Acquired Tracheoesophageal Fistula Unamenable to Endoscopic and Surgical Management

Introduction: Acquired, nonmalignant tracheoesophageal fistulas (TEFs) most commonly occur in the setting of prolonged use of endotracheal or tracheostomy tubes. In this report, we present a patient with tracheostomy who presented with recurrent aspiration pneumonia and was found to have a large TEF that was difficult to treat. We also discuss management challenges concerning TEFs. Case Description/Methods: A 26-year-old female with history of anterior spinal artery embolism resulting in quadriplegia and chronic ventilator dependance requiring tracheostomy placement presented with fever and increased secretions from tracheostomy site. She was found to have aspiration pneumonia and hypercapnic respiratory failure. CT scan of the chest showed an abnormal dilatation of the esophagus and upper thoracic trachea. Upper endoscopy revealed a TEF with a diameter of approximately 4 cm. (Figure 1A, B) This was deemed not amenable to endoscopic stenting or clipping due to its proximity to the upper esophageal sphincter and its size. A silicon Y stent was placed via bronchoscopy, after which another tracheostomy tube was placed through the stoma with subsequent improvement in ventilation; the patient improved, and she was discharged home. Over the next 2 months, she was admitted several times with recurrent aspiration pneumonia requiring Y stent exchange with no benefit. A surgical esophagectomy was not considered due to the location and size of the fistula and chronic ventilation dependence. Her respiratory status continued to worsen, and she eventually passed away after she was switched to comfort measures only. Discussion: The management of TEFs varies as there is limited data and consensus regarding best approach. Treatment depends on the TEF etiology, size, and location. Malignant TEFs contribute to more than 50% of acquired TEFs whereas benign TEFs are often caused by prolonged mechanical ventilation or cuff pressure from endotracheal/tracheostomy tubes. In general, benign TEFs < 5 mm are managed with local therapy such as fibrin glue injection, while > 5 mm ones are managed surgically. If surgery is not feasible, dual airway and esophageal stenting is often pursued for mid to proximal TEFs given there is a 20 mm safety margin both proximally and distally from the fistula, as studies report better clinical response compared to airway stenting alone. However, more distal TEFs are usually managed with esophageal stenting alone. More prospective studies are needed to compare the efficacy of each approach.Figure 1.: (A, B) Barium esophagram with evidence of oblique extrinsic compression of the esophagus (C, D) Contrast enhanced CT imaging showing aberrant R subclavian artery with posterior compression of esophagus (E) High resolution esophageal manometry with pulsatile high pressure zone in the upper esophagus.