Abstract
BACKGROUND: Double aortic arch (DAA) is a type of vascular ring, causing stridor and dysphagia owing to compression of the trachea and esophagus. In contrast, double innominate veins, which is a relatively rare venous anomaly, usually does not cause symptoms or require intervention. CASE PRESENTATION: A 2 month-old boy presented with mild stridor and a hoarse voice while crying, and was diagnosed as having DAA, small atrial septal defects and double innominate vein. The double innominate veins, crossing over and underneath the ascending aorta, did not contribute to airway compression. To release the narrow vascular ring caused by the DAA, surgical resection of the atretic side of the arch was performed by lateral thoracostomy at 4 months of age, before the appearance of signs of bronchomalacia. The double innominate veins were left untouched, and open-heart surgery was avoided. CONCLUSIONS: DAA should be suspected if airway symptoms are present in early infancy, particularly if a right aortic arch is noted. It is important not to hesitate to perform diagnostic imaging and release the DAA, as this might prevent or minimize airway complications. However, for double innominate veins, operative release is not necessary unless it interferes with surgical procedures or is causing symptoms.