CTICasesWilliams-Campbell syndrome
Imaging findings
CT scan of a 56-year-old male with chronic cough shows severe, symmetric cystic bronchiectasis involving the segmental and subsegmental bronchi, with sparing of the trachea and main bronchi. Left lower lobectomy pathology confirmed a developmental deficiency of bronchial cartilage.
Key takeaways
Williams-Campbell syndrome is a rare congenital disorder characterized by a deficiency of cartilage in the sublobar (segmental and subsegmental) bronchi, leading to bronchiectasis, airway collapse during expiration, and recurrent infections.
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