CTICasesBirt-Hogg-Dube Syndrome with Spontaneous Pneumothorax
Imaging findings
CT shows scattered, thin-walled, small cystic spaces predominantly in the lower lobes. Cysts exhibit varied morphology, including round, elongated, and irregular shapes, some running perpendicular to the pleura or along vessels. These subtle cysts were associated with a spontaneous pneumothorax.
Key takeaways
Birt-Hogg-Dube syndrome is an autosomal dominant disorder characterized by cystic lung disease (often subtle and small cysts of varied morphology, often basal-predominant or along septa/vessels), spontaneous pneumothorax, skin lesions (fibrofolliculomas, acrochordons), and increased risk of renal cell cancer. A family history of cystic lung disease or renal cell cancer is highly suggestive.
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