CTICasesBirt-Hogg-Dubé Syndrome
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Imaging findings
Chest CT demonstrates a moderate-sized right pneumothorax. The lung parenchyma contains multiple atypical, thin-walled, lentiform, and cylindrical subpleural cystic airspaces. These cysts predominantly follow the subpleural regions and bronchovascular bundles in the lower lobes.
Key takeaways
Birt-Hogg-Dubé syndrome is an autosomal dominant genetic disorder characterized by lung cysts, recurrent spontaneous pneumothoraces, renal tumors, and fibrofolliculomas. The pulmonary cysts are characteristically lentiform or oval-shaped and typically follow a subpleural or basilar, peribronchovascular distribution.
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