国人家族性Budd-Chiari综合征的影像学特点与介入治疗

Study on imaging feature and interventional treatment of Chinese familial Budd Chiari syndrome

目的 探讨国人家族性Budd Chiari综合征 (Budd Chiarisyndrome ,BCS)的影像学特点与介入治疗的有效性。方法 对 4例家族性BCS(两对姐妹 ,来自A、B两个家族 )行血管造影 ,并对 2例行经皮球囊血管成形术 (percutneoustransluminalangioplasty ,PTA) ,2例行内支架置入术。结果 A家族姐妹、B家族姐妹分别为下腔静脉肝后段节段性闭塞和膜性闭塞。A、B家族姐姐分别行内支架置入术 ,妹妹分别行PTA。 4例BCS行介入治疗后下腔静脉回流通畅。A家族姐妹随访 2年后下腔静脉出现再狭窄 ,姐姐再行内支架治疗 ,妹妹再行PTA ;7个月后姐姐因肝功能衰竭死亡 ,其妹妹至今未见复发。B家族姐妹随访 4年未见复发。结论 (1)国人家族性BCS病变类型多样。 (2 )静脉血栓是国人家族性发病的基本病因。 (3)PTA。

Objective To investigate the imaging feature of Chinese familial BCS, and to evaluate the efficacy of interventional treatment for Chinese familial BCS. Methods Angiography was taken in 4 familial BCS patients(from families A and B), and PTA was attempted in 2 of 4 patients, stents were placed in another 2 patients. Results Sisters in family A and sisters in family B had membranous obstruction of the inferior vena cava(MOVC) and segmental obstruction of the inferior vena cava(SOVC), respectively. PTA was attempted in younger sisters of the two families, stent was implanted in elder sisters of the two families. Patency of inferior vena cava and clinical symptom improvement were noted in 4 patients after interventional treatment. Restenosis was detected by angiography in sisters of family A after follow up 2 years later, and stenting was performed in the elder sister, PTA was performed in the younger sister once again. The elder sister was dead after the second stenting, and the younger sister had no symptom up to now. Sisters in family B had no restenosis after 4 years′ follow up. Conclusion 1.Lesion types of Chinese familial BCS were varied. 2.Vena cava thrombosis is the etiology of Chinese familial BCS .3.PTA, stenting and long time anticoagulation treatment may be effective methods in the treatment of familial BCS.