Chromosomal and Genetic Conditions

Tracheoesophageal Fistula and Esophageal Atresia

What is TEF/EA?

TEF/EA

(Left illustration) Normal anatomy (Right illustration) Esophageal atresia with distal tracheoesophageal fistula

Tracheoesophageal fistula (TEF) (pronounced TRAY-KEY-o-i-SOF-uh-JEAL FIS-chu-luh) is a condition in which an abnormal channel, called a fistula, connects the windpipe (trachea) to the tube that leads from the mouth to the stomach (esophagus). Food and saliva can get into the trachea and lungs through this channel. This can make a child cough or choke and lead to lung infections or pneumonia.

Esophageal atresia (EA) (pronounced i-SOF-uh-JEAL uh-TREE-zhuh) is a condition where the esophagus does not form completely. This usually 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.

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

TEF/EA can take several forms when they occur together. In the most common form, the upper part of the esophagus has a closed end and the lower part of the esophagus connects to the trachea. About 85% of children with TEF/EA have this form.

Doctors are not yet sure what causes TEF/EA. It is not thought to be inherited or passed from parent to child.

TEF/EA in children

TEF and EA are present when a child is born (congenital). About 1 in 4,000 children are born each year with TEF, EA or both. About one-third of these children are born early.

TEF/EA at Seattle Children’s

The experts you need are here

Children with TEF/EA need special care from a variety of providers, including surgeons, pediatric anesthesiologists, gastroenterologists, neonatologists and nutritionists.

At Seattle Children’s, you will be connected with the region’s most experienced team of doctors, who work together to meet your baby’s needs. We see many children with this condition because doctors from several states send patients to Seattle Children’s for TEF/EA treatment.

Symptoms of TEF and EA

Babies who have TEF but not EA usually don’t have symptoms at birth, and it can take doctors several weeks to diagnose the condition. Babies with TEF usually only have mild coughing or respiratory symptoms. Those symptoms usually happen while the babies are feeding. Signs include:

  • Coughing often while feeding
  • Frequent lung infections

When babies have both TEF and EA, the symptoms are usually obvious right after birth. The most common signs of combined TEF/EA are:

  • Breathing problems
  • Coughing or choking when babies try to swallow

  • Babies with TEF/EA may have other problems that are linked to the conditions. Doctors call this the VACTERL association. Each letter stands for a possible problem:

    • V = vertebral, problems with the bones in the spine
    • A = imperforate anus, a problem with the way a baby’s anus or rectum has formed
    • C = cardiac, problems with the heart
    • TE = tracheoesophageal fistula and esophageal atresia
    • R = renal (kidney) problems
    • L = limb, problems with the bones in the arms

    All babies with TEF/EA will need evaluations to see if they have any of these problems. These babies may need:

    • X-rays to check for spinal problems and problems with arms or legs
    • Ultrasound (echocardiogram, or echo) to check for heart problems
    • Kidney ultrasound and possibly other tests of their kidneys
    • Treatment for problems with how the anus is formed (imperforate anus)

    If your baby has any of these problems, the doctor will explain which tests or treatment they need.

Diagnosing Tracheoesophageal Fistula and Esophageal Atresia

  • Sometimes doctors can diagnose TEF/EA before your baby is born based on the results of a prenatal ultrasound. Our Prenatal Diagnosis and Treatment Program can help your family and your obstetricians get ready for the birth and plan your baby’s care.

  • After your baby is born, doctors diagnose TEF/EA by trying to insert a nasogastric tube (NG tube). The tube is supposed to pass through the baby’s nose, down the esophagus and into the stomach.

    In children who have EA, the NG tube hits a blocked end, usually in the mid-chest. Doctors can see this blockage on an X-ray. Usually they need no other diagnostic test.

    In children who have both EA and TEF, the X-ray also can show gas in the baby’s stomach. Because the esophagus has not formed properly, the only way gas can get into the stomach is through a fistula (the abnormal channel between the esophagus and trachea). Children who have only EA do not show gas in the stomach.

  • To find TEF without EA, doctors sometimes use a barium swallow test. The doctor has your child swallow a chalky liquid called barium. The barium helps the digestive tract show up on X-rays.

Treating Tracheoesophageal Fistula and Esophageal Atresia

TEF and EA affect only about 1 in 4,000 children a year, but Seattle Children’s sees about 10 to 15 children with these conditions each year. Our doctors and surgeons are very experienced at repairing this condition and helping your family through treatment. We are the only hospital in the region where patients with TEF/EA can get care from a team of specialists with expertise in all the problems these babies may have.

Surgery options

Most babies with TEF and EA will have surgery soon after they are born. Some babies who have other associated problems may need to wait a short time before having surgery.

The type of surgery depends on the baby’s condition. In many cases, surgeons correct the problem with one surgery. Other times, doctors need to correct the condition in stages. Your baby’s surgeon will go over the possibilities and options with you.

Your child will need anesthesia for the surgery. Our pediatric anesthesiologists are specially trained to give your child medicine to make them safely sleep without pain during their surgery.

  • The surgeon will make a cut, or incision, in your child’s chest, usually on the right side. Then the surgeon will find and cut through the abnormal channel (fistula) between the windpipe and esophagus and sew it closed. If the ends of the esophagus are close enough to each other, the surgeon will repair the tube by sewing the ends together.

    If the ends of the esophagus are too far apart to attach safely, the surgeon will repair the fistula connected to the trachea (windpipe), but not the esophagus. The surgeon will place a feeding tube in your child’s stomach. This is called a gastrostomy (pronounced gas-STRAH-stuh-mee). The surgery to repair the esophagus will be done later. Your surgeon will discuss the options with you.

    Surgery for TEF/EA usually takes 2 to 3 hours.

    When your baby leaves the operating room, they will probably need a machine to temporarily help them breathe (ventilator). Your baby may need to use the ventilator for several days after surgery until they have recovered enough to breathe on their own. We will care for your baby in our Neonatal Intensive Care Unit (NICU) both before and after the surgery.

    Seattle Children’s is the first hospital in Washington to have a Level IV NICU – the highest level of care for your baby.

  • If a baby has TEF but not EA, doctors can usually repair it through a small incision in the baby’s neck. The surgeon will find the fistula, divide it and sew the trachea and esophagus closed.

  • If a baby has EA but not TEF, doctors may wait until the baby grows larger to attempt to sew the esophagus together. This can let the esophagus grow and bring the 2 ends closer together.

    Sometimes, the 2 ends remain far apart. Several techniques can be used to repair or even replace the esophagus. Your surgeon will discuss these options with you.

    Your baby will need a gastrostomy to get nutrition until they have surgery to repair the esophagus.

  • A gastrostomy is a safe way to give your baby nutrition until they can eat by mouth. A surgeon makes an opening through the wall of your baby’s belly and places a tube into the stomach. Your baby receives nutrients through this tube. This tube also lets doctors give your baby medicine if needed. 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.

    Your surgeon will take out the gastrostomy after your baby has surgery to repair the esophagus. Some babies will need the gastrostomy tube for several months or even longer. This is because they need to learn to swallow with their new esophagus.

  • After surgery, we will give your child pain medicine to make them comfortable. Your baby will get fluids and nutrition through an IV (intravenous) line, a tube that goes into a vein.

    About a week after your baby’s surgery, we will have your child swallow a liquid called barium and take X-rays (barium swallow test). This helps doctors check for leaks at the spot where the esophagus was repaired. If there is no leak, your baby may be able to start feeding by mouth.

    Most babies start feeding slowly. They may not be able to get the nutrition they need by mouth for several days or weeks. Some babies need supplemental feedings through a feeding tube passed through the nose 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 is simple, they may need to stay for a little more than 2 weeks. Your baby may need to stay in the hospital for several more weeks or months if they have other health problems, if they have an esophageal atresia with no fistula or if doctors have to wait to do surgery.

  • About 1 to 3 weeks after your baby leaves the hospital, they 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. Your baby will need follow-up appointments to make sure everything is working well. We continue to monitor and support your child as they grow.

    Your child’s follow-up care will be coordinated by our Surgery Pulmonary Follow-Up (SPF) Clinic.

    As children with TEF/EA grow, they can eat fairly normally, but may need to eat more slowly and chew more than people who did not have TEF/EA.

    The most common long-term problem for babies with TEF/EA is a condition called gastroesophageal reflux. We usually can treat this with medicine. Children with TEF/EA also might get more colds and other respiratory illnesses than other children.

Contact Us

To learn more about treatment for TEF/EA at Seattle Children’s, call our General and Thoracic Surgery Department at 206-987-2794, ext. 4.