患者男,17岁,腹胀、食欲下降4月余。体检见全身皮肤、巩膜轻度黄染,腹部膨隆,移动性浊音阳性。总胆红素56.6μmol/L。彩超示:肝脏增大,脾大,大量腹水;肝左静脉、肝中静脉主干约5 mm,近心段均闭塞,经交通支引流;肝右静脉显示不清,肝内探及多条副肝静脉。腹部MRA示三支肝静脉显示不佳,肝内未示明显侧支血管影,腹水征(图1),两侧胸腔少量积液。诊断肝静脉型布-加综合征(buddchiari syndrome,BCS)。采用Seldinger法穿刺右颈内静脉,下腔静脉造影未见 Male patient, 17 years old, bloating, loss of appetite for more than 4 months. Physical examination see the whole body skin, scleral mild yellow dye, abdominal bulging, shifting dullness positive. Total bilirubin 56.6 μmol / L. Color ultrasonography: liver enlargement, splenomegaly, a large amount of ascites; liver left vena cava, the middle of the hepatic vein about 5 mm, the proximal segment were occluded, the traffic branch drainage; right hepatic vein showed unclear, intrahepatic exploration and a number of deputy Hepatic vein. Abdominal MRA showed poor visualization of the three hepatic veins, no evidence of collateral vessels in the liver, signs of ascites (Figure 1), and a small amount of effusion on both sides of the chest. Diagnosis of hepatic vein type Budd-Chiari syndrome (buddchiari syndrome, BCS). Seldinger puncture the right internal jugular vein, inferior vena cava angiography was not seen