先天性肝内门体分流25例诊疗分析  

Congenital intrahepatic portosystemic shunt in 25 children:evaluation and treatment strategies

作　　者：张金山[1,2] 李龙 Zhang Jinshan;Li Long(Department of General Surgery,Capital Institute of Pediatrics,Beijing 100020,China;Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment(2021RU015),Chinese Academy of Medical Science,Beijing 100020,China)

机构地区：[1]首都儿科研究所普通(新生儿)外科,北京100020 [2]中国医学科学院小儿外科微创诊疗创新单元,北京100020

出　　处：《中华普通外科杂志》2023年第11期814-818,共5页Chinese Journal of General Surgery

基　　金：国家自然科学基金(82170679);中国医学科学院小儿外科微创诊疗创新单元(2021RU015)。

摘　　要：目的总结先天性肝内门体分流(congenital intrahepatic portosystemic shunt,CIPSS)患儿的治疗策略。方法回顾性分析2017年8月至2022年12月期间于首都儿科研究所就诊的25例CIPSS患儿的临床资料。结果12例接受门体分流结扎术治疗;13例产前超声发现肝内门体分流患儿接受保守治疗。CIPSS的产前超声诊断率为72%(18/25)。高血氨发生率为80%(20/25)。黄疸和肝功能异常发生率分别为77.8%(14/18)和55.6%(10/18)。12例手术患儿术后血氨恢复正常,术后超声和CT检查显示门体分流消失。13例保守治疗患儿,5例超声随访显示门体分流消失;分流血管自发闭合率为38.5%(5/13)。所有保守治疗患儿的黄疸和肝功能异常治愈率为100%,治愈时年龄为1~8个月;高血氨治愈率为90.9%(10/11),治愈时年龄为1~7个月。结论CIPSS是一种可治愈的畸形。生后黄疸、肝功能异常和高血氨多可在1岁以内恢复。CIPSS患儿应保守治疗至1岁,1岁以上仍未闭合者可选择外科手术或介入治疗。Objective To investigate the treatment strategies for congenital intrahepatic portosystemic shunt(CIPSS).Methods The clinical and follow-up data of 25 children with CIPSS in Capital Institute of Pediatrics from Aug 2017 to Dec 2022 were retrospectively analyzed.Results Twelve patients underwent ligation of portosystemic shunt,13 patients with a prenatal diagnosis of CIPSS underwent conservative treatment.The prenatal ultrasound diagnostic rate of CIPSS was 72%(18/25).Hyperammonemia was common in CIPSS children with an incidence of 80%(20/25).Jaundice and abnormal liver function were found in 77.8%(14/18)and 55.6%(10/18)patients,respectively.After surgery,blood ammonia returned to normal.Ultrasound and CT showed that the portosystemic shunt disappeared.Among 5 of 13 children with conservative treatment,the portosystemic shunt disappeared as shown by ultrasound,the spontaneous closure rate was 38.5%(5/13),at the age of 1-7 months old.In all patients with conservative treatment,the cure rate of jaundice and liver dysfunction was 100%and the cure age was 1-8 months old,the cure rate for hyperammoniaemia was 90.9%(10/11)at the age 1-7 months old.Conclusions CIPSS is a curing abnormality.Postnatal jaundice,liver dysfunction,and hyperammonemia can be cured within one year of age.Surgery or interventional threapy should be withheld until 1 year postnatally.

关 键 词：门体分流 肝功能异常 产前超声诊断 诊疗分析 结扎术 超声随访 外科手术 CIPS 

分 类 号：R726.5[医药卫生—儿科]