Primary lymphangiomyomatosis is a benign tumour of lymphatic channels and lymph nodes, clinically manifested by chylous ascites. This disease is usually progressive and unresponsive to surgery, chemotherapy or irradiation. A case of a 36-year-old lady with chylous ascites due to underlying primary lymphangiomyomatosis is reported.
A 41-year-old Malay housewife presented with recurrent chylous ascites and progressive cachexia over 17 years. A diagnosis of lymphangioleiomyomatosis (LAM) was established by laparotomy where biopsy of the liver, peritoneum and adhesions from previous surgery showed smooth muscle proliferation in the blood vessels and lymphatics. Clinically and radiologically, there was no evidence of pulmonary involvement. She was treated with dietary fat restriction and medium-chain triglycerides. This is the first case of LAM reported in Malaysia.
The case report describes the presentation of a 19-year old female with tuberous sclerosis who presented with progressive dyspnoea over 2 days.
Chest radiograph revealed bilateral pneumothorax. Computed tomography showed features of pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas. The coexistence of both conditions may cause devastating morbidity and mortality.
Lymphangioleiomyomatosis (LAM) is a rare disease. The reported prevalence is around one per million in the West but the exact prevalence in Asia is unknown. It affects primarily young women of reproductive age, with a mean age of onset at 34 years. The disease is characterised by an abnormal proliferation of smooth muscle-like cells that grow over a course of time to obstruct airways, lymphatic and blood vessels. We report a case of LAM in a young woman who presented with a spontaneous pneumothorax. Computed tomography scan of the thorax and abdomen showed homogenous thin-walled cystic lesions with normal intervening lung parenchyma and multiple lesions in her kidneys with Hounsfield units similar to that of fat.