Epidemiology of Budd-Chiari syndrome and its treatment by IVR

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Imaging diagnosis of Budd-Chiari Syndrome (BCS) being easier and more accurate by the definite criteria of the disease, however, BCS is rather rare in Japan statistically. In this study epidemiological investigation was performed by the co-operation of Chinese hospitals, where more BCS patients were available. Fifty-four patients (33 males, 21 females) of BCS over 15 years of age were subjected to study for the symptoms and laboratory findings as well as the recent dietary habit of the BCS patients, compared with the 14 control patients of each circulatory and digestive disease in accordance with the age and sex.The average onset ages were 30.3 years for males and 18.7 for females. Sex ratio was 1.57 ; more male than female. There was no characteristic family history nor past history. Abdominal distension and edema in the lower extremities were found in more than 75% of male and female patients. In males, melena, hematemesis and abdominal pain were frequently observed and abdominal pain and anorexia in females. It was noted that serum albumin was low and many patients had anemia and low platelet counts. More than 50% of the patients had enlarged liver, splenomegaly, esophageal varicies and dilated tortuous veins on the skin of abdominal and/or chest region. Smoking was not related to the disease. As for diet, BCS patients had more frequently potatoes and less frequently dried bean paste, rice in the morning, pork, vegetables and pear than 2 control groups. For fundamental study, experimental rat BCS model was developed by the use of laminaria constrictor.Thirty-two patients of BCS were treated by interventional radiology (IVR). The initial treatment of 21 cases was percutaneous transluminal angioplasty (PTA)) only and later 11 cases were treated by expandable metallic stent (EMS) from the beginning. Re-stenosis was treated by PTA and/or EMS. The long term follow-up encourages the good outcome of IVR on BCS.