经颈静脉肝内门体分流术及肝静脉成形术治疗肝静脉型Budd-Chiari综合征的临床意义

目的分析经颈静脉肝内门体分流术(TIPS)及经皮肝或经颈静脉途径肝静脉成形术等介入手段治疗肝静脉型Budd-Chiari综合征(BCS)的临床意义。方法回顾性分析2000年5月至2012年10月收治的32例肝静脉型BCS的临床资料。其中男15例,女17例;平均年龄(38±6)岁;肝功能Child-Pugh评分(9.6±2.2)分;肝静脉近段闭塞8例,肝静脉闭塞合并下腔静脉狭窄4例,全肝静脉闭塞11例,肝小静脉闭塞9例;急性3例,亚急性或慢性29例。患者主要临床表现为顽固性腹水和食管静脉曲张破裂出血,采用彩色多普勒超声、CT血管造影或MRI血管造影、上消化道钡餐及内镜检查明确诊断。治疗方法包括:TIPS 9例,改良TIPS 11例,单独经皮肝或经颈静脉途径肝静脉成形术8例,经皮肝肝静脉成形术联合下腔静脉成形术4例。结果所有患者均成功完成介入或手术治疗,其中TIPS或改良TIPS术后,患者出血控制,腹水逐渐消退,肝功能明显好转;门静脉压力由(42±8)cm H2O(1 cm H2O=0.098 kPa)下降至(27±5)cm H2O(t=20.20,P=0.001),门静脉血流速度由(18±6)cm/s增加至(52±10)cm/s(t=15.02,P=0.001)。住院期间因肝功能不全死亡1例,肝门分流道急性阻塞1例。术后随访3~241个月,平均随访(102±26)个月,分流道狭窄性扩张内支撑2例,肝静脉狭窄再扩张2例。结论经皮肝或经颈静脉途径肝静脉成形术与TIPS均是治疗肝静脉型BCS的有效方法,可根据患者病情酌情选择治疗方法。

Clinical outcomes of transjugular intrahepatic portosystemic shunt and hepatic venous angioplasty in treatment of Budd-Chiari syndrome with hepatic veins occlusion

Objective To evaluate the clinical outcomes of transjugular intrahepatic portosystemic shunt（ TIPS） and hepatic venous angioplasty in the treatment of Budd-Chiari syndrome（ BCS） with hepatic vein occlusion. Methods The clinical data of 32 BCS patients with hepatic vein occlusion treated from May2000 to October 2012 were retrospectively analyzed. There were 15 males and 17 females with an average age of（ 38 ± 6） years. Preoperative Child-Pugh scores were 9. 6 ± 2. 2 on average. Hepatic vein proximal occlusion occurred in 8 cases,hepatic vein occlusion combined with inferior vena cava stenosis in 4,whole hepatic vein occlusion in 11,and hepatic venule occlusion in 9. Of them,3 patients were in acute course,and 29 were in subacute or chronic course. The typical clinical manifestations included refractory ascites and esophageal varices bleeding. Doppler ultrasound,CT or MR angiography,upper gastrointestinal barium meal and gastroscopy were used to make a definitive diagnosis. Surgical treatments included TIPS in 9 patients,modified TIPS in 11,hepatic venous angioplasty in 8,and hepatic venous and inferior vena caval angioplasty in 4. Results The interventional and surgical treatments were successfully achieved in all patients. After TIPS or modified TIPS,hemorrhage was controlled,ascites reduced gradually,liver function was improved significantly; portal venous pressure decreased from（ 42 ± 8） cmH2 O to（ 27 ± 5） cmH2O（ 1 cm H2 O = 0. 098 kPa,t = 20. 20,P = 0. 001） and portal venous flow velocity increased from（ 18 ± 6） cm /s to（ 52 ± 10） cm /s（ t =15.02,P = 0.001）. During the hospitalization,one patient died due to hepatic failure and the acute occlusion of shunt occurred in another patient. The follow-up period was（ 102 ± 26） months on average（ range,3 to 241 months）. The revisions of TIPS shunt were performed in 2 patients and the reinflation of stenotic hepatic vein in another 2 patients during the follow-up. Conclusions Both hepatic venous angioplasty and TIPS ca