Patterns of management of congenital tracheal stenosis

Stenosing airway disease, including congenital and acquired lesions, is rare in the pediatric age group. Untill recently, the outlook for patients with congenital tracheal stenosis (CTS) was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. This report reviews the short and long-term outcomes of a single-institution experience in the management of CTS in children, comparing different treatment modalities. From 1991 to 2002, 13 cases of CTS have been managed in the authors unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy established the diagnosis in all cases. According to clinical and endoscopical features, patients have been classified into 3 groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomic type, associated anomalies, treatment modality, complications, outcome, and time of follow-up. Seven girls and 6 boys have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 8 months), and 77% showed associated anomalies. Four patients presented mild or no symptoms and have been treated expectantly. The other 9 patients have been operated on because of persistent or severe symptomatology. The following procedures have been performed: costal cartilage tracheoplasty (n = 5), tracheal resection (n = 3), slide tracheoplasty (n = 2), endoscopical dilatation (n = 3), and laser resection (n = 1). Three patients required 2 or more procedures, and there were 3 early deaths, all after costal cartilage tracheoplasty. Overall mortality rate in the series is 23%. Follow-up is complete in all survivors (n = 10) ranging from 6 months to 10 years (mean, 4.7 years). Selection of the type of treatment depends on the patient’s clinical status and the anatomic pattern of the stenosis. In symptomatic cases of short-segment stenoses the authors prefer tracheal resection with end-to-end anastomosis; for long-segment stenoses, slide tracheoplasty is the procedure of choice.