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Tracheobronchomalacia is common in children with primary ciliary dyskinesia-A case note review

Synne S Kennelly1,2,3, Vegard Hovland1, Iren Lindbak Matthews1

  • 1Paediatric Department of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway.

Pediatric Pulmonology
|September 18, 2024
PubMed

Insights

Tracheobronchomalacia (TBM) is common in children with primary ciliary dyskinesia (PCD). This study found a high prevalence of TBM in these patients, suggesting it

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Genetics

Background:

  • Tracheobronchomalacia (TBM) affects approximately 1 in 2100 children.
  • Intrathoracic airway malacia is more prevalent in pediatric patients with chronic lung conditions like cystic fibrosis (CF), potentially increasing morbidity.

Purpose of the Study:

  • To determine the prevalence of tracheomalacia (TM), TBM, and bronchomalacia (BM) in patients diagnosed with primary ciliary dyskinesia (PCD).
  • To assess the clinical characteristics associated with these airway malformations in the PCD population.

Main Methods:

  • A retrospective review of medical records for children diagnosed with PCD between 2000 and 2021 at Oslo University Hospital.
  • Inclusion criteria required flexible fiberoptic bronchoscopy (FB) for TBM assessment.
  • Data collected included demographics, airway symptoms, PCD diagnostic details, bronchoscopy indications, atelectasis presence, microbiology, and FB findings.

Main Results:

  • Of 71 children with PCD, 32 underwent FB. Twenty-two children (69%) exhibited intrathoracic airway malacia.
  • Prevalence rates included: isolated TM (13%), TBM (22%), and isolated BM (34%), with some cases of lobar bronchomalacia and associated atelectasis.
  • Bronchoscopy-related complications occurred in 41% of patients (1 major, 12 minor).

Conclusions:

  • A significant prevalence of TBM was identified in children with PCD who underwent flexible fiberoptic bronchoscopy.
  • TBM may be a considerable comorbidity in pediatric PCD patients, impacting clinical management strategies.
Abstract

Keywords:
PCDairway malaciabronchiectasisflexible bronchoscopy

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