The development of bronchiectasis on chest computed tomography in children with cystic fibrosis: can pre-stages be identified?
OBJECTIVE
Bronchiectasis is an important component of cystic fibrosis (CF) lung disease but little is known about its development. We aimed to study the development of bronchiectasis and identify determinants for rapid progression of bronchiectasis on chest CT.


METHODS
Forty-three patients with CF with at least four consecutive biennial volumetric CTs were included. Areas with bronchiectasis on the most recent CT were marked as regions of interest (ROIs). These ROIs were generated on all preceding CTs using deformable image registration. Observers indicated whether: bronchiectasis, mucus plugging, airway wall thickening, atelectasis/consolidation or normal airways were present in the ROIs.


RESULTS
We identified 362 ROIs on the most recent CT. In 187 (51.7 %) ROIs bronchiectasis was present on all preceding CTs, while 175 ROIs showed development of bronchiectasis. In 139/175 (79.4 %) no pre-stages of bronchiectasis were identified. In 36/175 (20.6 %) bronchiectatic airways the following pre-stages were identified: mucus plugging (17.7 %), airway wall thickening (1.7 %) or atelectasis/consolidation (1.1 %). Pancreatic insufficiency was more prevalent in the rapid progressors compared to the slow progressors (p = 0.05).


CONCLUSIONS
Most bronchiectatic airways developed within 2 years without visible pre-stages, underlining the treacherous nature of CF lung disease. Mucus plugging was the most frequent pre-stage.


CONCLUSIONS
• Development of bronchiectasis in cystic fibrosis lung disease on CT. • Most bronchiectatic airways developed within 2 years without pre-stages. • The most frequently identified pre-stage was mucus plugging. • This study underlines the treacherous nature of CF lung disease.
Department of Pediatric Pulmonology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.|Department of Radiology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.
Leonie A
LA
Tepper
Department of Pediatric Pulmonology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.
Daan
D
Caudri
Department of Pediatric Pulmonology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands.|Biomedical Imaging Group Rotterdam, Departments of Radiology and Medical Informatics, Erasmus MC, Rotterdam, The Netherlands.
Adria Perez
AP
Rovira
Department of Pediatric Pulmonology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands. h.tiddens@erasmusmc.nl.|Department of Radiology, Erasmus MC, Sophia Children's Hospital, Rotterdam, The Netherlands. h.tiddens@erasmusmc.nl.|Department of Pediatric Pulmonology and Radiology, Erasmus Medical Center, Sophia Children's Hospital, Dr. Molewaterplein 60, room SP-3464, 3015 GJ, Rotterdam, The Netherlands. h.tiddens@erasmusmc.nl.
Harm A W M
HA
Tiddens
Biomedical Imaging Group Rotterdam, Departments of Radiology and Medical Informatics, Erasmus MC, Rotterdam, The Netherlands.|Department of Computer Science, University of Copenhagen, Copenhagen, Denmark.
Marleen
M
de Bruijne
D000293:Adolescent; D000328:Adult; D001987:Bronchiectasis / Q000000981:diagnostic imaging*; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000000981:diagnostic imaging*; D008297:Male; D015588:Observer Variation; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods* / Q000592:standards; D055815:Young Adult
Bronchiectasis; Cystic fibrosis; High resolution computed tomography; Lung disease; Paediatrics