Irene Maria Olivia Borzani MD, Presenter: Nothing to Disclose

Carla Colombo MD, Abstract Co-Author: Nothing to Disclose

Harm AWM Tiddens MD, Abstract Co-Author: Nothing to Disclose

Tania Tondolo, Abstract Co-Author: Nothing to Disclose

Alessandra Carnevali, Abstract Co-Author: Nothing to Disclose

Maria Angela Pavesi MD, Abstract Co-Author: Nothing to Disclose

Paola Barbaccia MD, Abstract Co-Author: Nothing to Disclose

Progression of lung disease in children with cystic fibrosis (CF) at our centre is usually monitored by 6-12 monthly chest radiography depending on severity of lung involvement. HRCT is used to better describe lung disease but is only performed in a few patients. The aim of the study is to describe the features and distribution of mucus plugging in children and young adults with cystic fibrosis (CF) and to compare chest radiography with HRCT

Between January 2007 and December 2008 HRCT was performed on 31pts (13 boys, 18 girls - mean age 13,5 years, range 6-18 years) followed at CF centre of Milan (334 patients under 18 years) to better characterized the lung disease. HRCTs were scored with Brody score and the score for mucus plugging was extrapolated for each lobe in all patients. Two pediatric radiologists re-evaluated chest radiography performed around the time of the HRCT (within 10 days) focusing only on the detection and location of mucus plugging. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of plain radiography in detecting mucus plugging was calculated using the HRCT findings as the reference standard.

Out of 31 patients, 26 showed evidence of mucus plugging on HRCT according to Brody’s score: 39,7% of lobes involving the “central lung” (finger-in-glove sign) and 66,7% of lobes involving the “peripheral lung”(centrilobular nodules), with a predominance in the right upper lobe (83,9%). Mucus plugging in peripheral lung was not detectable on chest radiography. The sensitivity, specificity, PPV and NPV of chest radiography for mucus plugging in large airways was respectively 55%, 89%, 86% and 62%.

HRCT appears to be the most sensitive modality for the detection of mucus plugging, especially when present in peripheral lung which, in our series, is more frequently involved than central lung. Chest radiography is very poor in detecting peripheral mucus plugging that, therefore, cannot be excluded. The sensitivity of chest radiography for central mucus plugging is better when HRCT score is high with a good agreement between the two radiological methods.

Mucus plugging in central lung is well detected by both HRCT and chest radiography, while HRCT is recommended when just peripheral involvement is suspected.

Borzani, I, Colombo, C, Tiddens, H, Tondolo, T, Carnevali, A, Pavesi, M, Barbaccia, P, HRCT and Radiographic Appearances of Mucus Plugging in Children and Young Adults with Cystic Fibrosis.  Radiological Society of North America 2009 Scientific Assembly and Annual Meeting, November 29 - December 4, 2009 ,Chicago IL. http://archive.rsna.org/2009/8012048.html