巴德－吉亚利综合征120例外科治疗分析

Analysis of surgical treatment results in 120 cases of Budd-Chiari syndrome

目的分析巴德-吉亚利综合征（BCS）的外科治疗方法及其疗效。方法回顾性分析2001年7月至2010年10月接受序贯治疗的120例BCS患者的临床资料。男性82例，女性38例；年龄11-72岁，平均（41±13）岁。BCS病变类型包括肝小静脉型5例、肝静脉型28例、下腔静脉型31例和肝静脉及下腔静脉联合梗阻型56例。本组25例患者接受介入治疗，包括肝静脉和（或）下腔静脉狭窄段的经皮腔内血管成形术或血管成形支架置人术；77例行右心房转流、部分心肺转流辅助下的经胸BCS根治术；其他方式治疗18例。结果本组随访1-120个月，随访率80．8％（97／120），围手术期病死率6．2％（6／97），随访期间病死率8．2％（8／97）。介入治疗后有3例术后发生下腔静脉、肝静脉再狭窄；根治术后有15例患者发生下腔静脉、肝静脉闭塞或重度狭窄；根治术后下腔静脉及肝静脉5年通畅率和生存率分别为64．5％和83．3％。结论根据BCS病变类型选择不同治疗方式，可以获得良好的治疗效果。根治术对特定病理类型及介入治疗失败病例是理想的治疗方法。

Objective To evaluate the results of the surgical treatment of patients with Budd-Chiari syndrome （BCS）. Methods The clinic data of 120 BCS patients who underwent various consecutive surgical treatments from July 2001 to October 2010 was analyzed. There were 82 male and 38 female patients, aging fi＇om 11 to 72 years with a mean age of （ 41±13 ） years. All patients experienced various examinations to indentify the pathological type of BCS. There were 5 cases of small hepatie veins type, 28 cases of large hepatic veins （LHV） type, 31 cases of inferior vena eava （ IVC ） type and 56 cases of combined obstruction of LHV and IVC. Totally, 25 patients experienced interventional treatment, include percutaneous transluminal angioplasty and/or stenting for stenosis of hepatic vein and/or IVC, 77 patients experienced open-thorax operation for BCS radical resection under protection of right atrium by-pass with extraeorporeal circulation. Results Totally 97 cases were followed up from 1 to 120 months after various surgical treatment methods. Perioperative mortality was 6.2% （6/97）. Follow-up period mortality was 8.2% （8/97）. The restenosis of IVC and/or hepatic vein happened in 3 cases out of 25 cases in intervention treatment group in contrast with 15 cases out of 77 cases in radical resection group. The 5-year pateney and survival rate of IVC/hepatic vein were 64. 5% and 83.3% . Conclusions The surgical treatment of BCS need to get accurate diagnosis and pathological classification firstly, then, to choose appropriate therapeutic strategies based on individual pathological classification. The BCS radical reseetion can be an alternative method in some particular pathological classifications and the cases who failed in interventional treatment.