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 .
It is thought to result froma deficiency of cartilage formation in the 4 to 6 order sub-segmental bronchi, resulting in distal airways collapse .
Cystic central bronchiectasis changes, usually symmetric and bilateral, typically involving 4 to 6order bronchi with preservation of the trachea and main bronchi .
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 .