Chromosomal and Genetic Conditions

Tracheoesophageal Fistula and Esophageal Atresia

What is TEF/EA?

TEF and EA happen while a baby is growing during pregnancy. Most often, TEF and EA happen together. But sometimes a child has 1 condition and not the other. Each name refers to a specific problem.

Doctors are not sure what causes these rare problems. They are present when a child is born (congenital). TEF and EA are not thought to be passed from parent to child (inherited).

  • The word fistula (FIS-chu-luh) means “abnormal connection.” Tracheoesophageal (TRAY-kee-oh-ee-SAH-fuh-JEE-ul) fistula (TEF) happens when a baby’s windpipe (trachea) and food tube (esophagus) are connected.

    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 such as pneumonia.  

  • Esophageal atresia (EA) happens when the tube that leads from the mouth to the stomach (esophagus) does not form all the way before birth. The tube leading down from the mouth and the tube leading up from the stomach do not meet. The condition is pronounced ee-SAH-fuh-JEE-ul uh-TREE-zhuh.

    A baby with EA cannot feed by mouth because there is no way for food and liquid to travel from their mouth to their stomach.

  • TEF/EA

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

    TEF/EA can take several forms. In the most common form, the upper part of the food tube (esophagus) has a closed end and the lower part connects to the windpipe (trachea). Most children with TEF/EA have this form. It is called esophageal atresia with distal tracheoesophageal fistula.

  • Some babies have other problems that are linked to TEF and EA. The problems may affect a baby’s heart, kidneys, digestive tract or bones. 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 forms
    • C = cardiac, problems with the heart
    • TE = tracheoesophageal fistula and esophageal atresia
    • R = renal (kidney) problems
    • L = limb, problems with bones in the arms or legs

TEF/EA at Seattle Children’s

Seattle Children’s has the only team in the region with expertise in all the areas that babies with TEF/EA may need. Sometimes care starts before birth, if an ultrasound during pregnancy suggests your baby has TEF/EA.

If you would like an appointment, ask your pregnancy care provider to refer you to our Prenatal Diagnosis and Treatment Program. If you would like more information or have a referral, call 206-987-5629 to make an appointment. Providers, see how to refer a patient.

    • At Seattle Children’s we care for 10 to 15 new patients with these rare conditions each year. We follow many other children long-term. Doctors from several states refer babies with TEF/EA to us.
    • If doctors suspect TEF/EA during pregnancy, the team at our Prenatal Diagnosis and Treatment team can do a detailed assessment. We help you prepare for what lies ahead.
    • We work with your birth hospital to arrange the special care your baby will need at birth and as they travel by ambulance to Seattle Children’s soon after they are born.
    • Our transport team of physicians, nurses and respiratory therapists are national leaders in safely moving fragile babies.
    • Seattle Children’s has the state’s first Level IV Neonatal Intensive Care Unit (NICU), the highest level of care. We are skilled and equipped to care for the sickest newborns.
    • Our surgery team is very experienced in performing procedures to repair TEF/EA. More cases mean greater skill and better outcomes.
    • Your baby needs care designed just for them. Babies differ from adults in how they react to illness, pain and surgery. That’s why our surgeons are all board certified in pediatric surgery.
    • Along with a surgeon, your baby’s team will include experts in newborn care (neonatology), the digestive system (gastroenterology), airway problems (otolaryngology), pain control (anesthesiology), nutrition and lactation support. We work together and with you to meet your baby’s needs.
    • Our experts focus on how today’s treatment will affect your child as they develop and become an adult. We base treatment plans on years of experience and the newest research on what works best for children.
    • As your child grows, they will get care as needed from experts from surgery, gastroenterology and our Aerodigestive Program, which focuses on problems that affect both breathing and swallowing.
    • Some babies with TEF/EA have other health problems at birth. Seattle Children’s ranks among the best in all 10 pediatric specialties rated by U.S. News & World Report in 2018. We are the only hospital in the Northwest to do so.

     

    • Having a baby born with a serious medical problem can be scary. At Seattle Children’s, you have a full team behind you before, during and after surgery. We work together to meet all your child’s health needs.
    • We take time to explain your child’s condition and help you fully understand your treatment options.
    • Our doctors, nurses, child life specialists and social workers help your family through the challenges of TEF and EA. We connect you to community resources and support groups.
    • We work with many children and families from around the Northwest and beyond. We can help with financial counseling, schooling for children in the family, housing, transportation, interpreter services and spiritual care. Learn about our services for patients and families.
  • Seattle Children’s leads research in the lab and in the clinic to improve treatment and quality of life for children born with problems like TEF/EA.

    • Our newborn specialists are leaders in research to improve outcomes for babies born very early or with complex problems. See Pushing Limits, Saving Lives.
    • We work to find the safest, best ways to reduce stress in the NICU and to ease pain. Learn more about Seattle Children’s neonatology research.
    • Many children born with TEF/EA have feeding problems and gastroesophageal reflux (GER). Our research helps parents and doctors decide which treatment is best.
    • Our patients have the option to take part in research studies of promising new treatments. These are called clinical trials.

     

Symptoms of TEF and EA

  • Symptoms of TEF/EA: Breathing problems and coughing or choking when your baby tries to swallow. The symptoms show up right after birth.
  • Symptoms of TEF only: Severe coughing or choking when your baby tries to swallow, usually after feeding. Some babies may have trouble breathing while feeding. If your baby has TEF alone, it may be weeks or months after birth before TEF is diagnosed as the cause of symptoms.
  • Symptoms of EA only: Your baby is not swallowing their saliva, is drooling a lot or has foamy mucus in their mouth or nose. Most often, EA alone is diagnosed before birth or right after birth.

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.

  • If doctors suspect your newborn has TEF/EA, they try to pass a tube from your baby’s nose or mouth into their stomach. The tube is called a nasogastric tube or NG tube.

    If a baby has EA, the NG tube hits a blocked end, often in the mid-chest. Doctors can see this blockage on an X-ray. Often that is the only diagnostic test needed. Your baby would be moved from their birth hospital to Seattle Children’s right away.

    If a child also has TEF, an X-ray will show gas in the baby’s stomach. Because the esophagus has not formed properly in EA, the only way gas can get into the stomach is through an abnormal connection between the windpipe and esophagus. Babies who have only EA do not show gas in their stomach.

  • To find TEF without EA, doctors sometimes use a test called an esophagram. First, your child swallows a liquid (gastrograffin or barium). The liquid helps the tube from the mouth to the stomach (esophagus) show up on X-rays.

    The doctor may take a series of X-rays to look at the esophagus, stomach and the first part of the small bowel. First, we give your child a contrast liquid that shows up on X-ray. This test is called an upper GI (gastrointestinal) series (PDF).

  • All babies with TEF/EA will be checked for other problems that are sometimes related. These tests include:

    • X-rays and ultrasound to check for problems with bones in the spine, arms or legs
    • Ultrasound of their heart (echocardiogram or echo)
    • Ultrasound and possibly other tests of their kidneys
    • Exam to see how well the anus is formed

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

Treating Tracheoesophageal Fistula and Esophageal Atresia

Babies with EA, with or without TEF, will have some type of surgery soon after they are born. Surgery for TEF alone is done soon after it is diagnosed, but this can take several months.

The type of surgery depends on your baby’s condition. In many cases, surgeons correct the problem with 1 surgery. Other times, doctors need to correct it in stages. Your baby’s surgeon will talk with you about the best option for your child.

  • Our transport team is very experienced in safely moving fragile babies to Seattle Children’s from their birth hospital. In our Level IV Neonatal Intensive Care Unit (NICU), we will give your baby the highest level of care.

    We carefully assess your child to decide the best treatment for them. This includes any breathing support they need and the timing of surgery.

    About one-third of children born with TEF, EA or TEF/EA are born early. This may happen because babies with EA cannot swallow normal amounts of amniotic fluid and it builds up inside the mother’s womb. Premature babies may need breathing support and treatment for other health problems at birth.

    Before surgery, your baby’s doctors will:

    • Stabilize your baby’s health.
    • Give your baby fluids through an intravenous (IV) line into their vein.
    • Place a tube through your baby’s nose into their esophageal pouch. The tube helps keep air and fluid out of the pouch so your baby is less likely to breathe spit into their lungs.
    • Do an ultrasound of the heart (echocardiogram). This helps ensure the surgery can be done as safely as possible.
    • Give your child medicine to make them sleep without pain during the surgery (general anesthesia). Our doctors are board certified in pediatric anesthesiology, with extra years of training in how to give pain medicine to babies and children safely.
  • The surgeon will:

    • Make a cut (incision) in your child’s chest, usually on the right side
    • Find the abnormal channel (fistula) between the windpipe and esophagus
    • Cut through the channel and sew it closed

    If the ends of the esophagus are close enough to each other, the surgeon will join them with stitches.

    If the ends of the esophagus are too far apart to connect safely, the surgeon will not repair the esophagus. The surgeon will:

    • Place a feeding tube in your child’s stomach. This is called a gastrostomy (pronounced gas-STRAH-stuh-mee).
    • Repair your baby’s esophagus later.
    • Talk with you about the options.
  • If your baby has TEF but not EA, doctors most often repair it through a small cut in your baby’s neck. The surgeon will:

    • Find the fistula
    • Cut through it
    • Sew the trachea and esophagus closed
  • If your baby has EA but not TEF, the 2 ends of the esophagus are too far apart to sew together right away. In this case, your baby will need surgery to place a feeding tube through their belly wall. This is called a gastrostomy. It is how your baby will get nutrition until they have surgery to fix their esophagus.

    Doctors will wait until your baby grows larger before doing the surgery to sew the esophagus together. This gives the esophagus time to grow so the 2 ends may get closer together.

    Sometimes, the 2 ends stay far apart. There are different ways to repair or even replace the esophagus. Your surgeon will talk with you about these options.

  • A gastrostomy is a safe way to give your baby nutrition until they can feed by mouth. The surgeon:

    • Makes an opening through the wall of your baby’s belly
    • Places a G tube into their stomach

    Your baby receives nutrients through this tube, and medicine if needed.

    We will teach you how to take care of the tube and how to feed your child through it.

    Your surgeon will take out the G tube after your baby can eat by mouth. Some babies will need the G tube for several months or even longer. This is because they need to learn to swallow after esophagus repair.

  • After surgery for TEF/EA or EA, your baby will likely need a machine to help them breathe (ventilator). It may be a few days to a week or more before your baby can breathe well on their own. If your baby had surgery for TEF alone, they will only need a breathing machine during surgery.  

    After surgery, we will give your child:

    • Pain medicine for their comfort
    • Fluids and nutrition through an intravenous (IV) tube into a vein
  • About a week after your baby’s surgery, we will do an imaging study to check the esophagus repair for leaks. This is called an esophagram. If there is no leak, your baby may be able to start feeding by mouth.

    Most babies start feeding slowly. Your baby may not be able to get all their nutrition by mouth for several days or weeks. Some babies need extra feedings from a tube passed through their nose and into their stomach. This is called a nasogastric tube or NG tube. Babies who had a gastrostomy tube placed early in life will keep using that until they can take all their feedings by mouth.  

    How long your child will need some or all of their nutrition by tube depends on how well they swallow and if they have problems with stomach contents flowing backwards into the esophagus. This is called gastroesophageal reflux (GER). With a feeding tube, your baby can still feed by mouth if they are able and, as needed, get breast milk or formula through the feeding tube.

    Our nurses and lactation specialists will do all that we can to support you in giving your baby breast milk. To keep your milk flowing and to build a milk supply for your baby, we help you with breast pumping and storing your milk. When your baby is ready, our nurses and feeding therapists help with the transition to feeding by bottle or breast.

    Our nutritionists keep a careful watch on your baby to be sure they are growing well. Our feeding therapists help your child learn to feed by mouth.

  • The hospital stay for babies with TEF and EA varies. If your baby is healthy and the repair is simple, they will likely stay for 2 weeks or a little longer.

    Your baby may need to stay in the hospital for many more weeks if:

    • They have other health problems.
    • They have EA but not TEF.
    • Doctors have to wait to do surgery.

    Your baby will be ready to go home when they are breathing well and have a feeding plan to help them gain weight.

    Before your baby goes home, we will teach you:

    • How to care for the incisions
    • About medicine they need
    • How to feed your child using a tube in their nose (NG tube) or stomach (G tube)
    • Signs of problems to watch for

    Our nurses will help set up any supplies and equipment you need. You will have plenty of time to practice all your baby’s care before going home.

  • About 1 to 3 weeks after your baby leaves the hospital, they will see the surgeon for a follow-up visit to make sure:

    • Your baby is breathing well.
    • Their incision is healing.
    • Your child is feeding, gaining weight and growing well.
    • There are no other health problems.

    Experts in our Surgery, Gastroenterology, Nutrition and Aerodigestive programs will give your child follow-up care they need. Our Aerodigestive Program cares for children with ongoing airway and feeding problems. Children who had EA/TEF repaired at Seattle Children’s and at other hospitals come to us for follow-up care.

    We continue to keep a watch on your child’s health until they reach college age.

    • We help with any feeding problems. As children with TEF/EA grow, they 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 stomach contents flowing backwards into the esophagus. This is called gastroesophageal reflux (GER). We often can treat this with medicine. Rarely, surgery is needed.
    • Children with TEF/EA might get more colds and other respiratory illnesses than other children.

Contact Us

If you would like an appointment, ask your pregnancy care provider to refer you to our Prenatal Diagnosis and Treatment Program. If you would like more information or have a referral, call 206-987-5629 to schedule an appointment.

Providers: See how to refer a patient