布加综合征的分期治疗

Stage management of Budd-Chiari syndrome

目的探讨布加综合征分期治疗的指征、可行性和临床效果。方法2007年2月至2009年6月收治布加综合征患者32例，其中Ⅰ型9例，Ⅲa型17例，HIb型6例，所有患者同时存在下腔静脉高压和门静脉高压。根据患者的临床病理分型和血流动力学代偿情况，32例患者分别行介入或手术治疗、一期手术（或介入）加二期手术（或介入）治疗。结果所有患者均安全渡过围手术期，无围手术期死亡。肝功能明显好转，临床症状好转或消失。13例肿大肝脏明显缩小，14例腹水消失。胸腹壁静脉曲张9例消失，下肢水肿均消失。并发症主要有胸腔积液3例，急性心功能不全2例，乳糜漏1例，均经保守治疗痊愈。本组所有患者均获随访，随访时间为4～24个月，平均（12±3）个月，效果良好。随访期间无复发病例。结论布加综合征分期治疗可明显降低患者围手术期的危险性，临床效果良好。其血流动力学代偿情况是进行分期治疗的依据。

Objective To investigate the indication, feasibility and clinical effectiveness of stage management of Budd-Chiari syndrome （B-CS）. Methods From Feb 2007 to June 2009, 32 cases of Budd-Chiari syndrome （9 cases of type Ⅰ , 17 cases of type Ⅲ a, 6 cases of type Ⅲ b） were admitted. Inferior vena cava hypertension （IVCHT） and portal hypertension （PHT） co-existed in all the patients. According to the clinicopathologic classification and hemodynamic compensation, these patients underwent single stage treatment （surgical procedure or radioactive intervention） or two-stage management （one-stage surgical procedure/radioactive intervention plus two-stage surgical procedure/radioactive intervention ）. Results Recovery was achieved in all patients without mortality. The main complications were pleural effusion in 3 cases, acute heart failure in 2 cases and celiac lymphatic leakage in 1 case respectively, which were cured after medical treatment. In 4 months to 2 years follow-up, no recurrent cases were identified and all the patients were in good condition. Conclusions Stage management of Budd-Chiari syndrome can alleviate the perioperative risk and clinical effectiveness can be achieved. The hemodynamic compensation is the basis on which stage management is adopted.