重症布-加综合征的诊断与治疗

目的：探讨重症布－加综合征的诊断标准与治疗方案。方法：对我院治疗的126例重症布－加综合征病人的临床资料进行回顾性分析。介入治疗10例，均行经皮肝穿肝静脉球囊导管扩张成形和内支架置入；手术治疗102例，其中行肠系膜上静脉－下腔静脉人工血管C型架桥术68例，脾静脉－颈内静脉人工血管架桥术33例，肠系膜上静脉－颈内静脉人工血管架桥术1例；介入加常规手术治疗14例，采用Seldinger技术行下腔静脉球囊导管扩张成形和内支架置入后行肠系膜上静脉－下腔静脉人工血管C型超桥术或改良脾－肺固定术。结果：围手术期死亡6例，随访120例，随访时间6个月－7年，效果优者89例（74．2％），良者31例（25．8％）。结论：提出了重症布－加综合征的诊断标准，不同的病变类型应采取不同的治疗方法。

Diagnosis and management of severe Budd-Chiari syndrome

Aim To investigate the diagnosis standards and treatment of severe Budd Chiari syndrome(BCS).Methods The clinical data of 126 patients with severe BCS from November 1994 to 2001 were retrospectively analyzed. Percutaneous transhepatic recanalization and dilation and/or stent placelment of main hepatic vein in 10 cases; Mesocaval C type shunt with artificial graft was performed in 68 cases, splenojugular shunt in 33 cases, mesojuglar shunt in 1 case; Mesocaval shunt or improved splenopneumopexy after percutaneous intraluminal angioplasty (PTA) and stent placement of inferior vena cava(IVC) in 14 cases. Results Six cases died during perioperation. One hundred and twenty patients were followed up for 6 months to 7 years and found that 89 cases had excellent result and 31 had good result.Conclusion Diagnosis standard of severe BCS is put forward. Proper treatment should be used according to the pathological changes of IVC and main hepatic veins (MHV).