Pneumothorax as first evidence of unknown lymphangioleiomyomatosis

Abstract

Pneumothorax is often the first manifestation of unknown lymphangioleiomyomatosis. In the presence of a persistent air leak, we prefer to do a small resection of the lung to be certain of the diagnosis because many conditions can mimic lymphangioleiomyomatosis. The surgical procedure must be completed with a pleurodesis to avoid recurrent pneumothorax.