Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease that most commonly affects women of reproductive age. The disease is manifested by diffuse destruction of the pulmonary parenchyma with the formation of cysts on a characteristic finding of multidetector computerized tomography (MD-CT) of the chest. It is thought that the presence of cells with estrogen and progesterone receptors among proliferating smooth muscle cells lead to the spread of cystic structures lined by endothelial cells. Towards the end of pregnancy and after childbirth, collapse of the lung parenchyma or rupture of the cyst wall occurs causing a dramatic clinical picture in the form of pneumothorax. Sirolimus is the only drug of choice that should improve and stabilize the patient’s pulmonary function and quality of life. Unfortunately, this drug is not always effective enough, so only option for treatment is bilateral lung transplantation. The authors present the patient in the last trimester of pregnancy who was admitted to the hospital with clinical picture of bilateral spontaneous pneumothorax. Dramatic picture of pneumothorax could not be solved solely by drainage in which atypical segmental resection of the lung was performed within a few days so on these samples lymphangioleiomyomatosis were diagnosed.