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Lymphangioleiomyomatosis with chylous ascites
A A Raymond1, M R Isa, T Abdullah
1Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.
Singapore Medical Journal
|June 1, 1993
Summary
This report details a rare case of lymphangioleiomyomatosis (LAM) in a Malaysian woman presenting with chylous ascites and cachexia. The diagnosis was confirmed through surgical biopsy, highlighting a unique presentation without pulmonary involvement.
Area of Science:
- Medicine
- Rare Diseases
- Gastroenterology
Background:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive disease characterized by smooth muscle cell proliferation.
- It primarily affects the lungs but can manifest in other organs, including the liver and peritoneum.
Observation:
- A 41-year-old Malay woman experienced 17 years of recurrent chylous ascites and cachexia.
- Clinical and radiological evaluations showed no signs of pulmonary LAM.
Findings:
- Laparotomy and subsequent biopsy confirmed LAM, revealing smooth muscle proliferation in abdominal blood vessels and lymphatics.
- The patient's condition was managed with dietary fat restriction and medium-chain triglycerides.
Implications:
- This case represents the first documented instance of LAM in Malaysia.
- It underscores the importance of considering extrapulmonary LAM in patients with unexplained chylous ascites and cachexia, even without lung symptoms.