This case reports the successful management of
chylothorax in a non-transplanted patient with pulmonary
lymphangioleiomyomatosis (pLAM). Prolonged initial
therapy failed, including
total parenteral nutrition, pleural drainage, surgical
pleurodesis, and pleurectomy. Commencement of
sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of
chylothorax after 20 days. Discontinuation of
sirolimus for abdominal surgery led to recurrence of the
chylothorax. Reinstitution of
sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years. We conclude that
sirolimus should be considered in the management of pLAM-related
chylothorax, perhaps before surgical intervention.