A tracheoesophageal fistula (TEF) is an abnormal connection in one or more places between the esophagus (tube going from the throat to the stomach) and the trachea (tube going from the throat to the windpipe and lungs). These are normally two separate tubes that are not connected. In an infant with a TEF, the lower esophagus connects with the airway. Reflux from the stomach (gastric juices) can thus pass through the lower part of the esophagus, through the fistula, and into the lungs. This can cause pneumonia and other respiratory problems.
In premature infants who have underdeveloped lungs and a TEF, air may go from the trachea into the esophagus and stomach, causing severe abdominal distension (swelling) and possible stomach rupture.
Esophageal Atresia
TEF often occurs along with another birth defect known as esophageal atresia. With esophageal atresia, the esophagus does not form properly in the developing fetus. This results in two segments, one that connects to the throat and the other that connects to the stomach. However, the lumens (hollow centers) of these two segments do not connect to each other. The upper and lower ends of the esophagus thus exist as blind pouches. When an infant feeds, the upper pouch fills up and liquid overflows into the trachea, causing severe respiratory problems. This overflow into the trachea is referred to as aspiration.
Esophageal atresia takes many forms, and there is a lot of variation in this group of defects. However, the spectrum of the defects includes five main types. The most common type (80%) has a fistula between the lower esophagus and the trachea; the upper part is a short segment of the esophagus that does not connect with any other structure. The second most common type (10% to 12%) of defect is esophageal atresia without a TEF; this is referred to as pure esophageal atresia. It comprises two ends of the esophagus that do not communicate with each other or with the airway.
Show AllAs a fetus grows, different organ systems develop and mature. Although adjacent to each other, the trachea and the esophagus develop as two separate tubes. When the wall between them does not form properly, TEF and/or esophageal atresia can occur. About 1 in 3000 to 5000 babies in the US is born with one or both of these problems.
These two conditions are not thought to be inherited, and cases in which multiple family members are affected are rare.
Babies with other birth defects often have a TEF and/or esophageal atresia. Birth defects that often occur with these two conditions are:
- Trisomy 13, 18, or 21
- Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia, or imperforate anus
- Heart problems, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus
- Kidney and urinary tract problems, such as horseshoe or polycystic kidney, absent kidney, or hypospadias
- Muscular or skeletal problems
- VACTERL Association, which involves vertebral, anal, cardiac, TE fistula, renal, and limb abnormalities
Symptoms of these two conditions are usually noted quite soon after birth. They may include:
- Frothy white bubbles in the mouth
- Coughing or choking when feeding
- Vomiting
- Blue color of the skin, especially during feeding
- Difficulty breathing
- Very round, full abdomen (TEF)
- Unusually flat abdomen (isolated esophageal atresia)
A complete medical history is taken and a thorough physical examination is conducted. This examination includes trying to place a tube into the stomach. If this cannot be easily accomplished, there is a high likelihood of an obstructed esophagus. Chest and abdominal X-rays are performed to determine whether one or both conditions are present.
If a child (infant) has a TEF or esophageal atresia, surgery will be needed, and this is done quickly after the infant has been stabilized. The type of surgery performed depends upon the following:
- Type of abnormality
- Overall health of the baby and medical history
- Opinion of the surgeon and other physicians involved in the baby's care
- Expectations for the course of the condition
- Surgery
When a TEF is repaired, the connection between the esophagus and the trachea is closed. In most cases, the two ends of the esophagus can be brought together in a single operation. Occasionally, when the distance between the two segments is too far to bring the two ends together, other more complex reconstructions using portions of the intestines or stomach can be used to create a new esophageal tube. Sometimes this defect requires more than one operation.
- Recurrent fistula (re-operation is required in 1% of cases)
- Esophageal strictures caused by scarring (10% to 15% of cases)
- Gastroesophageal reflux (approximately 50% of cases)
- Aspiration (uncommon)
Some children born with esophageal atresia have long-term problems. Swallowing food or liquids may be difficult due to:
- Absent or abnormal contraction of esophageal muscles
- Scarring in the esophagus can partially block the passage of foods
In some children, a narrowed esophagus can be successfully widened with a special endoscopic dilatation procedure performed under general anesthesia. In other children, additional surgery may be necessary to open up the esophagus so that food can pass to the stomach properly.
Approximately 50% of children who have undergone surgical repair for esophageal atresia will have problems with gastroesophageal reflux disease (GERD). GERD causes acid to move up into the esophagus from the stomach. As it moves, it causes a burning or painful feeling known as heartburn. GERD is usually treated with medications. However, an anti-reflux operation is sometimes required.
