儿童先天性门体分流的诊治

Diagnosis and treatment of congenital portosystemic shunt in children

目的总结儿童先天性门体分流的诊治经验。方法收集2014年1月至2021年4月收治于上海交通大学医学院附属上海儿童医学中心普外科及心内科的20例先天性门体分流患儿临床资料,其中男15例,女5例。肝外门体分流9例,肝内门体分流9例,肝外合并肝内门体分流2例。4例患儿合并先天性心脏病,2例合并肝脏良性肿瘤。治疗方法采用血管内介入、手术结扎、介入与手术结合以及肝移植。分析患儿围手术期及术后随访的临床数据。结果介入封堵分流以及手术结扎共14例,肝移植手术6例。9例肝外门体分流中,门腔分流6例,门-左肾静脉分流2例,肠系膜下静脉与髂内静脉分流1例。其中,6例一次手术结扎,2例延迟分次结扎,1例肝移植。9例肝内门体分流中,单纯封堵3例,手术结扎2例,手术加封堵1例,肝移植3例。2例肝外合并肝内门体分流均行肝移植。所有患儿术前症状均得到改善,1例肝移植术后出现脑出血。随访2~82个月,没有死亡病例,患儿生长发育较好。结论儿童先天性门体分流应按个体化方案进行治疗,血管介入治疗为首选方式,手术结扎也是重要手段,特殊病例最终需要肝移植。

Objective To explore the experience of diagnosing and treating congenital portosystemic shunt(CPS)in children.Methods Retrospective review was conducted for clinical data of 20 CPS children from January 2014 to April 2021.There were 15 boys and 5 girls.There were extrahepatic portosystemic shunt(n=9),intrahepatic portosystemic shunt(n=9),complex type(n=1)and mixed type(n=1).Four cases were complicated with congenital heart disease and 2 cases with benign liver tumor.Intravascular intervention,surgical ligation,combination of intervention&operation and liver transplantation were applied.Results There were shunt occlusion by surgical ligation or vascular intervention(n=14)and liver transplantation(n=6).Among 9 cases of extrahepatic portosystemic shunt,there were portal cavity shunt(n=6),portal left renal vein shunt(n=2)and inferior mesenteric vein&internal iliac vein shunt(n=1).One-time surgical ligation(n=6),delayed fractional ligation(n=2)and liver transplantation(n=1)were performed.There were simple blockage(n=3),surgical ligation(n=2),surgical sealing&blockage(n=1)and liver transplantation(LT,n=3).LT was performed in 2 case of intra/extrahepatic portosystemic shunt.All preoperative symptoms improved.There was no mortality during a follow-up period of(2-82)months.Both growth and development were decent.Conclusions CPS children should be managed according to individualized schemes.Intravascular interventional therapy is a primary choice.Surgical ligation is also vital.Ultimately LT is required for special cases.