[Pulmonary imaging of cystic fibrosis with a focus on children and adolescents]

CLINICAL ISSUE: Cystic fibrosis (CF) is the most common autosomal recessive multisystemic disease in Europe, with pulmonary complications significantly influencing patients' morbidity and mortality. The objective of this review is to compare different imaging modalities and present characteristic pulmonary CF manifestations. METHODS: X‑ray examinations of the chest are often considered the basic diagnostic tool increasingly replaced by magnetic resonance imaging (MRI). Cross-sectional imaging techniques (computed tomography [CT] and MRI) provide a more sensitive diagnostic of pulmonary manifestations of CF. Characteristic manifestations are-among others-bronchial dilatation, bronchial wall thickening, mucus retention, bronchiolitis pathologies with "tree-in-bud sign" and centrilobular nodules, consolidations, mosaic pattern with ground-glass opacities and air trapping. Typical pulmonary complications include acute exacerbations with inflammatory infiltrates and allergic bronchopulmonary aspergillosis (ABPA) with specific radiologic changes. METHODOLOGICAL INNOVATIONS: Because of improved technologies, radiation exposure of CT examinations is continuously decreasing. MRI also shows continuous technical optimization. An important technical advance of MRI is the ability to obtain functional perfusion and ventilation images without radiation exposure and without intravenous contrast agent application. PRACTICAL RECOMMENDATIONS: Due to improved treatment options-in particular modulator therapy-pulmonary manifestations typical of CF are today less pronounced than in the past. CT and MRI are suitable for therapy monitoring, with MRI being preferable because of its absence of radiation exposure and the possibility of functional information.

• Huisinga, C.
• Böttcher, J.
• Pfeil, A.
• Paquet, S.
• Kaireit, T. F.
• Dittrich, A. M.
• Renz, D. M.