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

G Pellen; C Pandit; C Castro; P Robinson; C Seton; D A Fitzgerald; K Waters; A T Cheng

doi:10.1016/j.ijporl.2019.109672

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

Int J Pediatr Otorhinolaryngol. 2019 Dec:127:109672. doi: 10.1016/j.ijporl.2019.109672. Epub 2019 Sep 13.

Authors

G Pellen¹, C Pandit², C Castro³, P Robinson⁴, C Seton⁴, D A Fitzgerald⁴, K Waters⁴, A T Cheng⁵

Affiliations

¹ Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Australia.
² Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Australia; Department of Respiratory and Sleep Medicine, The Children's Hospital at Westmead, Australia. Electronic address: [email protected].
³ Department of Respiratory and Sleep Medicine, The Children's Hospital at Westmead, Australia.
⁴ Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Australia; Department of Respiratory and Sleep Medicine, The Children's Hospital at Westmead, Australia.
⁵ Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Australia; Department of Ear Nose and Throat, The Children's Hospital at Westmead, Australia.

PMID: 31539787
DOI: 10.1016/j.ijporl.2019.109672

Abstract

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.

Keywords: Congenital tracheal stenosis; Non-invasive ventilation; Sleep disordered breathing.

MeSH terms

Child, Preschool
Constriction, Pathologic / complications*
Constriction, Pathologic / surgery*
Female
Humans
Infant
Infant, Newborn
Male
Noninvasive Ventilation*
Plastic Surgery Procedures
Polysomnography
Postoperative Period
Retrospective Studies
Severity of Illness Index
Sleep Apnea Syndromes / therapy*
Trachea / abnormalities*
Trachea / surgery
Treatment Outcome

Supplementary concepts

Tracheal agenesis