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Tracheoesophageal fistula and esophageal atresia repair

Tracheoesophageal fistula and esophageal atresia repair is surgery to repair two birth defects in the esophagus and trachea.

The esophagus is the tube that carries food from the mouth to the stomach. The trachea, or windpipe, is the tube that carries air into and out of the lungs.

These defects usually occur together:

  • Esophageal atresia (EA) occurs when the upper part of the esophagus does not connect with the lower esophagus and stomach.
  • Tracheoesophageal fistula (TEF) is an abnormal connection between the upper part of the esophagus and the trachea or windpipe.

This surgery is almost always done soon after birth. Both defects can usually be repaired at the same time. Briefly, the surgery takes place this way:

  • Medicine (anesthesia) is given so that the baby is in a deep sleep and pain-free during surgery.
  • The surgeon makes a cut on the right side of the chest between the ribs.
  • The fistula between the esophagus and windpipe is closed.
  • Then the upper and lower portions of the esophagus are sewn together.

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

  • Only the fistula is 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 to 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 through the abdominal wall into the stomach. Numbing medicines (local anesthesia) will be used so that the baby does not feel pain.
  • At the same time the tube is placed, the doctor may widen the 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. They need to be treated 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 of anesthesia include:

Risks of surgery include:

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

Before the Procedure

Your baby will be admitted to the neonatal intensive care unit (NICU) 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 are premature, have a low birth weight, or have other birth defects beside TEF and/or EA 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 the hospital's NICU. Your child will be placed in a special bed called an 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 TEF and EA are repaired:

  • A tube is 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 slowly. The baby may need feeding therapy.

If only the TEF is repaired, a gastrostomy tube is 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 depends on the type of surgery your child has. 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 a TEF and EA. 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. GERD may cause breathing problems.

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

References

Khan S, Orenstein SR. Congenital anomalies. In: Kliegman RM, Stanton BF, St. Geme JW III, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 311.

Song C, Upperman JS, Niklas V. Structural anomalies of the gastrointestinal tract. In: Gleason CA, Devaskar SU, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 69.

Updated: 1/31/2014

Kimberly G. Lee, MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.


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