Partial cricotracheal resection for congenital subglottic stenosis in children: The effect of concomitant anomalies

Abstract

Objective

To review the surgical outcomes of partial cricotracheal resection in children with severe congenital subglottic stenosis and define the effect of concomitant anomalies or syndromes affecting outcome.

Methods

Forty-one children with subglottic stenosis of congenital and mixed (acquired on congenital) etiologies who underwent partial cricotracheal resection were identified from a prospectively collected database. Children with congenital subglottic stenosis and concomitant anomalies/syndromes were compared to children with congenital subglottic stenosis with no syndromes or concomitant anomalies. Operation-specific decannulation rates and complication rates were the primary outcome measures. We performed a two-sample test of proportion using the STATA-10 software for categorical variables to detect differences in proportions. Significance was set at p value < 0.05.

Results

Twenty-seven (66%) of 41 children had concomitant anomalies/syndromes and 14 (34%) had congenital subglottic stenosis without concomitant anomalies/syndromes. Four patients needed revision surgery in the concomitant anomaly group and two patients needed revision surgery in the non concomitant anomaly group before achieving decannulation. The operation-specific decannulation rate in the concomitant anomaly group was 85% and 86% in the non anomaly group. When compared to children without concomitant anomaly, children with concomitant anomalies were more likely to have delayed decannulation following partial cricotracheal resection. However, this difference was not found to be statistically significant. The complication and operation-specific decannulation rates after partial cricotracheal resection were comparable to children without concomitant anomalies. Mortality rate was 11% (three of 27 patients) in the group with associated congenital anomalies or syndromes. Two patients succumbed to the primary pathology and one patient died due to tracheostomy-tube obstruction. There was no post-operative death in the non anomaly group.

Conclusion

Partial cricotracheal resection can be done safely and effectively in children with concomitant anomalies/syndromes to achieve decannulation. The post-operative course may be prolonged but the decannulation and the complication rates are comparable to those children with congenital subglottic stenosis without concomitant anomalies.

Introduction

Congenital subglottic stenosis (SGS) is the third most common congenital laryngeal anomaly after laryngomalacia and vocal cord paralysis [1]. This is also the most common laryngeal anomaly necessitating tracheostomy in children less than 1 year of age [2]. Subglottic stenosis can be classified into congenital, mixed or acquired types. In congenital stenosis, the patient will present with symptoms of respiratory obstruction with no other contributing factors. Mixed subglottic stenosis implies that a child born with a narrow but asymptomatic subglottic airway becomes symptomatic after a history of endotracheal intubation (acquired on congenital). The true incidence of congenital subglottic stenosis is difficult to assess due to the high incidence of intubation in patients with pre-existing narrow subglottic airway. The presence of subglottic pathology should be looked for in a child with genetic syndromes or other non-syndromic congenital anomalies, especially when these involve the mediastinum (cardiovascular or esophageal anomalies).

The choice between laryngotracheal reconstruction (LTR) and partial cricotracheal resection (PCTR) depends on the severity and extent of the subglottic stenosis as well as the vocal cord function. In severe established cases of congenital or acquired subglottic stenosis, partial cricotracheal resection has emerged as a better surgical option than laryngotracheal reconstruction with no adverse effect on the post-operative growth of the pediatric larynx. Decannulation has been achieved in a higher percentage of patients with a single operation for severe SGS (grade III and IV) following PCTR when compared to LTR [3], [4], [5]. In this manuscript, we compare the outcome in a cohort of patients with severe congenital SGS and associated concomitant anomalies to another group of children with severe congenital SGS without concomitant anomalies who underwent PCTR. We also draw attention to the various syndromes associated with congenital SGS and their influence on the prognosis and outcome following PCTR (Fig. 1, Fig. 2).