Birt-Hogg-Dubé Syndrome with Floppy Cysts
Imaging findings
CT demonstrates multiple thin-walled, floppy, irregular pulmonary cysts predominantly in the lower lung zones, located in close association with the pulmonary veins and pleural surfaces, in a 55-year-old male with subtle fibrofolliculomas.
Key takeaways
The pulmonary cysts in Birt-Hogg-Dubé syndrome are typically thin-walled, irregularly shaped, and located in the subpleural and perivascular regions of the lower lobes, distinguishing them from the diffuse cysts of lymphangioleiomyomatosis.
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