40例儿童门静脉海绵样变性的临床表现、手术选择及其转归评价  

Pediatric portal vein cavernous transformation:a comprehensive review of clinical manifestations,surgical treatment strategies,and outcomes in 40 cases

作　　者：刘罗海 李仕青[1] LIU Luo-hai;LI Shi-qing(Department of General Surgery,the First Affiliated Hospital of Bengbu Medical College,Anhui 233000,China)

机构地区：[1]蚌埠医学院第一附属医院普外科,安徽233000

出　　处：《肝脏》2024年第1期102-104,109,共4页Chinese Hepatology

基　　金：安徽省高校自然科学研究项目(KJ2021A0800)。

摘　　要：目的分析40例门静脉海绵样变性患儿的临床特征、手术选择及预后转归。方法2017年6月-2022年6月蚌埠医学院第一附属医院收治的40例门静脉海绵样变性患儿,分析其临床表现,总结手术治疗,并评价转归结局。结果儿童门静脉海绵样变性常以腹痛、腹胀、脾脏肿大为临床症状表现,伴随多项肝功能指标改变,影像学检查可见食管静脉曲张。40例门静脉海绵样变性患儿均行手术治疗,其中实施Rex手术28例(70.0%),实施脾肾分流手术8例(20.0%),实施肝移植手术4例(10.0%)。40例门静脉海绵样变性患儿术后均存活,随访结果显示,28例接受Rex手术患儿中,25例(62.5%)搭桥血管通畅,血流速度正常,无血管狭窄,剩余3例(7.5%)术后出现血管闭塞;8例接受脾肾分流手术患儿中,3例(7.5%)术后未见脾脏缩小,食管静脉曲张未缓解,但未发生呕血、黑便;4例接受肝移植手术患儿中,1例(2.5%)术后早期发生肝动脉血栓,经抗凝治疗后再通。结论儿童门静脉海绵样变性的临床表现具有一定特征,临床可根据患儿肝内外门静脉发育情况进行术式选择,其中Rex手术能够恢复门静脉系统正常解剖和生理功能,改善肝脏灌注情况,避免术后不良情形的发生。Objective To analyze the clinical characteristics,surgical intervention strategies,,and prognostic outcomes of 40 pediatric patients diagnosed with portal vein cavernous transformation.Methods Between June 2017 and June 2022,40 pediatric patients diagnosed with portal vein cavernous transformation admitted to our hospital were subjected to a comprehensive analysis of their clinical manifestations,a summary of the surgical treatments administered,and an evaluation of their subsequent outcomes.Results In children,cavernous degeneration of the portal vein typically manifests with clinical symptoms such as abdominal pain,bloating,and splenomegaly,often accompanied by alterations in liver function indicators and esophageal varices as revealed through imaging examination.Forty pediatric cases treated surgically for portal vein cavernous transformation at our institution were retrospectively analysed.The cohort comprised 28 cases(70.0%)undergoing Rex shunt prodedure,8 cases(20.0%)receiving splenorenal shunt surgery,and 4 cases(10.0%)treated with liver transplantation.Postoperatie survival was 100% among the 40 children.Follow-up assessments indicated that of the children who received Rex shunt,25(62.5%)exhibited patent bypass vessels with normal blood flow velocity and no stenosis,while 3(7.5%)experienced postoperative vascular occlusion.In the splenorenal shunt cohort,3(7.5%)did not show a reduction in splenic size or alleviation of esophageal varices,though no cases of hematemesis or melena were reported.Among the liver transplantation recipients,1 child(2.5%)developed early postoperative hepatic artery thrombosis,which was successfully treated with anticoagulant therapy.Conclusion The clinical manifestations of cavernous transformation of the portal vein in pediatric patients exhibit distinct characteristics.Surgical interventions are tailored based on the developmental status of the portal vein both intra-and extrahepatically.Among the availaboe surgical options,the Rex shunt procedure is notable for its ability to

关 键 词：儿童 门静脉海绵样变性 临床表现 手术治疗 转归 

分 类 号：R726.5[医药卫生—儿科]