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. 2024 Dec;59(12):3560-3568.
doi: 10.1002/ppul.27262. Epub 2024 Sep 18.

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

Synne S Kennelly  1   2   3 , Vegard Hovland  1 , Iren Lindbak Matthews  1 , Finn P Reinholt  4 , Håvard Skjerven  1   3   5 , Ketil Heimdal  6 , Suzanne Crowley  1   7
Affiliations

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

Synne S Kennelly et al. Pediatr Pulmonol. 2024 Dec.

Abstract

Background: The estimated prevalence of tracheobronchomalacia (TBM) in children is about 1:2100. Prevalence of intrathoracic malacia is higher in children 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 tracheomalacia (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 and 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, microbiology 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 years (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.

Keywords: PCD; airway malacia; bronchiectasis; flexible bronchoscopy.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Study flow diagram. Seventy‐one children with a diagnosis of PCD were followed up at Rikshospitalet, Oslo University Hospital, between January 2000 and December 2021. Thirty‐two patients had undergone flexible bronchoscopy where TBM was assessed. Twenty‐two children had intrathoracic airway malacia. PCD, primary ciliary dyskinesia; TBM, tracheobronchomalacia.
Figure 2
Figure 2
Location of malacia on FB (anatomical position). Patients with malacia in several areas appear more than once. FB, fiberoptic bronchoscopy.
Figure 3
Figure 3
Spirometry curves of patients nr 22 and 32, respectively, showing evidence of expiratory flow limitation in the form of a “shoulder” pattern.
See this image and copyright information in PMC

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