Dataset Information

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

ABSTRACT:

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).

Conclusion

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.

Key points

• 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.

SUBMITTER: Tepper LA

PROVIDER: S-EPMC5101271 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature

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Publications

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

Tepper Leonie A LA Caudri Daan D Rovira Adria Perez AP Tiddens Harm A W M HA de Bruijne Marleen M

European radiology 20160423 12

<h4>Objective</h4>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.<h4>Methods</h4>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 ...[more]

PMID: 27108295

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Project description:ImportanceThe ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.ObjectiveTo assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children.Design, setting, and participantsThis retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020.Main outcomes and measuresAccuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors.ResultsAmong 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%.Conclusions and relevanceThis study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Dataset Information

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

Objective

Methods

Results

Conclusion

Key points

Publications

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

Similar Datasets

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