Williams-Campbell-Syndrom
Beyond
bronchitis: a review of the congenital and acquired abnormalities of the bronchus. Williams-Campbell syndrome. a Frontal chest x-ray demonstrating bilateral architectural distortion and coarse interstitial markings with cystic appearing regions in the lower lobe consistent with the scarring and diffuse saccular bronchiectasis seen in Williams-Campbell syndrome. b Coronal CT imaging demonstrating the severe bilateral cystic bronchiectasis of the subsegmental (4th-6th generation) bronchi commonly seen in Williams-Campbell syndrome
Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. Imaging findings of congenital diseases causing bronchial dilatation. 3D reconstruction (a) and coronal CT image (b) show dilatation of the main airways in a patient with Mounier-Kuhn syndrome. Bronchiectasis, centrilobular opacities, and increased density predominantly affecting the lower lobes of the lungs are seen in a patient diagnosed with Kartagener (c, d). Upper lobe-predominant bronchiectasis and thickened bronchial walls are observed in cystic fibrosis (e–g). Severe and bilateral cystic bronchiectasis in the subsegmental bronchi (4th and 6th generation) is seen in a patient with Williams–Campbell syndrome (h)
Williams-Campbell syndrome (WCS) is a rare form of congenital cystic bronchiectasis, in which distal bronchial cartilage is defective.
Clinical presentation
Williams-Campbell syndrome may present with recurrent pneumonia, wheezing, barrel-chest deformity, and Harrison sulcus .
Pathology
It is thought to result froma deficiency of cartilage formation in the 4 to 6 order sub-segmental bronchi, resulting in distal airways collapse .
Radiographic features
CT
Cystic central bronchiectasis changes, usually symmetric and bilateral, typically involving 4 to 6order bronchi with preservation of the trachea and main bronchi .
Differential diagnosis
All causes of diffuse and bilateral bronchiectasis including:
- cystic fibrosis
- allergic bronchopulmonary aspergillosis (ABPA)
- Mounier Kuhn syndrome
- primary ciliary dyskinesia
History and etymology
The syndrome was first described by Howard Williams and Peter Campbell in 1960 .
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Assoziationen und Differentialdiagnosen zu Williams-Campbell-Syndrom:
