Congenital vascular anomalies and persistent respiratory symptoms in children

Congenital cardiovascular anomalies are a rare but treatable cause of strid er in the paediatric population. Despite this, we and the authors of other series have found a delay in referral for endoscopic diagnosis. The duratio n of symptoms prior to endoscopic diagnosis ranged from 6 weeks to 10 years . It has been estimated that up to 3% of the population have congenital ano malies of the aortic arch complex and we feel that many patients currently being diagnosed as having recurrent croup or atypical asthma must be exclud ed from this group. We had 16 cases of congenital vascular anomalies causin g tracheobronchial compression. Fifteen were due to innominate artery compr ession and one was due to a double aortic arch. Presenting symptoms include d strider (100%), persistent cough (75%), chronic dyspnoea (75%), reflex ap noea (60%), recurring respiratory tract infections (56%) and dysphagia (25% ). Direct laryngoscopy and bronchoscopy was diagnostic in all cases and is our investigation of choice. Eleven underwent aortopexy (69%), one had divi sion of the non-dominant aortic arch (6%) and four were managed conservativ ely (25%). They were followed for up to 9 years post-operatively and 75% ha d complete resolution of their symptoms. We feel that only a high clinical index of suspicion will enable these potentially fatal anomalies to be diag nosed. (C) 1999 Elsevier Science Ireland Ltd. All rights reserved.