Abstract
We reported a case of Budd-Chiari syndrome without stenosis of inferior vena cava. The case was a 33 years old man who consulted a doctor because of general fatigue and liver dysfunction pointed out at the time of health examination. Ultrasonography was performed for screening of liver dysfunction and we found an obstruction of left hepatic vein and recognized a strong echo at the outflow portion of right hepatic vein. He was admitted to our hospital on October 8, 1986. By this time he hada moderate hepatomegaly, but he had no ascites, no edema in legs, and no venous dilatation in the abdominal wall. We did hepatic vein catheterization and confirmed the stenosis of right hepatic vein at the portion of just before the IVC, flowing of the mid hepatic vein into right hepatic vein, obstruction of the left hepatic outflow, and no stenosis at the hepatic portion of the IVC. We diagnosed the case as Budd-Chiari syndrome without stenosis at the hepatic portion of IVC (Chiari disease).
In this case, an aggressive surgery was carried out. The stenosis was removed under the direct visualization, and the membranous obstruction of the right hepatic vein was seen macroscopically and histologically. There was a small hole, 2mm in diameter, in the center of the membrane. Presently, 2 years after the operation, he is healthy and has no abnormal findings in medical examinations and no ultrasonic findings.
In Japan, Chiari disease is very rare. We reported this case with 12 other Japanese cases of Chiari disease reported in the literature after 1970
