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Published online by Cambridge University Press: 07 October 2011
Imaging
Williams-Campbell syndrome is characterized by cystic bronchiectasis in the fourth to sixth order bronchi [1, 2] (Figure 8.1–8.3). If expiratory images are obtained, collapse of the bronchiectatic segments can be seen [1–3]. Hyperinflation or emphysematous changes may also be seen in the lung distal to the bronchiectatic regions. The cause of the bronchiectasis is due to defective or absent cartilage in the walls of the fourth to sixth order bronchi.
Importance
Williams-Campbell syndrome has been postulated to be a congenital abnormality. At least two separate reports have shown a familial occurrence [3]. Therefore, recognition of this abnormality in a patient should lead to additional investigations of family members.
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