CTICasesLymphangioleiomyomatosis presenting as Spontaneous Pneumothorax
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Imaging findings
Initial chest radiograph shows a large unilateral pneumothorax with underlying coarse, diffuse reticular opacities. Subsequent chest CT obtained after pleural catheter placement reveals extensive, thin-walled, round bilateral lung cysts of uniform size distributed symmetrically throughout all lung zones.
Key takeaways
Lymphangioleiomyomatosis is a progressive cystic lung disease that almost exclusively affects women of childbearing age, often presenting acutely with a spontaneous pneumothorax. On chest radiographs, the diffuse cysts can easily be mistaken for a reticular pattern, but chest CT is highly specific, showing uniform, thin-walled, round cysts with normal intervening lung parenchyma.
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