Spontaneous Pneumothorax in a Young Female With Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal proliferation smooth muscle-like cells which results in the formation thin-walled cysts and progressive lung destruction. It commonly presents with dyspnea that often associated history pneumothorax or chylothorax particularly among females reproductive age. In this report, we present case hydropneumothorax as initial presentation LAM 33-year-old woman, rather presentation. We also discuss pathogenetic mechanisms, diagnosis, treatment strategies using mTOR inhibitors like sirolimus.