Tracheobronchial Compression by Congenital Vascular Anomalies – Surgical Procedures and Results

Objectives: Congenital aortic arch anomalies are a rare cause of respiratory distress in the pediatric population. Most patients present with dyspnea, recurrent respiratory infections, persistent cough and stridor. Variations in age at presentation with mild or severe symptoms is variable. This study aimed to report mid-term results of the offered surgical treatment for this special subset of patients by retrospective review.

Methods: Since 2008, 2.1% (n = 68) of our patients (n = 3,280) underwent correcting procedures due to congenital vascular anomalies causing tracheobronchial compression. Most of them had division of the nondominant aortic arch (n = 40, 58.8%), vascular transfer of compressing vessels (aberrant innominate artery or lusoria artery, n = 13, 19.1%) sometimes in combination with an additional aortopexy (n = 12, 17.6%). Tracheal surgery (sliding plasty) because of long-segment-tracheal stenosis or a combination of tracheal stenosis and malacia was necessary for 19 patients (27.9%).

Results: Mean patient age was 3.1 ± 0.9 years. There was no operative mortality. Most patients were managed without cardiopulmonary bypass. Tracheal surgery and head-and-neck vessel transfer was performed on cardiopulmonary bypass with intraoperative bronchoscopy before and after surgical correction. Follow-up was 100% completed, there was no late mortality so far. Reoperations because of recurrent symptoms were rare. One patient underwent redosliding plasty (1.5%). Three patients 4.4%) underwent secondary tracheal correction after primary division of a double aortic arch. Postoperative course was sometimes stormy, one patient (1.5%) had to be managed on venovenous ECMO for nine days because of respiratory failure (hypercapnia and respiratory acidosis). Two patients were managed with a tracheostoma for a longer period.

Conclusion: Congenital aortic arch anomalies causing tracheobronchial compression is a rare but well treatable disease. Survival is excellent and midterm results are promising. Relief of symptoms after primary repair is 94.2 and 100% after successful reoperation.