Ultra-low dose computed tomography chest chest radiography in paediatric primary ciliary dyskinesia: A prospective study.

BACKGROUND

Primary ciliary dyskinesia (PCD) is a rare condition characterised by dysmotile, immotile, or absent cilia. As a result of the impairment in respiratory mucociliary clearance, patients with PCD typically develop neonatal respiratory distress, nasal congestion, otitis media and recurrent respiratory infections leading to bronchiectasis and structural lung changes. These changes have been shown by chest computed tomography (CT) to develop in infancy and early childhood. Recent development and refinement of radiation-reducing CT techniques have allowed significant radiation dose reductions, with chest CT doses now in the range of chest radiography (CR).

AIM

To evaluate the efficacy of ultra-low dose CT (ULDCT) chest in identifying pulmonary changes within a PCD paediatric patient cohort.

METHODS

Paediatric patients with PCD who presented for routine clinical outpatient follow-up within the study period, were eligible for inclusion in the study. ULDCT and CR were performed on these patients and the results compared. Comparison metrics included radiation dose, subjective and objective image quality and disease severity.

RESULTS

Six paediatric patients (mean age 9 years) underwent clinically indicated ULDCT chest examinations and CR for surveillance of their PCD. The mean effective dose was 0.08 ± 0.02 mSv, a dose that approximates that of a frontal and lateral chest radiograph. The average Brody II score across the entire cohort was 12.92, with excellent inter-rater reliability and intra-class correlation coefficient (ICC) of 0.98. The average Chrispin-Norman score on CR was 1 with excellent inter-rater reliability and ICC of 0.92.

CONCLUSION

ULDCT demonstrates superior diagnostic capabilities, minimal radiation dose penalty, and high interobserver reliability in comparison to CR. Thus, we advocate for ULDCT to be the preferred modality for surveillance imaging in paediatric PCD.