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Williams-Campbell syndrome

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 result from a deficiency of cartilage formation in the 4th to 6th order sub-segmental bronchi, resulting in distal airways collapse 7.

Cystic bronchiectasis changes, usually symmetric and bilateral, typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2

The syndrome was first described by Howard Williams and Peter Campbell in 1960 8.

Chest

Article information

rID: 9743
Synonyms or Alternate Spellings:
  • Williams-Campbell syndrome
  • Williams-Campbell syndrome (WCS)

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Cases and figures

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