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Tracheoesophageal fistula and esophageal atresia repair

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 carries 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 a connection between the upper part of the esophagus and the trachea or windpipe.

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) may be placed to give your child nutrition.
  • Your child will have another surgery later to repair the esophagus.

Sometimes the surgeon will wait 2 - 4 months before doing the surgery. Waiting 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.

Alternative Names

TEF repair; Esophageal atresia repair

Why the Procedure Is Performed

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 the stomach into the 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

Risks for any anesthesia include:

Risks for any surgery include:

Risks for this surgery are:

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

Before the Procedure

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 the Procedure

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:

  • Antibiotics as needed, to prevent infection
  • 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
  • Oxygen
  • Pain medicines as needed

If both the tracheoesophageal fistula and esophageal atresia are repaired:

  • A tube will be 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 a lot of encouragement.

If only the tracheoesophageal fistula was repaired and not the esophageal atresia, a gastrostomy 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 gastrostomy tube.
  • You will also be sent home with an extra gastrostomy tube. The nurses will inform a home health supply company of your equipment needs.

How long your 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 he or she is taking feedings by mouth or gastrostomy tube, and is gaining weight.

Outlook (Prognosis)

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 occurs 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.

References

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.

Updated: 1/30/2010

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.


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