1Department of Gastroenterology, Jichi Medical School, 2Department of Cardiovascular surgery, Jichi Medical School, 3Department of General surgery, Jichi Medical School
Budd-Chiari syndrome, color Doppler ultrasonography, direct removal of thrombus, inferior vena cava thrombosis, vasculo-Behcet’s disease
A 31-year-old man had experienced repeated oral ulcerations since 1991, and genital ulceration and pain and swelling of the legs had started in September 2000. He was admitted to our institution in December 2000 complaining of back pain and abdominal distension. He had several aphthous lesions in the oral cavity, eruptions on the right leg, scarring from genital ulceration, and a positive needle reaction. Laboratory tests for inflammation and liver function were normal. Ultrasonography showed total occlusion of the hepatic portion of the inferior vena cava and the roots of the right hepatic vein, and thrombi in both sites. Color Doppler imaging showed blood flowing from the right hepatic vein to the middle hepatic vein, and regurgitation from the left hepatic veins. Portal flow drained into the veins of the abdominal wall through collaterals, predominantly through the paraumbilical vein. Thus our diagnosis was Budd-Chiari syndrome resulting from vasculo-Behcet's disease with thrombosis of the inferior vena cava. When liver function worsened despite initiation of medical treatment, including administration of prednisolone and anti-coagulation therapy, the occluded inferior vena cava and right hepatic vein were reconstructed by direct removal of the thrombus. Collateral flow decreased with normalization of flow in the inferior vena cava. However, thrombosis of the inferior vena cava recurred in the early postoperative period. Massive thrombosis from the inferior vena cava extended to all the hepatic veins, and collateral blood flow returned. Color Doppler imaging proved useful in evaluating the hemodynamics of this condition, but further evaluations will be necessary to determine the usefulness of direct removal of thrombi in vasculo-Bechet's disease.