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目的 探讨Budd Chiari综合征 (BCS)的病变特点及合理分型。方法 对来自 5个BCS高发地区并按统一方案确诊的 4 12例BCS患者的血管造影等影像学检查结果及临床资料进行统计学分析。结果 将BCS分为单纯肝静脉病变 (Ⅰ型 )、下腔静脉 (IVC)膜性病变 (Ⅱ型 )、IVC膜性病变伴远端腔内血栓形成 (Ⅲ型 )和IVC节段性病变 (Ⅳ型 ) 4种类型 ,并将Ⅰ型分为局限型和弥漫型 ,将Ⅱ～Ⅳ型各分为伴有和不伴有 1支以上肝静脉开放的 8种亚型。根据该方案分型 :Ⅰ型 4 9例 ,占11 9% ;Ⅱ型 2 0 8例 ,占 5 0 5 % ;Ⅲ型 19例 ,占 4 6 % ;Ⅳ型 136例 ,占 33 0 %。 4组患者间平均发病年龄无显著性意义 (F =1 6 3,P >0 0 5 )。但平均病程有明显不同 :I型短于其他 3型 (P <0 0 5 ) ;Ⅳ型长于Ⅱ型 (q =4 33,P <0 0 5 )和I型 (q =6 36 ,P <0 0 5 ) ;而Ⅱa与Ⅱb及Ⅳa与Ⅳb型之间的平均病程比较无显著性意义 (P >0 0 5 )。结论 (1)伴有 1支或 1支以上肝静脉开放的IVC膜性阻塞性病变 (Ⅱa型 )是该组BCS的主要类型。 (2 )BCS血管病变的各种类型考虑是一由狭窄到闭塞、由膜性向节段性病变逐渐发展演化的过程 ,尤其是Ⅱ型、Ⅲ型和Ⅳ型病变是整个疾病进展过程中具有因果关系的不同阶段。 (3)再分型方案对BCS治疗方法的选择和预
Objective To investigate the pathological features and reasonable typing of Budd Chiari syndrome (BCS). Methods The angiographic findings and clinical data of 4 12 BCS patients diagnosed from 5 BCS high incidence areas and according to the unified protocol were statistically analyzed. Results BCS were classified into simple hepatic vein lesions (IV type), IVC membranous lesions (IV type), IVC membranous lesions with distal intraluminal thrombosis (III type) and IVC segmental lesions Ⅳ type), and classified type Ⅰ into diffuse type and diffuse type. Type Ⅱ to type Ⅳ were divided into 8 subtypes with and without hepatic veins with 1 or more open. According to the classification of the program: type I 49 cases, accounting for 11 9%; Type II 208 cases, accounting for 50%; Type 19 19 cases, accounting for 46%; Type IV 136 cases, accounting for 33 0%. There was no significant difference in mean age of onset between the 4 groups (F = 163; P> 0.05). But the average course of disease was significantly different: type I was shorter than other type 3 (P <0 05); type IV was longer than type II (q = 433, P <0 05) and type I (q = 6 36, P < 0 0 5). There was no significant difference in the average course of disease between Ⅱa and Ⅱb, Ⅳa and Ⅳb (P> 0.05). Conclusion (1) IVC membranous obstructive lesions (type IIa) with one or more hepatic veins open are the main types of BCS in this group. (2) Various types of BCS vascular lesions are considered to be a process of gradual progression from membranous to segmental lesions, especially from type Ⅱ to type Ⅲ, and type Ⅳ. It is the cause and effect of progression of the entire disease Different stages of the relationship. (3) re-classification of BCS treatment options and pre-treatment options