Tracheoesophageal Fistula – Symptoms, Types, Diagnosis, Repair, Treatment

What is Tracheoesophageal Fistula?

Tracheoesophageal fistula (TEF) is a condition in which there is an abnormal connection or passageway between the trachea and esophagus. This occurs when the trachea and the esophagus did not separate normally during the embryonic development. Often, this abnormality accompanies esophageal atresia (EA) in which there is no opening between the esophagus and stomach.

About 1 in 3,000 live births are born with this condition. Affected patients are most likely to be premature infants and those born to mothers diagnosed to have excessive amniotic fluid (polyhydramnios) during pregnancy.

Tracheoesophageal Fistula Symptoms

An infant born with TEF exhibits the 3C’s during feeding:

• Coughing
• Choking
• Cyanosis

Other manifestations include:

• Blowing bubbles of copious and frothy mucus in the mouth
• Excessive salivation and drooling
• Abdominal distention
• Respiratory distress

Types

EA with TEF on the lower portion of the esophagus

Approximately, 90% of diagnosed cases have this type. The upper segment of the esophagus ends in a blind pouch while the lower segment of the esophagus and stomach are connected by a fistula to the trachea.

TEF without EA

This type, often referred to as Type H, occurs at an incidence rate of 4.2 %.  The esophagus connects normally to the stomach. However, there is a fistula connecting the esophagus to the trachea.

EA with TEF on the upper portion of the esophagus

This type rarely occurs (0.8%). In here, the upper segment of the esophagus ends in a blind pouch and is connected by a fistula to the trachea.

EA with TEF to both upper and lower portion of the esophagus

Around 0.7% of cases have two fistulas present. The upper segment of the esophagus ends in a blind pouch and is connected by a fistula to the trachea.  There is another fistula which connects the lower segment of the esophagus and stomach to the trachea.

Diagnosis

Diagnosis of Tracheoesophageal fistula (TEF) is made by inserting a nasogastric catheter and obstruction is met as the catheter passes around 10 to 13 centimeter from the nostrils. There is also inability to aspirate gastric contents. On abdominal x-ray, the stomach is seen to be distended with air coming from the trachea.  Other diagnostic tests include barium swallow and bronchial endoscopy. If a radiopaque x-ray is done, the catheter is seen to coil around the upper esophagus.

Treatment and Repair

• Tracheoesophageal fistula (TEF) is corrected through a surgical repair. This involves the closing of the fistula and connecting or anastomosing the ends of the segments of the esophagus together.
• A portion of the colon may be taken and used to connect the segments of the esophagus if these are too far apart from each other. The surgeons may wish to complete the surgery in stages depending on the severity of the case. Simple fistulas may be repaired endoscopically with the use of fibrin glue to seal off the fistula.
• Early surgery lessens the chance for pneumonia to set in and improves the prognosis of the condition. Survivability of patients after corrective surgery of Tracheoesophageal fistula (TEF) can be as high as 90%.

Some important treatment points of Tracheoesophageal fistula (TEF) are

• Patients for Tracheoesophageal fistula (TEF) correction are usually not permitted to take anything by mouth in preparation for the surgery.
• Tracheoesophageal fistula (TEF) patients placed upright to avoid any aspiration.
• Intravenous fluids and antibiotics are prescribed for fluid balance and for any superimposed infections respectively.
• Feeding via a gastrostomy tube may be done 48 hours after the surgery while oral feedings are started 5 to 10 days thereafter.