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.
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