Williams-Campbell syndrome

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:

History and etymology

The syndrome was first described by Howard Williams and Peter Campbell in 1960 .