Williams-Campbell syndrome

Williams-Campbell syndrome (WCS) is a rare form of congenital cystic bronchiectasis, in which distal bronchial cartilage is defective.

Pathology

It is thought to result from a deficiency of cartilage formation in the 4th to 6th order segmental bronchi.

Radiographic features

HRCT
  • shows cystic bronchiectatic changes, usually symmetric and bilateral, typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2

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