Tracheal Stenosis Associated With Operation for Pneumothorax With Marfan Syndrome: A Case Report

Marfan syndrome is a genetic disorder in which impaired protein leads to connective tissue weakness. We herein report a case of unexpected tracheal stenosis that was diagnosed just before an operation for a recurrent right pneumothorax with Marfan syndrome. A 16-year-old boy with bilateral repeated pneumothoraces associated with Marfan syndrome came to our emergency room complaining of dyspnea. A chest radiograph showed recurrent right pneumothorax. An operation was planned due to prolonged air leakage even after chest tube drainage. On induction of general anesthesia for repairing pneumothorax, a sudden difficulty occurred during manual ventilation, and the blood oxygen saturation temporarily decreased to 50%. Therefore, emergent intubation with a single-lumen tube was applied, which led back to full saturation. Bronchoscopy revealed a tortuous and flattened trachea. An endobronchial blocker tube was applied due to difficulty in double-lumen tube insertion, and bullectomy was accomplished without any other unexpected events. Patients with Marfan syndrome may have asymptomatic tracheal stenosis due to structural abnormalities and latent tracheomalacia, and general anesthesia could be a trigger to develop the symptoms. Surgeons should bear this in mind, cooperate with anesthesiologists well, and prepare for emergent intubation when managing patients with Marfan syndrome in the perioperative settings.