[Anesthetic approach in 2 patients with pulmonary lymphangiomyomatosis]

Pulmonary lymphangiomyomatosis (PLM) is an idiopathic disease of females in fertile age. It results in respiratory failure characterized by obliteration of the small airways, emphysema, formation of bullae, hemoptysis, pneumothorax, pulmonary fibrosis, severe hypoxemia and reduced carbon monoxide diffusion capacity. The major lymphatic ducts are also involved, resulting in chylous pleural effusion and ascites. Pulmonary abnormalities improve objectively and subjectively after surgical therapy, which consists in bilateral oophorectomy. Its evolution results in death in no more than 10 years. We report the anesthetic approach to 2 patients with PLM. Patient 1 was a female who had already been diagnosed of PLM and had received medical and surgical therapy, requiring reoperation for the resection of cystic intestinal masses and abdominal eventration. Patient 2 had been scheduled for bilateral oophorectomy after a diagnosis of PLM. We discuss the clinical condition of both patients, the course of the disease, the previous treatments and the anesthetic technique in each case.