Tracheomalacia with necrosis and tracheoesophageal fistula secondary to tracheostomy

A 35-year-old man with a long history of muscular dystrophy presented for evaluation of a neck mass increasing in size. The patient had been diagnosed with muscular dystrophy at age 21 and a tracheostomy was performed 3 years prior for ventilatory support. Except for minor airway infections, the patient had been medically stable and fully ventilator-dependent. He received full nutrition through a percutaneous gastric feeding tube. There were no fevers or chills, no night sweats or weight loss, and no particular secretions suctioned from the tracheostomy tube. On examination, the patient was afebrile and awake. A firm, rounded mass was palpable immediately below the tracheal stoma anteriorly. There was no redness, tenderness, or discharge from the stoma. When reviewing the images, the caretakers reported, after further questioning, that there was some leakage around the tube and the patient had requested to inflate the cuff more. This was done on quite a few occasions over the preceding months. The leak would disappear but reappear a few days later. Rigid endoscopy and flexible esophagoscopy were performed and showed extensive focal tracheomalacia and necrosis of the posterior membranous wall. A large tracheoesophageal fistula was also present. The patient and family were presented with all options, including surgical repair of the large defect, but opted for comfort care only. The final diagnosis was focal severe tracheomalacia and tracheal necrosis with tracheoesophageal fistula resulting from chronic cuff overinflation.