Williams-Campbell syndrome
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Williams-Campbell syndrome (WCS) is arare 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 8.
Pathology
It is thought to result froma deficiency of cartilage formation in the 4th to 6th order sub-segmental bronchi, resulting in distal airways collapse 7.
Radiographic features
CT
Cystic central bronchiectasis changes, usually symmetric and bilateral, typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2.
Differential diagnosis
All causes of diffuse and bilateral bronchiectasis including:
- cystic fibrosis
- allergic bronchopulmonary aspergillosis (ABPA)
- Mounier Kuhn syndrome
- primary ciliary dyskinesia
History and etymology
The syndrome was first described by Howard Williams and Peter Campbell in 1960 8.
-<p><strong>Williams-Campbell syndrome (WCS)</strong> is a<sup> </sup>rare form of <a href="/articles/congenital-cystic-bronchiectasis">congenital cystic bronchiectasis</a>, in which distal bronchial cartilage is defective.</p><h4>Clinical presentation</h4><p>Williams-Campbell syndrome may present with recurrent pneumonia, wheezing, barrel-chest deformity, and <a href="/articles/harrison-sulcus">Harrison sulcus</a> <sup>8</sup>.</p><h4>Pathology</h4><p>It is thought to result from<sup> </sup>a deficiency of cartilage formation in the 4<sup>th</sup> to 6<sup>th</sup> order sub-segmental bronchi, resulting in distal airways collapse <sup>7</sup>.</p><h4>Radiographic features</h4><h5>CT</h5><p>Cystic bronchiectasis changes, usually symmetric and bilateral, typically involving 4<sup>th</sup> to 6<sup>th </sup>order bronchi with preservation of the trachea and main bronchi <sup>2</sup>. </p><h4>History and etymology</h4><p>The syndrome was first described by <strong>Howard Williams</strong> and <strong>Peter Campbell</strong> in 1960 <sup>8</sup>.</p>- +<p><strong>Williams-Campbell syndrome (WCS)</strong> is a<sup> </sup>rare form of <a href="/articles/congenital-cystic-bronchiectasis">congenital cystic bronchiectasis</a>, in which distal bronchial cartilage is defective.</p><h4>Clinical presentation</h4><p>Williams-Campbell syndrome may present with recurrent pneumonia, wheezing, barrel-chest deformity, and <a href="/articles/harrison-sulcus">Harrison sulcus</a> <sup>8</sup>.</p><h4>Pathology</h4><p>It is thought to result from<sup> </sup>a deficiency of cartilage formation in the 4<sup>th</sup> to 6<sup>th</sup> order sub-segmental bronchi, resulting in distal airways collapse <sup>7</sup>.</p><h4>Radiographic features</h4><h5>CT</h5><p>Cystic <a href="/articles/central-bronchiectasis">central bronchiectasis</a> changes, usually symmetric and bilateral, typically involving 4<sup>th</sup> to 6<sup>th </sup>order bronchi with preservation of the trachea and main bronchi <sup>2</sup>. </p><h4>Differential diagnosis</h4><p>All causes of diffuse and bilateral <a title="Bronchiectasis" href="/articles/bronchiectasis">bronchiectasis</a> including:</p><ul>
- +<li><a href="/articles/cystic-fibrosis">cystic fibrosis</a></li>
- +<li><a href="/articles/allergic-bronchopulmonary-aspergillosis">allergic bronchopulmonary aspergillosis (ABPA)</a></li>
- +<li><a href="/articles/mounier-kuhn-syndrome">Mounier Kuhn syndrome</a></li>
- +<li>
- +<a title="primary ciliary dyskinesia" href="/articles/primary-ciliary-dyskinesia">primary ciliary dyskinesia</a><ul>
- +<li><a title="Kartagener syndrome" href="/articles/kartagener-syndrome-1">Kartagener syndrome</a></li>
- +<li><a title="Young syndrome" href="/articles/young-syndrome-1">Young syndrome</a></li>
- +</ul>
- +</li>
- +</ul><h4>History and etymology</h4><p>The syndrome was first described by <strong>Howard Williams</strong> and <strong>Peter Campbell</strong> in 1960 <sup>8</sup>.</p>
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