Rex-bypass shunt联合贲门周围血管离断治疗小儿肝前性门静脉高压

Efficacies of Rex-bypass shunt plus paraesophagogastric devascularization for prehepatic portal hypertension due to cavernomatous transformation of portal vein in children

目的评估Rex-bypass shunt联合贲门周围血管离断术治疗小儿门静脉海绵样变性引起的肝前性门静脉高压症的疗效。方法回顾性分析2008年8月至2013年12月行Rex-bypass shunt联合贲门周围血管离断术的21例门静脉海绵样变性的患儿临床资料。男13例，女8例，年龄3449岁，按照年龄分为A、B、C三组，术前完善门静脉系统彩色多普勒超声、CT门静脉造影（CTV）及其三维成像、肝功能等检查，留作术后随访对照，手术方式为Rex-bypass shunt搭桥术或转流术联合贲门周围血管离断术，存在脾大患儿同时行脾脏切除术或脾脏次全切除术（〈5岁患儿）。结果无术中死亡病例，术后随访6～64个月，16例于搭桥或转流术后后1周内行B型超声检查示血管通畅，1例血管通而不畅，5例术后近期血栓形成，2例分别于3个月和6个月后检查复通，3例不通。17例胃镜检查食管胃底静脉曲张均已明显减轻，其中6例消失，8例仍有轻度静脉曲张，3例仍有中度静脉曲张。Fisher’s Exact检验及配对秩和检验处理，术后肝功能可明显改善（P=0．042），早期手术优于延迟手术（四格表Pearson Chi-Square检验，P=0．001）。结论Rex-bypass shunt联合贲门周围血管离断术是安全有效的，可成为治疗小儿门静脉海绵样变性引起肝前性门静脉高压症的首选术式。

Objective To evaluate the efficacies of Rex-bypass shunt plus paraesophagogastric devascularization for prehep~/tic portal hypertension （PHT） due to cavernomatous transformation of portal vein （CTPV） in children. Methods The clinical data of 21 children with prehepatic vascular hypertension undergoing Rex-bypass shunt plus paraesophagogastric devascularization were retrospectively analyzed. There were 13 males and 8 females with an age range of 3-49 years. According to age, they were divided into 3 groups after color Doppler ultrasound of portal vein system. Computed tomographic portal vein angiography （CTV）, three dimensional imaging and liver function test were performed preoperatively. And the procedures were Rex-bypass shunt plus paraesophagogastric devascularization, splenectomy or subtotal splenectomy. Results There was no instance of intraoperative mortality. The follow-up period was 6-64 months. There were vascular bypass with blood flow （n = 16）, partial obstruction （n = 1）, thrombosis （n = 5） and recanalization （n =2）. Among 17 cases with esophageal and gastric varices, there were significant improvements, including complete remission （n = 6）, mild varicosity （n = 8） and moderate varicosity （n = 3）. The results of Fisher＇s exact test and paired rank indicated improved hepatic function （P = 0. 042）. Four-table Pearson＇s Chi-square test indicated better efficacies of early over delayed operation. Conclusions Rex-bypass shunt plus pericardial devascularization is both safe and efficacious for prehepatic portal hypertension due to CTPV in children.