Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch

Complex tracheobronchial obstruction and malacia can be associated with conotruncal and aortic arch anomalies. A circumflex aortic arch composed of a left aortic arch and right descending aorta is an extremely rare anomaly that can severely affect the distal trachea and mainstem bronchi, resulting in severe respiratory symptoms. We report the case of a patient with circumflex aortic arch and severe left mainstem bronchial obstruction and malacia in which the external aortic compression and residual bronchomalacia were addressed with direct bronchial and tracheal intervention.