Congenital vascular rings: a rare cause of respiratory distress in infants and children

BACKGROUND: Congenital vascular rings may often cause unexplained respiratory symptoms in infants and young children. Their diagnosis and treatment are often delayed. Few studies of vascular rings have been reported in China. The aim of this study was to describe the clinical presentation, diagnosis and surgical management of infants and children with congenital vascular rings. METHODS: Clinical histories, physical examinations, investigations, image studies and surgical interventions were retrospectively evaluated in 7 children (age range: 2 months-4 years, mean 7 months) with congenital vascular rings. Chest radiography was performed in all patients. Echocardiography and computed tomography (CT) with 3-dimensional (3D) reconstructions were performed in 6 patients. Esophagography, cardiac catheterization and angiography, and bronchoscopy were performed in 1, 1 and 4 children, respectively. RESULTS: Six of the 7 patients had respiratory symptoms, including recurrent cough, stridor and wheeze. Age at onset of symptoms ranged from 1 month to 11 months. Chest X-ray showed nothing important on the vascular rings, besides bronchitis and pneumonia. Contrast-enhanced CT diagnosed vascular rings in 6 patients. Four patients had double aortic arches, two had balanced arches and two were right arch dominant. One patient had a right aortic arch with left ligament and 1 patient had a pulmonary artery sling. Echocardiography failed to diagnose vascular rings in 2 patients. The esophagogram of 1 patient showed esophageal compression. Bronchoscopy of 4 patients showed compression of the distal trachea. Five of the 7 patients underwent surgical division of the vascular rings. Surgical observation confirmed the CT findings in each patient. CONCLUSIONS: Patients, especially infants or young children, with recurrent respiratory symptoms such as chronic cough, stridor and wheeze, should be examined for the possible presence of congenital vascular rings. Contrast-enhanced CT can clearly show the anatomy of vascular rings. As a noninvasive technique, echocardiography is helpful for diagnosis. Early surgical management in symptomatic patients is effective.