Peng Yuming,Yin Qiang,Yuan Miaoxian,et al.Treatment strategy of portal vein cavernous degeneration shunt and liver transplantation in children[J].Journal of Clinical Pediatric Surgery,2022,21(05):431-436.[doi:10.3760/cma.j.cn101785-202203043-007]
儿童门静脉海绵样变性的临床特点及手术治疗策略
- Title:
- Treatment strategy of portal vein cavernous degeneration shunt and liver transplantation in children
- Keywords:
- Portal Vein/PP; Cavernous Transformation of Portal Vein; Splenorenal Shunt; Surgical; Por- tasystemic Shunt; Surgical; Liver Transplantation; Surgical Procedures; Operative/MT; Treatment Outcome
- 摘要:
- 目的 探讨儿童门静脉海绵样变性的临床特点及手术治疗策略。方法 回顾性分析湖南省儿童医院2010年5月至2022年1月收治的27例儿童门静脉海绵样变性患儿临床资料,其中男12例,女15例;年龄1岁7个月至11岁11个月。患儿均经腹部超声及CT检查确诊为儿童门静脉海绵样变性,并经数字减影血管造影(digital subtraction angiography,DSA)评估肝内外门静脉通畅情况,根据病变情况选取手术方式。结果 27例患儿均实施手术。其中Rex手术19例,行Rex手术的患儿术后门静脉系统压力均明显下降;其中2例利用粗大肝外侧支静脉与门静脉矢状部行侧侧吻合,肝内门静脉获得满意灌注量;1例行巨脾切除后利用脾静脉行经典Rex手术;2例搭桥血管血栓形成,经抗凝治疗后失败,血管闭塞。3例因严重门静脉海绵样变性和反复消化道出血实施肝移植手术,其中1例早期发生肝动脉血栓,经抗凝治疗后再通。3例实施远端脾肾分流手术,分流血管通畅。2例实施近端脾肾分流手术,其中1例出现脾静脉附壁血栓,经抗凝治疗后好转。结论 儿童门静脉海绵样变性常常以门脉高压相关症状为临床特点,手术方式应根据肝内外门静脉发育和代偿情况综合分析后进行选择,搭桥血管可以根据患儿自身血管特点进行选择。首选Rex手术的原因在于其能够恢复门静脉系统正常解剖通道和生理作用,改善肝脏灌注和生长发育,避免肝性脑病的发生。
- Abstract:
- Objective To explore the clinical characteristics and surgical treatment of spongiosis of por- tal vein in children at a single center. Methods From May 2010 to January 2022, clinical data were retrospec- tively reviewed for 27 children with portal cavernous degeneration. There were 12 boys and 15 girls with an age range of (19-143) months. Form of informed consent was signed. Cavernous portal vein degeneration was di- agnosed by abdominal ultrasound and computed tomography (CT). Digital subtraction angiography (DSA) was employed for further evaluating the patency of portal vein in and outside liver. Finally surgical approach was se- lected according to the pathological findings. Results All of them underwent surgery, including 19 cases of Rex operation,19 cases of high preoperative portal vein pressure, pressure declined markedly after Rex bypass. In 2 cases, large lateral hepatic branch vein was anastomosed with sagittal part of portal vein. And intrahepatic portal vein was perfused with satisfactory amount. In 1 child, splenic vein was used for classical Rex surgery af- ter splenic splenectomy. In 2 cases, thrombosis was formed in bypass vessels. Despite anticoagulant therapy, vas- cular occlusion occurred. Three children underwent liver transplantation due to severe portal spongiform degen- eration and recurrent gastrointestinal bleeding. One child of early hepatic artery thrombosis became recanalized after anticoagulant therapy. Distal splenorenal shunt was performed in 3 cases and shunt vessels stayed unobstr- ucted. In two children of proximal splenorenal shunt, one case developed mural thrombosis of splenic vein and improved after anticoagulant therapy. Conclusion Children’s portal cavernous degeneration is often character- ized by symptoms related to portal hypertension. The operation method should be selected according to the com- prehensive analysis of the development and compensation of the portal vein inside and outside the liver. The by- pass vessel can be selected according to the characteristics of the children’s own blood vessels. The reason for the first choice of Rex surgery is that it can restore the normal anatomical channel and physiological function of the portal vein system, improve liver perfusion and growth and development, and avoid the occurrence of hepatic encephalopathy.
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备注/Memo
收稿日期:2022-3-13。
基金项目:湖南省卫生健康委员会技术创新项目（编号:湘卫医政医管处便函[2018]187号）,湖南省儿童医院国际化人才项目
通讯作者:尹强,Email:qiangyin@hotmail.com