Swyer-James syndrome with vascular ring in a child: Echocardiogrpahic diagnosis

Abstract

Swyer-James syndrome (SJS) is a relatively uncommon type of postinfectious obliterative bronchiolitis as a result of respiratory tract infection (RTI) in early childhood. Vascular ring (VR) is usually an underlying cause of RTI. When VR complicated with SJS, both diseases can be a relationship of cause and result, but have not been published in the past. Herein, we described an 8-year-old female school-age child who had history of recurrent RTI since age of 6 months. After screening echocardiographic findings of hypoplastic left pulmonary artery and aberrant right subclavian artery (ARSCA), confirmed by multisliced CT and functional evaluation by bronchoscopy and manometry. After she underwent aortopexy for ARSCA and balloon dilatation of constrictive bronchus and tracheal stenosis, and her growth pattern and pulmonary function improved progressively. Early detection by echocardiography and elimination of the associated underlying airway obstruction were mandatory for improvement of pulmonary function.