Williams-Campbell syndrome

Case contributed by Dr Michael P Hartung

Presentation

Shortness of breath and productive cough. Reported history of emphysema.

Patient Data

Age: 55 years
Gender: Male
CT

Day 1

Saber-sheath configuration of the trachea and mild paraseptal emphysema. Extensive central, cystic bronchiectasis with bronchial secretions, mucous plugs, dilated bronchi with air-fluid levels, and lower lobe consolidation. Specific note is made that the trachea is not dilated. The pattern of bronchial dilation is most pronounced in the mid (4th-6th order) bronchi.

X-ray

Day 1

Diffusely coarsened interstitium of the mid through lower lung fields. Mid-lower lung bronchiectasis with air-fluid levels. 

Case Discussion

Bronchiectasis represents irreversible dilation of the airway due to infection, obstruction, or congenital abnormalities. The three types of bronchiectasis are cylindrical (smooth, tubular), varicose (undulating, irregular), and cystic (saccular dilation). In this case, there is diffuse cystic bronchiectasis with a central predominance. The differential diagnosis would be allergic bronchopulmonary aspergillosis (ABPA), Mounier-Kuhn syndrome (tracheobronchomegaly), and Williams-Campbell (WC) syndrome. ABPA results in mucoid impactions and a characteristic "finger in glove" appearance with areas of bronchiolitis distally. Mounier-Kuhn syndrome affects the trachea, mainstem bronchi, and distal airways. WC is a congenital cartilage disorder mostly affecting the mid-bronchi (4th-6th order).

The dramatic appearance of the bilateral, diffuse, central cystic bronchiectasis with air-mucus levels could be mistaken for cystic lung disease. Given the extensive nature of the disease, a congenital cause is most likely. The trachea is narrowed (saber sheath appearance) rather than dilated, which would be the major feature of Mounier-Kuhn syndrome. There is some enlargement of the mainstem bronchi; however, the predominant feature in this case is dilation of the middle (4th-6th order) bronchi, making WC the most appropriate diagnosis. There may be some overlap in the spectrum of the underlying cartilage deficiency resulting in this appearance, or perhaps multiple bouts of infection and inflammation have further exacerbated his imaging findings. Surprisingly, the patient was not aware of this diagnosis prior to this study despite his later age of presentation.

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Case information

rID: 57467
Published: 2nd Jan 2018
Last edited: 23rd Jun 2019
System: Chest
Inclusion in quiz mode: Included

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