Inchingolo Riccardo, Posa Alessandro, Mariappan Martin, Tibana Tiago Kojun, Nunes Thiago Franchi, Spiliopoulos Stavros, Brountzos Elias
Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy.
Department of Radiology, Gemelli Hospital, Roma 00135, Italy.
World J Gastroenterol. 2020 Sep 14;26(34):5060-5073. doi: 10.3748/wjg.v26.i34.5060.
Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.
布加综合征（BCS）是一种相对罕见的临床病症，症状表现多样，由肝静脉流出道梗阻引起。若不治疗，死亡率很高。其治疗基于逐步治疗方法，取决于临床表现，包括从抗凝治疗到介入放射学技术（如经颈静脉肝内门体分流术（TIPS））等不同治疗手段。如今TIPS被认为是一种安全且高效的治疗方法，应推荐给BCS患者，包括那些等待原位肝移植的患者。本文综述了BCS的病理生理学、诊断和治疗选择，特别关注了有关TIPS治疗BCS的技术和结果的已发表数据。此外，还将讨论未解决的问题和未来研究方向。
