Use of non-invasive ventilation in children with congenital tracheal stenosis.

INTRODUCTION

Congenital tracheal stenosis (CTS) is a rare airway condition characterized by complete tracheal rings. Most patients undergo a slide tracheoplasty, which greatly reduces mortality but significant morbidity remains. The assessment of sleep disordered breathing (SDB) and use of non-invasive ventilation (NIV) in these children has not been described.

AIM: To describe the presence of SDB and use of NIV in children diagnosed with CTS over a 10-year period (2005-2015).

DESIGN

Retrospective case series at a tertiary children's hospital.

RESULTS

There were 16 patients identified with CTS with a median [range] age at diagnosis of 2.5 months (0-9 months). One child died in the immediate post-operative period following a slide tracheoplasty, leaving 15 survivors. There were no later deaths during follow-up while using NIV for up to 3 years after surgery. Slide tracheoplasty was undertaken in (12/15) with long-segment tracheal stenosis. 3/15 patients had a short-segment tracheal stenosis and were managed conservatively. The use of NIV occurred in 10/15 (66.67%) patients, all of whom had long-segment CTS. Pre-operative polysomnography (PSG) showed a median (±SD) obstructive apnoea/hypopnoea index (OAHI) of 14.6/hr (±6.2) which reduced to 7.2/hour (±4.2) on NIV prior to slide tracheoplasty. The median oxygen desaturation index (ODI) before NIV use was 15.3 (±19.4) episodes/hour, which reduced to 6.3 (±11) on NIV. The median period of NIV use was 5 [1-24 months] months.

CONCLUSION

Patients with CTS have obstructed sleep disordered breathing. Trials of NIV are well-tolerated and improve sleep disordered breathing.