What is Esophageal Atresia and Tracheoesophageal Fistula?

Visitors at this website will likely have some knowledge of EA/TEF, nevertheless an introduction may prove useful. There is a lot of background information available (reference 1).

Infants born with esophageal atresia (EA) have a portion missing (a gap) in their esophagus, the tube that goes from the back of the mouth to the stomach. With part of the esophagus missing, the baby cannot swallow food or even its own saliva. These defects are discovered either before birth by ultrasound examination or very shortly after birth in the delivery room. The reason these birth defects occur is unknown but, luckily, it rarely recurs in the immediate family.

EA takes many forms and there is a lot of variation in this group of defects. The spectrum of the defects including the five main types, are shown in Figure 1. The approximate percentages of each type are shown. Many additional variations, however, can occur. Because of the many children who have come here from outside our area, we treat a much higher percentage of long gap EA (type a). Overall, our series has had 20% pure EA (type a) and more recently it has been even higher.

Figure 1. Diagram of the main types of EA with or without a tracheoesophageal fistula (TEF). Type a is EA without a TEF and is often called pure EA. Shown are the blind upper and lower esophageal pouches next to the ringed windpipe (trachea) and the branches (bronchi) which lead to each lung. Type b has a connection (fistula) between the upper pouch and the trachea (a TEF). Type c is by far the most common form of EA and has a fistula between the lower esophagus and the trachea (one form of TEF) with a blind upper pouch. A rare form (1%) is type d with two TEFs, one between both the upper and lower esophageal segments and the trachea. Type e has only a TEF and no EA. This is usually referred to as an H or N shaped fistula and may be 2-4% of this group. The H-fistulas are divided surgically and nothing further needs to be done to the esophagus which is intact and reaches normally to the stomach.

Most of these babies (about 85%) will have an upper esophageal pouch that ends blindly and a lower esophageal segment that connects into the trachea (Figure 1c). For 8-10%, however, there will not be a connection between esophagus and trachea (no TEF). This is considered to be pure esophageal atresia (EA) with two blind esophageal ends (Figure 1a). For these babies, the distance between the two esophageal ends is usually longer (a long gap EA). Similarly, when the TEF is only between the upper pouch and the trachea, the lower end of the esophagus tends to be short leaving a long gap between the ends (Figure 1b).

For all the babies with EA, with or without a TEF, the missing segment between the two ends of the esophagus must be connected for normal eating. If the EA repair cannot be carried out within a matter of days, a gastric tube is usually placed directly into the stomach. This allows the child to be fed until the ends of the esophagus are put together and the baby is able to take food by mouth.

University of Minnesota Medical School