Lymphangiomyomatosis with chylous ascites: treatment with dietary fat restriction and medium chain triglycerides

Lymphangiomyomyoma is a tumor of lymphatic channels and lymph nodes, clinically manifested by recurrent chylous pleural effusions and ascites. The disease is usually progressive and unresponsive to surgery, chemotherapy, or irradiation. A case of lymphangiomyomatosis in a 24-year-old woman who presented with chylous ascites is described. The patient was treated with dietary fat restriction supplemented with medium chain triglycerides and has remained free of recurrent effusions for 2 years.