[Lymphangioleiomyomatosis of lungs and spontaneous pneumochylothorax].

Lymphangioleiomyomatosis occupies a special place among rare systemic diseases of lungs, which are manifested by respiratory failure and the gradual development of pulmonary heart. Manifestations of the pleural cavity in the form of pneumothorax and pleural effusion syndrome are characteristic for the disease. In order to verify the process, the authors recommend a study of pleural punctate with Sudan-III for detection of lymph and a CT-scanning after removal of the contents of the pleural cavity. "Cell" pattern of lungs in combination with chylothorax confirm the diagnosis of lymphangioleiomyomatosis. In particularly complex cases, in their opinion, the lung and pleura biopsy is required.