[Pulmonary lymphangioleiomyomatosis. Case report].

Pulmonary lymphangioleyomiomatosis (LAM) is a rare disease that affects only women. About one hundred cases are reported in the literature. This disease progresses to respiratory failure and its course can be slowed by hormonal therapy. A new case is reported; this woman was admitted to our Division for a right hydropneumothorax and after pleural drainage fluid demonstrated to be a chylous effusion; after a thoraco-abdominal CT scan the diagnosis of LAM was suspected. She was operated on after a few days because of continuous air and chyle leakage. A closure of lung leakage was performed with a stapler device that allowed the removal of a small specimen of lung for histopathological examination and a parietal pleurectomy was added with the aim of obtaining a pleural space obliteration. The histopathological examination revealed changes consistent with LAM and also positive was the hormonal steroid receptors assay. Adhesion of the lung to the chest wall was obtained but an antero-basal pouch persisted with continuous chylous leakage. A transposition of omentum into the chest was made with the aim of obliterating the pouch and favour chyle reabsorption and no drainage was left. Another CT scan demonstrated complete obliteration of the pouch and no fluid accumulation; the procedure proved to be successful and the patient was discharged with hormonal therapy.