Abstract:

History，current situation and development of the diagnosis and treatment of Budd-Chiari syndrome                    WANG Zhong-gao*，LI Zhen. *Department of Vascular Surgery, Xuanwu Hospital, Capital Medical University，Beijing100053, China
Corresponding author: WANG Zhong-gao, E-mail: zhonggaowang@yeah.net
Abstract    Budd-Chiari syndrome (BCS) is portal hypertension and / or inferior vena cava syndrome due to the obstruction of hepatic vein or over the opening of inferior vena cava because of various reasons. Part patients with atypical symptoms are easily misdiagnosed and can not be timely treated. In recent years，with the progress of the knowledge of the disease and medical imaging，the preliminary diagnosis rate of BCS has been improved. Due to the change of incidence in different area，and uneven level of diagnosis and treatment in the different hospitals，it is far from standardized and individualized treatment，and long-term efficacy still needs to be improved. Endovascular therapy has become the preferred treatment of Budd-Chiari syndrome. In patients with endovascular treatment failure and those should not be interventional treatment due to various reasons，or in patients with multiple recurrences after endovascular treatment，surgery can be performed. Multiple bypass types pioneered by the auther are still effective solutions for complex and critically patients of BCS with not suitable for endovascular therapy.

Key words: Budd-Chiari syndrome, transjugular intrahepatic portosystemic shunt, endovascular therapy, multiple bypass types

摘要：

布加综合征（BCS）是各种原因引起的肝静脉或其开口以上的下腔静脉阻塞导致的淤血性门静脉高压和（或）下腔静脉高压综合表现。病人早期因症状不典型易被误诊而不能得到及时治疗，近年来，随着对该病认识水平的提高与影像医学的进步，其初诊率有明显改善。但因各地发病率不同及不同医院对该病诊治水平参差不齐，目前远未达到规范化及个体化治疗，远期疗效仍亟待提高。血管腔内治疗已成为治疗BCS的首选。对于腔内治疗失败及各种原因不宜行腔内治疗者，或术后多次复发病人可行外科手术治疗。笔者在国际上首创并沿用至今的多种门体转流术式对于复杂、危重且不适合行腔内治疗的BCS病人具有良好的疗效。

关键词: 布加综合征, 门体静脉分流术, 腔内治疗, 门体转流