Vascular anomalies causing symptomatic tracheobronchial compression.

Objectives/hypothesis: To review the clinical presentation and diagnostic evaluation of patients with symptomatic congenital vascular anomalies causing tracheobronchial compression and to establish the short- and long-term results of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence.

Study Design: Retrospective review.

Methods: Chart review and telephone follow-up.

Results: Between 1987 and 1996, 35 children underwent surgical intervention to relieve symptomatic tracheobronchial compression resulting from a congenital vascular anomaly. Historically, the onset of symptoms occurs within the first months of life; however, only 12 (34%) of patients were diagnosed by 6 months of age and 13 (37%) were diagnosed at greater than 1 year of age. Excluding anomalous innominate artery, chest radiography or barium swallow was suggestive of a congenital vascular anomaly in 30 (94%) of the patients. Magnetic resonance imaging correctly delineated the anatomy of the vascular anomaly in 29 patients. Bronchoscopy was diagnostic in all three patients with anomalous innominate arteries causing tracheal compression. Postoperative follow-up was obtained in 32 (91%) of patients; 25 (78%) of these were asymptomatic at the time of their most recent examination. The remaining patients had persistent stridor, recurrent respiratory tract infections, and/or chronic cough. In all three patients who underwent postoperative bronchoscopy for persistent symptoms, tracheomalacia was demonstrated in the region of previous compression.

Conclusions: Tracheobronchial compression from congenital vascular anomalies is a rare but treatable cause of respiratory symptoms. Early diagnosis requires a prompt, thorough clinical and radiologic evaluation. Surgery affords excellent long-term resolution of symptoms.