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Published on: January 17, 2011
Use of non-invasive ventilation in children with congenital tracheal stenosis
G Pellen1, C Pandit2, C Castro3
1Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Australia.
Insights
Congenital tracheal stenosis (CTS) is linked to obstructed sleep disordered breathing. Non-invasive ventilation (NIV) is well-tolerated and effectively improves breathing in these children.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Congenital Airway Abnormalities
Background:
- Congenital tracheal stenosis (CTS) is a rare condition involving complete tracheal rings, often requiring slide tracheoplasty.
- While slide tracheoplasty reduces mortality, significant morbidity, including sleep disordered breathing (SDB), persists.
- The role of SDB assessment and non-invasive ventilation (NIV) in pediatric CTS has not been previously described.
Purpose of the Study:
- To investigate the prevalence of SDB in children with CTS.
- To evaluate the use and efficacy of NIV in managing SDB in this population.
- To describe the 10-year experience (2005-2015) with SDB and NIV in pediatric CTS patients.
Main Methods:
- Retrospective case series conducted at a tertiary children's hospital.
- Inclusion of 16 patients diagnosed with CTS between 2005 and 2015.
- Analysis of pre-operative and NIV-assisted polysomnography (PSG) data, including obstructive apnea/hypopnea index (OAHI) and oxygen desaturation index (ODI).
Main Results:
- Of 16 CTS patients, 15 survived slide tracheoplasty (12 long-segment, 3 short-segment).
- 10 out of 15 survivors (66.7%) utilized NIV, all with long-segment CTS.
- NIV use significantly reduced median OAHI from 14.6/hr to 7.2/hr and ODI from 15.3/hr to 6.3/hr.
Conclusions:
- Pediatric patients with CTS frequently experience obstructed sleep disordered breathing.
- Non-invasive ventilation (NIV) is a well-tolerated and effective intervention for improving SDB in children with CTS.
- NIV can be a valuable tool in managing residual respiratory morbidity post-tracheoplasty.
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.
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