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.