Esophageal Atresia/Tracheoesophageal Fistula

Esophageal atresia (EA) is a disorder of the digestive system in which the esophagus does not develop properly. This occurs in the womb before birth. Parents usually discover it when they try to feed their child and the child chokes, coughs and turns blue. These babies also throw up what they do manage to eat. Other signs are an unusually large amount of saliva so that babies drool a lot, being fussy and not eating a lot (even though they are hungry), and gagging. These children frequently develop pneumonia or respiratory problems because they breathe food and saliva into the lungs until the condition is discovered.

The esophagus is the tube that normally carries food from the mouth to the stomach. In most cases of esophageal atresia, the upper esophagus “dead ends” and does not connect with the lower esophagus and stomach. Picture a garden hose that someone has cut in two. Sometimes, the bottom half of the esophagus instead hooks up to the trachea (or windpipe). This is called tracheoesophageal fistula. No one knows why this happens, but it is one of the most common esophageal birth defects.

While the mother is still pregnant, if the obstetrician notices a lot of extra amniotic fluid, he or she may order a test to check for EA. After the baby is born, as soon as EA is suspected, a small feeding tube is inserted through the baby’s mouth or nose into the stomach. If the baby has EA, the tube cannot be passed into the stomach. An X-ray of the stomach shows an air-filled pouch and air in the stomach and intestines. If a feeding tube was inserted, it will be coiled up in the upper esophagus.

Surgery is the only treatment, and it is considered an emergency – the child is starving. As soon as the baby is stable, surgery should be performed. The doctors and nurses are very careful before surgery to make sure that the child doesn’t eat and that there is no fluid in the lungs. The surgery connects the two ends of the “garden hose,” the esophagus, so that the child’s food will go to the stomach and not to the lungs. If there isn’t enough esophagus to use, some tissue will be removed from the large intestine to join them. Surgery usually is successful.

As with any operation, the surgery involves risk of bleeding, infection and damage to other areas near the surgery site. After surgery, reflux (gagging) is common, and the baby will be closely monitored for pneumonia and other complications. The baby will have an intravenous tube to keep him or her hydrated while he or she cannot drink. A tube also will be placed in the mouth to remove spit and mucus.
