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Williams-Campbell syndrome (WCS) is a rare form of congenital cystic bronchiectasis, in which distal bronchial cartilage is defective.
Williams-Campbell syndrome may present with recurrent pneumonia, wheezing, barrel-chest deformity, and Harrison sulcus 8.
It is thought to develop from a deficiency of cartilage formation in the 4th to 6th order sub-segmental bronchi, resulting in distal airways collapse and bronchiectasis 6,7.
Cystic central bronchiectasis changes, usually symmetric and bilateral, typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2. On expiratory aquisitions, there is characteristic collapse of affected bronchi 9.
History and etymology
The syndrome was first described by Howard Williams and Peter Campbell in 1960 8.
All causes of diffuse and bilateral bronchiectasis including: