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Williams Campbell syndrome

The Williams Campbell syndrome is considered a rare form of congenital cystic bronchiectasis. There appears to be some overlap in the definition of the two entities.

Pathology

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

Radiographic features

HRCT
  • Shows cystic bronchiectatic changes typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2

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