Tracheoesophageal fistula and esophageal atresia repair is surgery to repair two birth defects in your esophagus and trachea. Your esophagus is the tube that carries food from the mouth to the stomach. Your trachea, or windpipe, is the tube that normally air to the lungs.

These defects usually occur together:

• Esophageal atresia is when the upper part of the esophagus does not connect with the lower esophagus and stomach.
• Tracheoesophageal fistula (TEF) is when the upper part of the esophagus connects to the trachea or windpipe, instead of to the lower esophagus and stomach.

This surgery is almost always done soon after birth. Surgery is done while the child is in a deep sleep and pain-free from general anesthesia.

Tracheoesophageal fistula and esophageal atresia can usually be repaired at the same time.

• The surgeon will make a cut on the right side of the chest between the ribs.
• The fistula, which is the abnormal connection between the esophagus and windpipe, is closed off.
• Then the upper and lower portions of the esophagus are sewn together.

If the 2 parts of the esophagus are too far apart, then:

• Only the fistula will be repaired during the first surgery.
• A gastrostomy tube (a tube that goes through the skin into the stomach (also called a g-tube) may be placed to give your child nutrition.
• Your child will have another surgery later to repair the esophagus.

Sometimes the surgeon will want to wait 2 to 4 months before doing the surgery. Waiting a little allows your baby to grow or have other problems treated. If your child's surgery is delayed:

• A gastrostomy tube will be placed. The gastrostomy tube is often placed using numbing medicines (local anesthesia) so that your baby does not feel pain.
• During this time, the doctor may want to widen your baby’s esophagus with a special instrument called a dilator. This will make the future surgery easier.

Tracheoesophageal fistula and esophageal atresia are life-threatening problems and need treatment right away. If these problems are not treated:

• Your child may breathe saliva and fluids from their stomach into their lungs. This is called aspiration. It can cause choking and pneumonia (lung infection).
• Your child cannot swallow and digest food safely, or at all.

Risks for any anesthesia include:

• Reactions to medicines
• Breathing problems

Risks for any surgery include:

• Bleeding
• Infection

Risks for this surgery are:

• Narrowing of the repaired organs
• Food leakage from the area that is repaired
• Re-opening of the fistula
• Collapsed lung (pneumothorax)
• Hypothermia (low body temperature)

Your child will be admitted to the neonatal intensive care unit (NICU) or infant care center (ICC) as soon as the doctors diagnose either of these problems.

Your baby will receive nutrition by vein (intravenous, or IV) and may also be on a breathing machine (ventilator). The nurses may use suction to keep fluids from going into the lungs.

Some infants who have a low birth weight or other birth defects may not be able to have surgery until these other problems have been treated or have gone away.

After surgery, your child will be cared for in a hospital's NICU. Your child will be placed in a special bed called an isolette (incubator), which provides warmth and helps prevent infection.

Additional treatments after surgery usually include:

• Oxygen
• Breathing machine (ventilator)
• Chest tube (a tube through the skin into the chest wall) to drain fluids and keep the lungs open
• Intravenous (IV) fluids, including nutrition
• Antibiotics as needed, to prevent infection
• Pain medicines as needed

If both the tracheoesophageal fistula and esophageal atresia are repaired:

• A tube was placed through the nose into the stomach (nasogastric tube) during the surgery.
• Feedings are usually started through this tube a few days after surgery.
• Feedings by mouth are started very slowly. The baby may need feeding therapy and lots of encouragement.

If only the tracheoesophageal fistula was repaired and not the esophageal atresia, a g-tube will be used for feedings until the atresia can be repaired.

• While your baby is in the hospital the nurses will show you how to use and replace the g-tube.
• You will also be sent home with an extra g-tube. The nurses will inform a home health supply company of your equipment needs.

How long an infant stays in the hospital will depend on what type of surgery your child needed. You will be able to bring your baby home once they are taking all their feedings by mouth or g-tube, and they are gaining weight.

Surgery can usually repair tracheoesophageal fistula and esophageal atresia. Most children who have had this repair grow up and have normal lives.

Once healing from the surgery is complete, your child may have these problems:

• The part of the esophagus that was repaired may become narrower. Your child may need to have more surgery to treat this.
• Your child may have heartburn or gastroesophageal reflux (GERD). This is when acid from the stomach goes up into the esophagus.

During infancy, some children will have problems with breathing, growth, and feeding, and will need to continue seeing both their primary care provider and specialists.

Orenstein S, Peters J, Khan S, Youssef N, Hussain SZ. Congenital anomalies: esophageal atresia and tracheoesophageal fistula. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 316.

Long JD, Orlando RC. Anatomy, histology, embryology, and developmental anomalies of the esophagus. In: Feldman M, Friedman LS, Sleisenger MH, eds. Sleisenger & Fordtran’s Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006:chap 40.