Figure 4

Radiological features. A and B: CT scan from a girl at the ages of 9 (A) and 13 (B) years old. A: Typical aspect of crazy paving pattern on thin axial sections: extensive areas of ground glass opacities (asterisks) superimposed with interlobular septa thickening (black arrows) and intralobular lines (black arrowheads). Irregular thickening of fissures are also noted (white arrows). B: Ground glass opacities, interlobular septa thickening and intralobular lines are less extensive than on the first CT scan while cystic lesions of a few millimetres (white arrowheads) are observed, with a subpleural distribution. Irregular traction bronchiectasis (white arrows) appeared that are initial signs of pulmonary fibrosis. C and D: CT scan of a girl at the ages of 8 months (C) and 5 years old (D). C: This CT scan image shows a symmetric combination of extensive ground-glass opacities (asterisks), intralobular lines (arrowheads) and interlobular septa thickening (black arrows), associated to consolidation (black curved arrows) in posterior areas of the lungs. Note the airspace hyperinflation in the anterior compartment of the lung (white asterisks) responsible of an increasing density gradient from anterior to posterior areas. D: Extensive ground-glass opacities, intralobular lines and interlobular septa thickening are still present. Subpleural cystic lesions (white arrowheads) and signs of fibrosis: honey-combing (white curved arrows) and traction bronchiectasis (white arrows) appeared.