布加综合征合并肝癌十例

Budd-Chiari syndrome associated with hepatoceilular carcinoma： a report of 10 cases

目的探讨布加综合征合并肝癌病人的临床特征、预后及治疗情况。方法对10例布加综合征合并肝癌病人的临床资料进行回顾性分析。彩色多普勒超声探查或（和）下腔静脉造影和肝静脉造影确诊布加综合征：Ⅰa型4例，Ⅱ型3例，Ⅲa型3例。肝细胞癌由彩色多普勒超声、增强螺旋CT和病理检测诊断；肝癌单发8例，多发2例。8例均成功开腹进行肝癌切除术，3例并行肠系膜上静脉-下腔静脉人工血管反C型分流术（肠-腔分流术）；5例开腹手术前2～3d介入行下腔静脉球囊扩张成形术或（和）（副）肝静脉扩张成形术。2例多发肝癌合并布加综合征病人行介入超选择性肝动脉化疗栓塞术＋下腔静脉球囊扩张成形术或（和）（副）肝静脉扩张成形术。结果1例行肠-腔分流术＋肝癌切除术病人术后5d死于肝衰竭；1例行肠-腔分流术病人术后4d出现乳糜漏，治疗15d后好转。随访9例，随访时间为术后半年至5年；2例多发肝癌病人介入术后1年和半年死于肝衰竭；2例病人在术后1年和2年死于肝癌复发转移。结论布加综合征合并肝癌病人预后不良，在解除布加综合征病人肝脏淤血的状态的同时尽早并尽可能的切除肝癌病灶是目前最有效的治疗方法。

Objective To investigate the clinical features, prognosis and treatments of BuddChiari syndrome （B-CS） associated with hepatocellular carcinoma （HCC）. Methods The clinical data of 10 patients with B-CS associated with HCC treated in our hospital were retrospectively analyzed. B-CS was diagnosed by color Doppler and/or inferior veno cavagraphy and percutaneous transhepatic hepatovenography. It was of Type Ⅰ a in 4 patients, Type Ⅱ in 3 and Type Ⅱ a in the left 3. HCC was diagnosed by color Doppler, SCT and pathology detection. HCC in 8 patients was resected successfully and mesocaval shunt performed in 3. The percutaneous transluminal angioplasty （PTA） was conducted in 5 patients 2-3 d before the operation. Two patients underwent PTA＋ TACE （transcatheter arterial chemoembolization）. Results One patient died of hepatic failure on postoperative day 5. One patient with chylorrhea after operation was cured. Nine cases were followed up for 6 months to 5 years. Two patients died of hepatic failure 0.5 and 1 year after the operation, respectively. Two patients died of HCC recurrence 1 and 2 years after the operation. Conclusion The patients with B-CS associated with HCC have worse prognosis. Relieving hepatic congestion of B-CS and resecting the tumor as soon as possible is an effective method for its treatment now.