CTICasesPresumed Williams-Campbell Syndrome
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Imaging findings
Chest CT demonstrates central, symmetric, cystic-appearing bronchiectasis extending from the fourth- to sixth-order bronchi with relative sparing of the trachea, main bronchi, and the subpleural lung periphery. There is mild associated tree-in-bud nodularity and airway secretions.
Key takeaways
Williams-Campbell syndrome is a rare congenital disorder characterized by a deficiency of cartilage in the subsegmental bronchi, resulting in inspiratory ballooning and expiratory collapse. Although typically diagnosed in infancy, rare adult-diagnosed cases present with recurrent pulmonary infections and a characteristic morphologic pattern of central bronchiectasis with sparing of the central airways.
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