Vascular anomalies causing symptomatic tracheobronchial compression

Objectives/Hypothesis: To review the clinical presentation and diagnostic e valuation of patients with symptomatic congenital vascular anomalies causin g tracheobronchial compression and to establish the short- and long-term re sults of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence. Study Des ign: Retrospective review. Methods: Chart review and telephone follow-up. R esults: Between 1987 and 1996, 35 children underwent surgical intervention to relieve symptomatic tracheobronchial compression resulting from a congen ital vascular anomaly. Historically, the onset of symptoms occurs within th e first months of life; however, only 12 (34%) of patients were diagnosed b y 6 months of age and 13 (37%) were diagnosed at greater than 1 year of age , Excluding anomalous innominate artery, chest radiography or barium swallo w was suggestive of a congenital vascular anomaly in 30 (94%) of the patien ts. Magnetic resonance imaging correctly delineated the anatomy of the vasc ular anomaly in 29 patients, Bronchoscopy was diagnostic in all three patie nts with anomalous innominate arteries causing tracheal compression. Postop erative follow-up was obtained in 32 (91%) of patients; 25 (78%) of these w ere asymptomatic at the time of their most recent examination, The remainin g patients had persistent strider, recurrent respiratory tract infections, and/or chronic cough. In all three patients who underwent postoperative bro nchoscopy for persistent symptoms, tracheomalacia was demonstrated in the r egion of previous compression. Conclusions: Tracheobronchial compression fr om congenital vascular anomalies is a rare but treatable cause of respirato ry symptoms. Early diagnosis requires a prompt, thorough clinical and radio logic evaluation. Surgery affords excellent long-term resolution of symptom s.