Early chylopneumothorax in a patient with pulmonary lymphangioleiomyomatosis

A 50-year-old woman was admitted with the complaint of cough and dyspnea on exertion for the previous two months. A radiograph of the chest showed a right-sided hydropneumothorax, which was proven to be a chylous effusion by lipoprotein electrophoresis and was very refractory to tetracycline-pleurodesis. The chylopneumothorax was cured by ligation of the thoracic duct and surgical pleurodesis. After an open lung biopsy, lymphangioleiomyomatosis (LAM) was diagnosed histopathologically with smooth muscle nodules scattered throughout the lungs, obstructing the small airways, venules and lymphatics. An immunohistochemistry study using the avidin biotin complex method with monoclonal antibodies for actin and desmin showed the small nodules to be of muscle origin. During the past two years, the patient has remained stable both in respiratory status and roentgenographically without hormonal manipulation or oophorectomy. We present this case to illustrate the heterogeneous nature of this condition. While our patient's initial presentation was acute and associated with chylothorax, her postoperative course has shown no progression despite withholding of hormonal therapy.