Tracheoesophageal Fistula and Esophageal Atresia

Our transport team is very experienced in safely moving fragile babies to Seattle Children’s from their birth hospital. In our Level 4 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 IV (intravenous) 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 (saliva) 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 (gas-STRAH-stuh-mee) tube.
• Repair your baby’s esophagus through aa series of procedures over time.
• 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 (gastromy) 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 IV (intravenous) 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 NC (nasogastric) 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. 
• If they have problems with stomach contents flowing backwards into the esophagus (gastroesophageal reflux or GER). With a feeding tube, your baby can still feed by mouth if they are able. As needed, they can get breastmilk/chestmilk or formula through the feeding tube.

Our nurses and lactation specialists will do all that we can to support you in giving your baby breastmilk/chestmilk. To keep your milk flowing and to build a milk supply for your baby, we help you with 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/chest.

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

Our Tracheoesophageal Fistula and Esophageal Atresia Program team, including specialists in pulmonology, gastroenterology, otolaryngology, nutrition and feeding, will give your child the follow-up care they need. 

• We help with any feeding problems. As children with TEF/EA grow, they may have difficulty swallowing liquids without it getting into their airway. They may need to eat more slowly and chew more than other people.  Speech language pathologists can recommend specific treatments based on  your child’s age and feeding abilities. 
• The most common long-term problem for babies with TEF/EA is stomach contents flowing backwards into the esophagus (gastroesophageal reflux or GER). This can be related to the structural shape of the esophagus. Foods your child eats can also cause reflux. The esophagus may be more sensitive and allergic to specific food groups. We often can treat this with medicine.
• We may need ongoing diagnostic pictures like esophagrams and a visual scope into the esophagus. If your child’s  reflux or difficulty eating and swallowing gets worse, your doctor will suggest treatment. They may need to stretch (dilate) the esophagus where the scar formed from their earlier surgeries.
• Children with TEF/EA might have a floppy airway. This can lead to more symptoms like coughing or trouble breathing. They may get more colds or other respiratory illnesses that seem to last longer or are more intense.  Preventing illness is incredibly important and requires routine involvement with the lung doctor (pulmonologist) with the TEF/EA team. 

Our team of experts will work with you to check your child’s health until they reach early adulthood. We provide ongoing support and resources for you and your child.