Diagnostic value of chest ultrasound in children with cystic fibrosis – Pilot study

Abstract

Cystic fibrosis (CF) is one of the most common genetic disorders among the White population. The disease has a progressive course and leads to a reduction in the quality of life and of life expectancy. Standard diagnostic procedures used in the monitoring of CF patients include methods which expose patients to ionizing radiation. With increasing life expectancy in CF the cumulative dose of ionising radiation increases, prompting clinicians’ search for safer imaging studies. Despite its safety and availability lung ultrasound (LUS) is not routinely used in the diagnostic evaluation of CF patients. The aim of the study was to evaluate the diagnostic value of LUS in children with CF compared to a chest X-ray, and to assess the diagnostic value of the recently developed LUS score—CF-USS (Cystic Fibrosis Ultrasound Score). LUS was performed in 48 CF children and adolescents aged from 5 to 18 years (24 girls and 24 boys). LUS consisted of the assessment of the pleura, lung sliding, A-line and B-line artefacts, "lung rockets", alveolar consolidations, air bronchogram and pleural effusion. Chest radiography was performed in all patients and analyzed according to the modified Chrispin-Norman score. LUS was analyzed according to CF-USS. The correlation between the CF-USS and the modified Chrispin-Norman scores was moderate (R = 0.52, p = 0.0002) and strong in control studies. In 75% of patients undergoing LUS, small areas of subpleural consolidations were observed, which were not visible on x-rays. At the same time, LUS was not sensitive enough to visualize bronchial pathology, which plays an important role in assessing the progression of the disease. Conclusions: LUS constitutes an invaluable tool for the diagnosis of subpleural consolidations. CF-USS results correlate with the conventional x-ray modified Chrispin–Norman score. LUS should be considered a supplementary radiographic examination in the monitoring of CF patients, and CF-USS may provide clinicians with valuable information concerning the progression of the disease.

Lung ultrasound for the diagnosis of cystic fibrosis pulmonary exacerbation

“…Magnetic resonance imaging (MRI) is another modality that has recently been proposed for life-long imaging surveillance of CF patients, with the advantage of lacking ionizing radiation [ 10 ]. Ultrasound is currently the most commonly used imaging technique; therefore, lung ultrasound (LUS) can be an important tool in the evaluation of children with CF having multiple advantages, including availability, cost-effectiveness, non-invasiveness, safety, and bedside usability in critically ill patients [ 11 , 12 ]. In the current study, we aimed to determine the diagnostic performance of LUS in the evaluation of CF pulmonary exacerbation.…”

Ten Years of Pediatric Lung Ultrasound: A Narrative Review

“…Lower-frequency transducers are used for older children and overweight or obese adolescents ( Lovrenski et al, 2016 ). Curved array transducers are used to insonate between ribs, below the diaphragm, or from the suprasternal notch ( Joshi et al, 2019 ; Strzelczuk-Judka et al, 2019 ).…”

Lung ultrasound in children, WFUMB review paper (part 2)

“…The presence of interstitial syn-drome may indicate the presence of fluid in the interstitial space (for instance, in pulmonary fibrosis) or inflammatory infiltrate within this space (fig 1). In paediatric patients, this syndrome most often occurs in the course of lower respiratory tract infections (especially viral pneumonia), acute respiratory distress syndrome, cardiogenic pulmonary oedema, hyperhydration of patients receiving haemodialysis, interstitial lung disease, cystic fibrosis and post-obstructive pulmonary oedema [4][5][6][7].…”

“…A study suggested the presence of B lines were typical for small bronchiec-tasis [68], but it has been demonstrated that B line profile lacks specificity as they are seen in several diseases including interstitial inflammation and fibrosis [69]. It has been stated that a good correlation exists between LUS, expressed by an adapted bronchiolitis score [13] and chest X-ray evaluated by a modified Crispin Norman score, highlighting that, given the variability of the diseases described by B lines, it would be inadequate to make the diagnosis of CF lung only by ultrasound [6]. A small study compared a LUS score (evaluating only the presence of consolidation and B-lines pattern) with the modified Bhalla CT score and showed a correlation between the two methods and some partial correlation with the lung function expressed by FEV1 and FVC [70].…”