CTICasesWilliams-Campbell Syndrome
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Imaging findings
Chest radiography and CT show severe, bulbous bronchiectasis localized predominantly to the lower lobes, with dilated fourth-to-sixth order bronchi filled with secretions, bronchial wall calcifications (broncholiths), and prominent bronchial artery hypertrophy. The trachea and central bronchi are normal in caliber.
Key takeaways
Williams-Campbell syndrome is a rare congenital bronchopathy characterized by a deficiency of cartilage in the subsegmental bronchi, leading to bronchiectasis and recurrent infections. While classically diffuse, localized or lower-lobe predominant variants can occur, and long-standing chronic infection can result in broncholithiasis and bronchial artery hypertrophy.
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