophagus has not formed normally, the only way for gas to get into the stomach is through a fistula. So gas tells us a baby with EA also has a fistula. (In children with only EA, there is no gas in the stomach.)

In children who have only TEF and no EA, there are usually no noticeable problems at birth. The diagnosis is usually made several weeks or months later after noticing that the baby coughs frequently with feeding or has frequent lung infections. Special tests, like a special type of barium swallow, are needed to diagnose this. Barium is a chalky liquid that makes the digestive tract shows up in an X-ray.

Problems sometimes linked to TEF/EA

Babies with TEF/EA may have certain associated problems. This is commonly referred to as the VACTERL association. Each letter stands for a possible problem. (V=vertebral, A=imperforate anus, C=cardiac, TE=tracheoesophageal fistula and esophageal atresia, R=renal, L=limb.) All babies with TEF/EA will need a series of evaluations to check whether they have any of these other problems.

All will need X-rays to check for spinal problems. Some will be born with no anus and will need treatment for this. All will need an ultrasound of the heart (echocardiogram, or ECHO) to check for cardiac problems, and a kidney ultrasound and possibly other tests of their kidneys. Some may have limb problems, usually problems with their arms. Plain X-rays may be all that are needed to assess limb problems. If any of these problems are found, your surgeon will explain any treatment your baby needs.

What is your experience with TEF and EA at Children’s?

We treat many children with TEF/EA at Children’s. We usually see 10 to 15 children every year with one or both of these conditions.

When you come to Children’s, you have a team of people to care for your child before, during and after surgery. Along with your child’s surgeon, you are connected with doctors trained to care for new babies with complex problems (neonatologists), nurses, dietitians, child life specialists, social workers and others. We work together to meet all of your child’s health needs and help your family through this experience.

Since 1907, Children’s has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.

How do you treat TEF and EA?

Surgery is the only treatment for TEF and EA. Most babies will have surgery soon after birth. Some babies may need to wait a short time before having surgery if they have other associated problems, such as heart problems.

The exact choice of operation for your baby will depend on your baby’s condition. Your surgeon will be able to talk with you about the specifics once we know your baby’s needs. In many cases, surgeons correct the problem with one operation. Sometimes doctors need to correct the problem in stages depending on the condition of the baby or any associated problems. For some infants, this can be complex. Your surgeon will go over the possibilities with you.

Surgery

At the time of surgery, your child will have general anesthesia. This means we will give your child medicine to make them sleep without pain during the surgery.

In babies who have both TEF and EA (the most common situation), the surgeon will make a cut, or incision, in your child’s chest, usually on the right side. Then surgeon will cut through the fistula and sew it closed. Next the surgeon will repair the esophagus by sewing the two ends together, if they are close enough for this to be done safely. The surgery usually takes about 2 to 3 hours. All of these babies will leave the operating room still on the breathing machine (ventilator) and may be ventilated for several days after surgery. All of these infants will be in the intensive care unit before and after surgery.

If the ends of the esophagus are too far apart to attach safely, the surgeon will repair the fistula but not the atresia during the first surgery. The surgeon will also place a feeding tube in your child’s stomach. This is called a gastrostomy (pronounced gas-STRAH-stuh-mee). This is done through a separate small incision in the baby’s belly. Then the baby will be allowed to grow for a while so the esophagus gets longer and is easier to sew together in the future, usually one to four months later.

Babies who have atresia without a fistula will also be allowed to grow for a while so the esophagus gets longer and is easier to sew together in the future. In the meantime, these babies will need a gastrostomy in order to get nutrition until after their atresia is repaired.

If your child has TEF and no atresia, the repair is done very differently. It can usually be done through a small incision in the baby’s neck. The surgeon will find the fistula, divide it, and sew the trachea and esophagus closed.

Gastrostomy

For a gastrostomy, the surgeon makes an opening through the wall of your child’s belly and places a tube into the stomach. This provides a safe way to give your child nutrition (and medicine, if needed) until they can feed by mouth. We will teach you how to take care of the tube and the skin around it, and how to feed your child through the gastrostomy. The surgeon will take out the gastrostomy after your child has surgery to repair the esophagus, this has time to heal, and your child can take all nutrition by bottle. This is usually at least several months later on and often longer.

After surgery

After surgery, we will give your child pain medicine to make them comfortable. They will not be able to eat by mouth at first and will get fluids and nutrition through an IV (short for intravenous) line, a tube that goes into a vein. Many babies will need a special IV called a PICC line.

If your child had surgery to connect the esophagus, we will give them a liquid called barium and do a swallow test after five to seven days. This test is to check for any leaks where the esophagus was repaired. If there is no leak, your baby can start feeding by mouth. Most babies will start slowly and may not be able to take all the nutrition they need by mouth for several days or weeks. Some babies will need supplemental feedings given by a small feeding tube passed through the nose, down the esophagus and into the stomach.

The time babies need to stay in the hospital after surgery for TEF and EA varies. If your baby is healthy and the repair was simple, they may need to stay for a minimum of two weeks. If your baby has other health problems, if your baby has a pure esophageal atresia with no fistula, or if the repair was delayed, your baby may need to be in the hospital for weeks to months.

About one to three weeks after discharge from the hospital, your child will need to see the surgeon for a follow-up visit. The surgeon will make sure the incision is healing and your child is recovering, gaining weight and growing properly. Besides this appointment, you may need frequent follow-up appointments with the doctor to check that everything is working well. The most common long-term problem for babies with TEF/EA is a condition called gastroesophageal reflux. We can usually treat this with medicine. As they grow older, children with TEF/EA will usually find they can eat normally. But they probably need to eat more slowly and chew more thoroughly than people who did not have TEF/EA.

Resources

Read more about what to expect when your child visits a clinic at Children's, has surgery here or needs to stay in the hospital. You can get practical details about topics like what to do and bring the day of surgery and who will be on your child's care team. You can also take a virtual tour of our surgery rooms and other parts of the hospital.

To learn more about this condition, you may want to visit our resource list.