Lung Transplantation in Mounier-Kuhn Syndrome: Successful Consecutive Cases and Review of the Literature

Background: Mounier-Kuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, respiratory infections, bronchiectasis, and progressive respiratory failure. Lung transplantation (LTx) in this condition has only rarely been reported and may be technically challenging because of the abnormal size and structure of the main airways. Case presentation: We describe two consecutive patients with Mounier-Kuhn syndrome who underwent bilateral lung transplantation at our center. Both patients had advanced respiratory disease with marked tracheobronchial dilatation. In the first patient, preoperative planning included patient-specific three-dimensional reconstruction and printing of the trachea and proximal bronchi. Because of severe enlargement of the recipient bronchus, a telescopic bronchial anastomosis was used. The postoperative course was complicated by bronchial stenosis requiring repeated bronchoscopic interventions and stent placement. The second patient underwent bilateral LTx without major airway complications and had a favorable early postoperative course. Conclusions: Lung transplantation can be successfully performed in selected patients with end-stage respiratory failure due to Mounier-Kuhn syndrome. Careful preoperative airway assessment and individualized planning of bronchial anastomosis are crucial in such patients.