Tracheoesophageal fistula (TEF) is a birth defect where the esophagus is connected to the windpipe (trachea). This abnormality causes problems with swallowing. The esophagus is the muscular tube that carries food and drink from the mouth to the stomach. The trachea is a pipe that carries air from the nose and mouth to the lungs. In the anatomy of a healthy child these two tubes are not connected.
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
- Frothy white bubbles in the mouth
- Coughing or choking when feeding
- 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.
Surgery is the primary treatment for a tracheoesophageal fistula. Surgical treatment of tracheoesophageal fistulas can successfully separate the esophagus and trachea and properly align them with the stomach and lungs. Doctors at Riley at IU Health try to perform surgery as soon as possible.
Treatment options for tracheoesophageal fistula include:
- Reconstructive surgery. Your child's doctor will plan to perform surgery as soon as possible to separate the esophagus from the trachea and put them back in the proper place. Surgery is often performed through the chest to reach the part of the esophagus just before it meets the stomach. After surgery, your baby must receive IV or feedings through a tube (enteral feedings) for about a week to ensure the repair is successful. He or she can then begin normal feedings. Your baby may have to stay in the hospital for up to one month after reconstructive surgery.
- Parenteral nutrition. If your baby was born prematurely or has another medical condition that makes surgery too risky, he or she can be fed using IV to ensure proper nutrition and growth until surgery can be performed.
- Upper endoscopy. The esophagus may narrow at some point after surgery or later in your child’s life. If this happens, endoscopy procedures can be used to widen the narrowed opening.
Key Points to Remember
Key Points to Remember
- A tracheoesophageal fistula is an abnormal connection between the esophagus and trachea.
- This condition prevents normal swallowing and allows stomach acid to get into the lungs.
- Surgery separates the esophagus and trachea, connects the esophagus to the stomach and closes the opening between the trachea and the esophagus.
- About one half of infants with a tracheoesophageal fistula also have another birth abnormality.
- Children with this condition may experience gastroesophageal reflux disease (GERD) and a narrowing of the esophagus later in life.
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