CTICasesBirt-Hogg-Dube syndrome
Imaging findings
Chest CT shows multiple thin-walled, oval or elliptical lung cysts of variable sizes, mostly in a subpleural and basal/mid-lung distribution, with an associated spontaneous pneumothorax. Abdominal CT demonstrates a small, solid renal tumor.
Key takeaways
Birt-Hogg-Dube syndrome is a rare autosomal dominant condition characterized by pulmonary cysts, spontaneous pneumothorax, fibrofolliculomas of the skin, and renal tumors. The cysts typically have a lower lung, subpleural, or septal/fissural predominance and are often oval or lenticular.
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