儿童先天性门静脉腔化不全致门脉系统血栓——附2例病例报告暨文献复习

Children Congenital Cavernous Transformation of Portal Vein-Related Portal Vein System Thrombosis——2 Cases Reports

目的通过2例儿童少见先天性门静脉腔化不全致门脉系统血栓形成的临床特点,进行分析并作文献复习,以提高对本病的认识。方法以北京儿童医院2006年1-6月收治的2例先天性门静脉腔化不全致门脉系统血栓形成的病例资料,进行分析及文献复习。结果2例病人起病年龄早;病前无特殊病史,发病急;影像学提示门静脉显示不全、门静脉海绵样变性伴门脉系统多发性血栓;早期溶栓、抗栓治疗暂时有效,但病情反复,再次溶栓效果不佳;并发症严重,一病人因消化道出血死亡,另一病人合并严重感染。及早门脉分流手术是唯一的治疗方法。结论①先天性门静脉腔化不全是儿童门脉系统血栓的少见原因:②并发症后果严重,不经有效治疗病情迁延、反复甚至危及生命;③早期溶栓治疗有效,晚期无效。④及时门静脉分流手术是唯一的治疗方法。

Objective Reporting 2 cases of congenital cavernous transformation of portal veinrelated thrombosis in portal vein system and reviewing the related literature, to improve the cognizing of this rare disease. Methods 2 case reports from Beijing children＇ s hospital in 2006 and literature review. Results Two pediatric patients were early age, healthy condition before, but acute episode. Radiology showed that portal vein couldnt completely seen followed with in this area the vein sponged-like changing and the thromboses in portal vein system. Good effect of thrombolytic and antithrombotic treatment in early period, but no use at late. The complications were usually severe： one patient died of bleeding, another had sepsis. The shunt operation in time was the only effective solving way. Conclusion ① Congenital Cavernous Transformation of Portal Vein is one of rare causes of portal vein thrombosis in childhood, ② the complications are very severe. ③ Thrombolytic and antithrombotic treatment can have transient remission, but could not gain the real solution. ④ The shunt operation in time is the only effective method.