Tracheoesophageal Fistula

A tracheoesophageal fistula occurs during fetal development, when the esophagus connects to the trachea instead of the stomach. This allows stomach acid and other liquid to enter the lungs. When this happens, aspiration, pneumonia and other respiratory conditions can develop.

Babies born with this condition may not present with any immediate problems, but once they start to swallow their own saliva, it becomes obvious that something is not functioning properly.

The most apparent symptom of tracheoesophageal fistula is trouble swallowing. Other symptoms include:

• Inability to swallow
• Aspiration
• Frothy white bubbles in the mouth
• Hiccups
• Coughing or choking when feeding
• Vomiting
• Blue color of skin (lack of oxygen)
• Trouble breathing
• Swollen abdomen

There are different kinds of tracheoesophageal fistulas depending on where the abnormal connection between the esophagus and trachea occurs (top, bottom, middle or at both ends). The rarest form (called an H-type because the esophagus and trachea form an H shape) involves a side connection between the two pipes. An H-type fistula allows food to travel to the stomach but causes a delayed hiccup, because some fluid gets into the windpipe or lungs.

In about one half of babies with tracheoesophageal fistulas, another birth defect is also present. They include:

• Trisomy 13, trisomy 18 or trisomy 21 (Down syndrome)
• Digestive malformations (diaphragmatic hernia)
• Heart conditions
• Kidney conditions
• Muscular and skeleton conditions
• Vertebral, anal, cardiac, tracheoesophageal fistula, renal and limb abnormalities (VACTERL syndrome)

Children with tracheoesophageal fistulas may experience gastroesophageal reflux disease (GERD) and a narrowing (stricture) of the esophagus. 

Diagnosis of Tracheoesophageal Fistula

Pediatricians and pediatric surgeons at Riley at IU Health perform the following exams and tests to diagnose a tracheoesophageal fistula:

• Orogastric tube. A thin tube will be passed from the mouth into the esophagus toward the stomach to suction out fluids. If the passage of the tube is blocked, a fistula may be the cause.
• Chest X-ray. An X-ray of the chest will let the doctor see the esophagus, trachea and lungs. Any malformations are usually noticeable on the X-ray images.
• Abdominal X-ray. An abdominal X-ray shows pictures of the internal structures in the abdomen, including the spot where the esophagus normally connects with the stomach.