TEF repair

Alternative names
Tracheoesophageal fistula and esophageal atresia repair; Esophageal atresia repair

Definition
This surgery is used to repair two congenital defects of the airway and the digestive tract: tracheoesophageal fistula (TEF) and esophageal atresia. These defects usually occur together, but they may occur separately.

Description

In early fetal development, the trachea (airway) and upper portion of the digestive tract fail to develop normally in babies with TEF. In children with esophageal atresia, the esophagus is interrupted and ends in a blind pouch. In most cases, the lower portion of the esophagus is abnormally connected to the airway (trachea) by a small channel or tube called a fistula. There are five basic anatomic variations of TEF, but management is similar for all types.

This congenital anomaly is thought to occur in 1 in 1,500 to 3000 infants. Boys and girls are affected equally. Associated congenital anomalies of the heart, gastrointestinal tract, and nervous system are common.

Surgery is performed while the child is under general anesthesia (unconscious and pain-free). An incision is made in the right chest between the ribs (thoracotomy). The tracheoesophageal fistula is closed off, then the upper and lower portions of the esophagus are sewn together.

In some cases, infants are not suitable candidates for immediate repair. This is the case if other congenital anomalies are present that contribute to severe illness, or if the infant was born prematurely, with a low birth weight. These infants undergo surgical repair after their other problems have resolved.

In cases where surgery will be delayed, a venting gastrostomy tube is placed in the stomach to decompress the stomach and prevent fluid from going up the esophagus and into the trachea. The gastrostomy tube is often placed using local anesthesia.

Certain types of TEF in otherwise healthy infants are simpler to repair after a period of time has passed to allow the baby to grow. During this period, which usually is 2 to 4 months, periodic dilations of the underdeveloped esophagus may be performed to enlarge it and make subsequent repair easier. Such infants will require a venting gastrostomy tube for decompression of the stomach and feedings until repair can be accomplished.

Indications
These are life-threatening problems requiring immediate intervention. Saliva and gastric secretions may be aspirated into the lungs through the abnormal opening in the trachea. Normal swallowing and digestion of food cannot occur with the abnormal esophagus.

Risks
Risks for any anesthesia include the following:

     
  • Reactions to medications  
  • Problems breathing

Risks for any surgery include the following:

     
  • Bleeding  
  • Infection

Additional risks include the following:

     
  • Narrowing of repaired organs  
  • Collapsed lung (pneumothorax)  
  • Hypothermia

Expectations after surgery
This problem can usually be corrected surgically. If the two portions of the esophagus are too short, further reconstruction may be necessary and a gastrostomy tube may be temporarily inserted for feedings.

Convalescence

The infant will be cared for before and after the surgery in a neonatal intensive care unit. The baby is placed in an isolette (incubator) to keep warm and prevent infection. Oxygen and/or mechanical ventilation may be necessary. A chest tube may be placed to drain fluids. Intravenous fluids and pain medications will be given.

Feedings will be started as soon as possible, depending on the extent of the repair. The length of hospitalization varies, depending on the seriousness of the surgery and the baby’s condition.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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