Double aortic arch: an elusive diagnosis in a child with CHD

An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

1640 Stridor in An Infant with Double Aortic Arch

An eleven-month-old infant presented to our casualty with stridor that had been noticed by the parents a few weeks earlier and became increasingly evident lately. They did not recall a specific event when the infant “chocked”, but they denied any recent history of respiratory tract infection. The infant had no relevant medical or surgical history, and he was born full term with normal vaginal delivery. Examination of the infant showed moderate biphasic stridor with both suprasternal and intercostal recessions. Oxygen saturation on room air was 94% and temperature was 37.1°. Chest auscultation revealed bilateral equal air entry, though decreased, with transmitted stridor. No lung crepitations were appreciated. Suspicion of unwitnessed foreign body aspiration arose, and a decision of diagnostic rigid bronchoscopy was taken. Rigid bronchoscopy revealed no foreign body along the tracheobronchial tree, but evident circumferential constriction of the lower trachea was noted with tendency of the tracheal wall to collapse upon withdrawal of the bronchoscope. Following recovery, computed tomography with contrast of the neck and chest was done and a vascular ring, consisting of double aortic arch, was found compressing and narrowing the trachea. Further follow up over the following 6 months showed marked improvement of his distress, and active observation was continued. Conclusions Although foreign body aspiration is the commonest cause of stridor in infants and young children, external compression of the trachea, particularly with vascular rings, should be considered in children with otherwise unexplained stridor.