Congenital tracheal stenosis & associated cardiac anomalies: operative management & techniques.

Abstract

Congenital tracheal stenosis can lead to symptomatic airway obstruction in children and often mandates surgical correction. Over the past half-century, numerous tracheal reconstruction techniques have been developed, including tracheal resection with end-to-end anastomosis (for short-segment complete tracheal stenosis), patch tracheoplasty, slide tracheoplasty, and homograft and autograft augmentation repairs. However, operative management of congenital tracheal stenosis is often complicated by the presence of congenital heart disease, the most common of which is pulmonary artery sling. When present concomitantly, combined repair of both defects is feasible and is currently the preferred approach. Questions have been raised about the optimal timing and sequence of surgery, and some have advocated staged repair for patients with complex associated cardiac lesions. However, evidence from the past two decades suggests that concomitant repair can be performed with excellent results. The current standard of care involves the use of cardiopulmonary bypass to simultaneously repair the tracheal defect using slide tracheoplasty and all associated cardiac anomalies. Advances in operative techniques and extracorporeal circulation, progressive understanding of the pathological basis of combined congenital tracheal and cardiac disease, and a multidisciplinary approach to patient care have all contributed to the successful outcomes seen in the modern era. This article describes the combined surgical correction of tracheal stenosis and double-outlet right ventricle-tetralogy of Fallot type in an infant, provides a detailed step-by-step description for performing a slide tracheoplasty along with various other less favored tracheoplasty techniques, and reviews the current literature discussing such combined repairs.