布-加综合征的进展与展望

本文叙述了布-加综合征的演变,自1845年和1899年由Budd和Chiari报道的不同肝静脉阻塞引起的门脉高压,在病因和流行病学方面开始研究。我国、印度、南非等国的病变大多涉及下腔静脉,今后以改名为肝腔静脉阻塞综合征更为恰当。在病变分类的基础上,出现了一系列治疗方法。在急性期,纤溶疗法是指征。慢性病例采用的方法：各种腔内治疗法、间接减压术、断流术、各种促进侧枝循环的手术、直接减压术,包括各型肠系膜上静脉或下腔静脉或水母头与右心房或颈内静脉之间的转流术、根治性矫治术和肝移植术,后者指征要严格。今后至少要把下腔静脉隔膜性的病因查清,要做到预防和早期诊断,要对复杂性病变的介入和手术方法加以探讨。

Evolution and prospects of Budd-Chiari syndrome

This paper reviewed the research progress of Budd-Chiari syndrome,a portal hypertension caused by hepatic venous occlusion.Budd and Chiari reported their findings of the occlusive status of the hepatic veins in 1845 and 1899 respectively.Since then etiologic and epidemiologic investigations on the disease were carried out.Renomination of Budd-Chiari syndrome to the hepatocaval occlusive syndrome is perhaps much more appropriate because the occlusive lesions were mainly located in the inferior vena cava in China,India,and South Africa.According to the classification of lesions,different treatments were developed.In the acute stage,the fibrolysis therapy is indicated.In the chronic stage,it includes various PTA,indirect decompression,peri-gastroesophageal devascularization,promoting collateral procedures,direct decompression,including cavo-or meso-even capitus medusa to right atrium or internal jugular vein shunt and the radical resection is a means for radical cure.However,major surgery and liver transplantation is the last option and the indication must be strict.Further research should focus on the etiology of the membranous obstruction of the inferior vena cava,early diagnosis and early intervention and PTA or surgery in treating the more complex lesions.