Tracheobronchomalacia is common in children with primary ciliary
dyskinesia -- a case note review
Abstract
Background: The estimated prevalence of tracheobronchomalacia
(TBM) in children is about 1:2100. Tracheomalacia (TM) prevalence is
higher in patients with chronic lung disease such as bronchiectasis and
cystic fibrosis (CF) and may contribute to increased morbidity.
Objective: To determine the prevalence and assess clinical
features of TM, TBM and bronchomalacia (BM) in patients with primary
ciliary dyskinesia (PCD). Methods: We performed a retrospective
case-note review of all children with a confirmed or highly likely
diagnosis of PCD attending Oslo University Hospital between 2000-2021.
We selected those who had undergone flexible fiberoptic bronchoscopy
(FB) and in whom the presence of TBM was assessed. We retrieved
demographic and clinical data, including airway symptoms, PCD-diagnostic
criteria, indication for bronchoscopy, the presence of lobar
atelectasis, bacteriology and the descriptive and unblinded
video-recorded results of FB. Complications occurring during and after
bronchoscopy were noted. Results: Of 71 children with PCD, 32
underwent FB and were included in the review. The remaining 39 were
included for TBM prevalence calculation only. Median age at FB was 6.0 y
(3.1 – 11.9). Twenty-two children (69%) had intrathoracic airway
malacia. Four (13%) had isolated TM, seven (22%) had TBM, and 11
(34%) had isolated BM affecting either main (n=4) or lobar bronchi
(n=7) (LBM), including four with associated lobar atelectasis. FB
related complications, one major, 12 minor, were documented in 13
children (41%). Conclusion: We found a high prevalence of TBM
among children with PCD undergoing FB. This may represent a significant
comorbidity and have implications for patient management.