Overview
A tracheoesophageal fistula (TEF) is an abnormal connection between the oesophagus (gullet) and the windpipe, which are two tubes in the throat and chest that are usually separate from one another. Some patients are born with this disorder, but it can also develop later in life. In cases where it is present at birth, the condition usually occurs alongside oesophageal atresia, an obstruction of the gullet.
The most common cause of acquired TEF is cancer, such as tumours in the gullet. Other causes include injury resulting from medical treatment or previous surgery, blunt or penetrating chest or neck trauma, certain types of infection, ingesting corrosive fluid and inhalation burns. TEF can also develop as a result of prolonged mechanical ventilation using a tube such as a tracheostomy tube.
Symptoms
A congenital (present at birth) TEF may be suspected based on symptoms found during prenatal ultrasounds, such as excess amniotic fluid in the amniotic sac, the absence of fluid in the stomach, a small abdomen or enlarged areas in the gullet. Infants born with a TEF may vomit, gag, cough, emit large amounts of oral secretions, have bluish skin, and possibly respiratory distress. They may also develop abdominal swelling. The first feeding can cause sudden arrest and death.
Patients with an acquired TEF who have been ventilated may experience unexplained weight loss, recurrent chest infections and repeated failures to wean. In non-ventilated patients, symptoms include chest pain, shortness of breath, difficulty swallowing, coughing up blood, hoarseness, fever, repeated respiratory tract infections and pneumonia. Patients with cancer will have the same symptoms, in addition to experiencing symptoms associated with the tumour.
Diagnosis
Imaging techniques can be used to diagnose a TEF. If your doctor thinks your unborn baby may have a TEF, a foetal MRI is the test of choice. For infants born with a TEF, the condition is usually diagnosed by chest radiography.
When a nasogastric tube is inserted into the mouth of a patient with a TEF, it is not possible to pass the tube to the stomach; instead, the tube will coil in the middle section of the chest cavity. TEFs can also be diagnosed by way of CT radiography, but these are not commonly used. Contrast-enhanced studies are sometimes required to confirm the diagnosis of a TEF, and to identify its location, size and direction. Acquired TEFs can also be diagnosed by direct visualisation using a flexible metal tube with a camera.
Treatment
TEF is treated with surgery. If an infant is healthy and has no pulmonary complications, primary repair is performed within the first few days of life. Repair may be delayed in patients with low birth weight, pneumonia or other conditions. These patients should first be treated conservatively, with intravenous feeding, inserting a feeding tube through the skin and directly into the stomach, and upper pouch suction, until they are considered to be low risk enough to undergo the procedure.
Precautions must be taken before the procedure to prevent leakage and damage in the gullet. These include placing an endotracheal or tracheostomy tube. A draining gastrostomy tube may also be applied to reduce reflux.
TEFs that are a result of cancer are often inoperable. In such cases, a stent may be placed in the gullet to isolate the TEF and prevent food and other material from entering into the windpipe or lungs.
Bibliography
1. Bluestone CD. Pediatric otolaryngology. Saunders (2003) ISBN: 0721691978.
2. Engum SA, Grosfeld JL, West KW, “Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades,” Arch Surg. May 1995; 130(5): 502-8; discussion 508-9.