Pediatric Tracheal Stenosis And Vascular Rings

Pediatric tracheal stenosis includes a variety of entities characterized by a diminished airway lumen. Congenital entities can be classified into (i) intrinsic disease caused by short(e.g. tracheal webs) or long-segment forms of tracheal stenosis (e.g. tracheal atresia/agenesis and primary tracheomalacia) and (ii) extrinsic disease caused by cardiovascular anomalies such as vascular rings and/or congenital tumors. Acquired entities include intubation trauma and tracheostomy-induced formation of granulation tissue. Surgical and endoscopical techniques developed in the last 20 years have dramatically improved the outcome of these patients. With the wider availability of higher-resolution imaging, minor or subclinical forms are increasingly recognized. Asymptomatic mild cases or symptomatic cases with no respiratory embarrassment can be safely managed in an expectant manner. Very symptomatic patients with an intrinsic, short tracheal stenosis generally undergo resection and anastomosis, whereas in those with a long tracheal stenosis a slide tracheoplasty is performed. Patch tracheoplasty is recommended in patients with intrinsic, long tracheal and bronchial stenosis. Endoscopic techniques such as dilation, laser excision or stents play a large and expanding role in managing complications, although a tracheostomy or second open tracheoplasty might be unavoidable. In place of evidence-based recommendations and standards, patient care remains individualized, based on each institution’s expertise and past experience. Close coordination between various services in a multidisciplinary approach can optimize the airway treatment and ensure the most favorable long-term outcome for the child.