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The development of bronchiectasis on chest computed tomography in children with cystic fibrosis: can pre-stages be

Leonie A Tepper1,2, Daan Caudri1, Adria Perez Rovira1,3

  • 1Department of Pediatric Pulmonology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.

European Radiology
|April 25, 2016
PubMed

Insights

Bronchiectasis in cystic fibrosis (CF) often develops rapidly within two years, frequently without clear pre-stage indicators on CT scans. Mucus plugging is the most common early sign, highlighting the aggressive nature of CF lung disease.

Area of Science:

  • Pulmonary Medicine
  • Radiology
  • Genetics

Background:

  • Bronchiectasis is a significant complication of cystic fibrosis (CF) lung disease.
  • The developmental pathways and progression determinants of bronchiectasis in CF remain incompletely understood.

Purpose of the Study:

  • To investigate the development of bronchiectasis in CF patients using serial computed tomography (CT) scans.
  • To identify factors associated with the rapid progression of bronchiectasis.

Main Methods:

  • Analysis of volumetric CT scans from 43 CF patients with at least four biennial scans.
  • Utilized deformable image registration to track changes in regions of interest (ROIs) identified on the most recent CT.
  • Assessed for bronchiectasis, mucus plugging, airway wall thickening, consolidation, or normal airways within ROIs across serial scans.

Main Results:

  • Out of 362 ROIs, 175 (48.3%) showed the development of bronchiectasis.
  • In 79.4% of newly developed bronchiectasis, no distinct pre-stage was identified on prior scans.
  • Mucus plugging was the most frequent pre-stage (17.7%), followed by airway wall thickening (1.7%) and atelectasis/consolidation (1.1%).
  • Pancreatic insufficiency was more common in patients with rapid bronchiectasis progression (p=0.05).

Conclusions:

  • The majority of bronchiectatic airways in CF develop rapidly within two years, often without identifiable precursors on CT.
  • Mucus plugging represents the most common identifiable pre-stage of bronchiectasis in CF.
  • These findings underscore the challenging and often rapid progression of lung disease in cystic fibrosis.
Abstract

Keywords:
BronchiectasisCystic fibrosisHigh resolution computed tomographyLung diseasePaediatrics

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