[Lymphangiomatosis as the cause of therapy refractory chylothorax and ascites].

After five weeks of progressing dyspnoea and pain in the lower abdomen a 44-year old woman was admitted in our hospital unit. A massive chylothorax was identified as the cause of the dyspnoea. Clinical and pathological findings were consistent with the diagnosis of lymphangiomyomatosis. The ligature of the thoracic duct as well as pleurodesis and the application of medroxyprogesterone were without any significant effect. Severe complications made further treatment in the intensive-care unit imperative. Symptomatic treatment led only to a short remission. Five months after the outbreak of the disease the patient died from multiple organ failure.