A 45-year-old woman presented with fever, sore throat, stridor, cough, dyspnea, and speech disturbance. The patient reported a history of similar episodes since childhood, with symptoms such as hoarse voice; barking cough with stridor; impaired exercise tolerance; and dyspnea provoked by coughing and forced expiration. There was a tendency of SpO2 level to decrease. The patient was initially diagnosed with bronchial asthma and treated with ICS. Temporary difficulty in speaking led to a tentative diagnosis of spasmodic dysphonia. The symptoms and episodes resulted in deteriorated quality of life over time, while diagnosis remained unclear. A history of smoking for 17 years and predisposition to obesity with a BMI of 30.8 was noted.
Upon admission, laryngoscopy revealed right-dominant, swollen tonsils with a white coating. No subglottic stenosis or laryngeal edema was noted. Empiric antibiotics and steroids were administered intravenously, with the presumption of a diagnosis of suppurative tonsillitis. Although the inflammation improved, the patient reported stridor and dyspnea while walking down the hospital hallway. Expiratory dominant stridor was obvious in the cervical area, although the lungs were relatively clear to auscultation. Thus, considering other differential diagnoses was warranted.
Chest radiography revealed narrowing of the bronchi on exhalation (Fig. 1), which was further confirmed by CT at the end of forced expiration/inspiration, revealing a collapsed trachea, leading to a diagnosis of ECAC. Virtual endoscopic images were generated from the CT data (Fig. 2A and B), showing protrusion of the membranous portion of the trachea at exhalation. The patient was discharged once the condition stabilized. However, soon after, the patient experienced rapid recurrence of dyspnea with a gradually deteriorating condition.
The patient was referred to a university hospital, where a diagnosis of hypoxic respiratory failure was made, and admitted to the intensive care unit. Upon admission to the intensive care unit, clinical impression was airway collapse combined with bronchial asthma. Thus, we administered steroids and applied CPAP. This management successfully stabilized the patient's condition that was unclear whether ECAC alone led to it. Bronchoscopy was first performed immediately before rigid bronchoscopy followed by stent insertion. The bronchoscopic procedure was performed under mild sedation at the end of forced expiration/inspiration immediately before general anesthesia induction. Bronchoscopy revealed protrusion of the membranous portion of the central airways into the tracheal lumen (Fig. 2C and D). Reduction in the cross-sectional area of the trachea at the aortic arch level measured by repeated CT scanning at expiration was 64% compared with that at inspiration, supporting ECAC diagnosis. Although CPAP was effective in initial stabilization, it did not provide sufficient therapeutic effects in the patient to be discharged or improve quality of life. TBP was also considered as a treatment option. To estimate the efficacy of this surgical procedure, a dynamic Y tracheobronchial stent was placed (Fig. 3), following an algorithm commonly used to manage ECAC. The Y-stent significantly improved the patient's symptoms. However, the stent was not tolerated because of irritation and removed 5 days later. Two weeks later, the patient underwent successful TBP.
The approach was a standard posterolateral thoracotomy through approximately 20 cm skin incision. A long spiral single-lumen tube was used for ventilation and moved back and forth depending on surgical steps to avoid cuff injury and confirm surgical safety. The endotracheal tube was first placed in the upper trachea, where bronchial observation confirmed significant airway collapse by exerting negative pressure via suction. Then the tube was advanced to the left main bronchus to collapse the right lung. Once the right main bronchus was dissected and encircled, an aortic clamp was applied to occlude the right bronchus for later procedures. After the membranous portion of the trachea was fixed, the endotracheal tube was moved back to the upper trachea to confirm surgical efficacy. After fixation of the trachea, airway collapse did not occur by applying negative pressure at all. While keeping the endotracheal tube kept in the middle trachea, the left main bronchus was fixed. Then the endotracheal tube was advanced to the left main bronchus. Additionally, the aortic clamp on the right bronchus was removed to fix the right main bronchus and bronchus intermedius. An appropriately trimmed polypropylene mesh was sutured with polydioxanone sutures (Johnson and Johnson, New Brunswick, NJ, USA), to the membranous portion of the airway. This fixation was from the upper trachea through the carina, down to the left main bronchus close to the secondary carina, as well as down the right main bronchus and bronchus intermedius (Fig. 4). Stitches were placed to plicate the membranous portion. A total of 64 stitches were placed to complete the procedure that lasted for 453 min without complications. On CT imaging, the airways at segmental levels appeared intact. Otherwise, surgical indication would have been doubtful.
Recovery after surgery was uneventful, with a significant improvement in symptoms. The patient was discharged 16 days after surgery. Six months later, CT demonstrated good outcomes of surgery (Fig. 5). The patient reported resolution of symptoms. The recovery time from exertion (for example, upon exercise) was significantly reduced after TBP.