Innominate artery enlargement in congenital arteriovenous fistula with subsequent tracheal compression and stridor

To demonstrate that airway obstruction may be the first manifestation of a congenital fistula, a female newborn is reported who presented with increasing stridor during her first 23 months of life. Magnetic resonance imaging and Doppler echocardiography revealed an enlarged innominate artery with turbulent flow. Angiography demonstrated an arteriovenous fistula between the right subclavian artery and right subclavian vein and an abnormal origin of the right internal thoracic artery. Bronchoscopy showed a pulsating compression of the middle section of the trachea. Closure and division of the fistula and aortotruncopexy were performed. The stridor disappeared, and there was marked relief of the tracheal obstruction, confirmed by bronchoscopy. It is concluded that, a search for enlarged vessels is necessary in cases of airway obstruction.