Unravelling the Complexity of Congenital Tracheal Stenosis with 'O' Rings

Congenital tracheal stenosis (CTS) in paediatric patients is a rare condition, accounting for only 0.3% to 1% of all laryngotracheal stenoses. It is characterised by complete cartilaginous 'O'-shaped tracheal rings replacing the normal posterior membranous portion. This monocentric case series analyses management strategies and assesses surgical and functional outcomes.

Twenty-one patients (12 males, 9 females) underwent surgery for CTS. Nineteen patients received slide tracheoplasty, while two underwent tracheal resection with primary anastomosis. Pre- and postoperative assessments focused on respiratory, swallowing, and vocal functions. The study also examined correlations between prior non-airway surgeries, extent of stenosis, and postoperative complications.

All patients demonstrated postoperative improvement in respiratory, swallowing, and vocal functions. Wilcoxon signed-rank testing revealed a statistically significant improvement in respiratory scores, whereas improvements in voice scores did not reach statistical significance. Swallowing scores showed no variation pre- and postoperatively and were thus excluded from this analysis. A significant positive correlation was observed between cardiopulmonary bypass duration and postoperative intubation time (Pearson's r = 0.585, 95% CI 0.205-0.812, P = .007). In contrast, correlations between ICU length of stay and complication severity (Spearman's ρ  =  0.316, 95% CI -0.113 to 0.787, P = .175) as well as between patient age at surgery and complication severity were not statistically significant. Additionally, linear associations were identified between prior non-airway surgeries and increased complication rates and between bypass time, hospital stay duration, and complication severity.

Slide tracheoplasty remains the preferred technique for long-segment CTS, offering structural and functional restoration. Short-segment disease can be effectively managed with segmental resection and anastomosis. Prognosis could be influenced by stenosis length, associated comorbidities, and prior interventions, but larger studies would be needed to achieve adequate statistical power. Careful preoperative evaluation, tailored surgical planning, and attentive postoperative care are vital for optimal outcomes. Surgical intervention may be warranted even in select mildly symptomatic patients to prevent progression to life-threatening airway compromise.