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Acquired Tracheoesophageal Fistula

Acquired tracheoesophageal fistula (TEF) is an abnormal connection between the trachea and esophagus caused by another condition (e.g., malignancy, trauma). Acquired TEF typically manifests with cough, dysphagia, dyspnea, and/or recurrent lung infections. Barium esophagogram and/or endoscopy confirm the diagnosis. Treatment involves a multidisciplinary team focusing on preventing aspiration, managing the underlying cause, and closing the fistula via surgery or endoscopy.

See also “Congenital tracheoesophageal fistula.”

  • Malignancy [1]
    • Esophageal cancer (most common malignant cause) [1]
    • Lung cancer
  • Prolonged endotracheal intubation or tracheostomy
  • Excessive cuff pressure of tracheostomy or endotracheal tube
  • Mediastinal surgery (e.g., esophagectomy)
  • Esophageal or airway stent erosion
  • Trauma (e.g., penetrating neck injury)
  • Infection (e.g., tuberculosis, histoplasmosis, actinomycosis) [2]
  • Ingestion (e.g., foreign body ingestion, caustic ingestion)
  • Inflammatory diseases (e.g., rheumatoid arthritis, inflammatory bowel disease) [2]

Clinical features vary based on size, location, and rate of formation of the fistula. [1]

  • Nonventilated patients [2]
    • Cough
    • Dysphagia
    • Dyspnea
    • Fever
    • Chest pain
  • Ventilated patients [1]
    • Air leak despite inflated cuff
    • Inability to wean off ventilator
    • Gastric distention
  • Symptoms of underlying cause
    • Symptoms of esophageal cancer
    • Symptoms of lung cancer
  • Symptoms of complications [2]
    • Recurrent aspiration pneumonia
    • Malnutrition
  • Confirm the diagnosis with barium esophagram and/or endoscopy (i.e., esophagoscopy, bronchoscopy). [1]
  • Obtain additional diagnostics under expert guidance to guide treatment, e.g.: [1]
    • CT chest
    • Esophagoscopy
    • Bronchoscopy

Treatment is guided by a multidisciplinary team, including gastroenterology, pulmonology, thoracic surgery, and oncology. [1]

Initial management [1][2][3]

  • Prevent aspiration in acquired TEF.
  • Remove nasogastric tubes and orogastric tubes to minimize further injury.
  • Provide nutritional support via jejunostomy tube or parenteral feeding.
  • Manage underlying cause (e.g., malignancy, infection).
  • Determine definitive management in consultation with multidisciplinary team.

Acute respiratory distress

In patients with known acquired TEF presenting with respiratory distress signs or clinical features of airway obstruction:

  • Consider intubation past the level of the TEF if airway compromise is present.
  • Patients with tracheostomy: See “Tracheostomy complications.”
  • Consider repeat diagnostics (e.g., endoscopy, CT chest) to assess for evolution of the TEF.
  • Start treatment of pulmonary aspiration.

Aspiration prevention in acquired TEF [1][2]

  • Establish NPO status.
  • Elevate head of bed ≥ 45 degrees.
  • Perform frequent oral suctioning.
  • Administer acid suppression medications.
  • Consider gastrostomy tube for gastric decompression.
  • In ventilated patients, advance ETT below TEF.

Definitive management [1][2]

Spontaneous closure of acquired tracheoesophageal fistula is rare, and intervention is typically required. [2]

  • Malignant acquired TEFs: esophageal stent ± airway stent
  • Nonmalignant acquired TEFs: Management options vary by size and location and may include the following.
    • Surgical repair
    • Endoscopic therapy (e.g., fibrin glue)
    • Esophageal stent ± airway stent
  • Aspiration pneumonia [2]
  • Malnutrition [2]

We list the most important complications. The selection is not exhaustive.