儿童先天性门体分流的影像学表现  

Imaging characteristics of congenital portosystemic shunt in children

作　　者：姚琼 谢婵来 龚英 阳朝霞 乔中伟 YAO Qiong;XIE Chan-lai;GONG Ying;YANG Zhao-xia;QIAO Zhong-wei(National Children's Medical Center/Department of Radiology,Children’s Hospital,Fudan University,Shanghai 201102,China)

机构地区：[1]国家儿童医学中心/复旦大学附属儿科医院放射科,上海201102

出　　处：《复旦学报（医学版）》2023年第5期705-709,716,共6页Fudan University Journal of Medical Sciences

摘　　要：目的探讨儿童先天性门体分流(portosystemic shunt,PSS)的影像学特点。方法回顾性收集复旦大学附属儿科医院2008年3月—2022年12月间确诊的先天性PSS患儿资料,分析其临床资料和影像学表现。2名放射科医师独立读片,观察门静脉及其属支的形态、门体分流血管走形及侧枝血管分布。结果共纳入26例先天性PSS患儿。CT血管造影(computed tomography angiography,CTA)发现16例为肝外型PSS(Ⅰb型4例,Ⅱ型12例),回流静脉包括下腔静脉2例、左肾静脉5例、髂内静脉6例、胃冠状静脉3例。回流入髂内静脉/胃冠状静脉患儿均合并直肠静脉丛/胃底静脉丛扩张及上、下消化道出血。肝内型PSS共10例(Ⅰ型5例,Ⅱ型2例,Ⅲ型2例,Ⅳ型1例),门静脉及肝内属支发育正常。2例CTA诊断为肝外Ⅰb型的患儿经数字减影血管造影(digital subtraction angiography,DSA)检查发现肝内发育不良门脉血管,修正诊断为肝外Ⅱ型。2例肝外Ⅱ型患儿同期行磁共振血管造影(magnetic resonance angiography,MRA)检查,检查结果与CTA一致,但肝内门脉细小分支显示较差。结论先天性PSS解剖结构多变,CTA/MRA诊断准确率高。但对于肝外Ⅰ型诊断须谨慎,以DSA结果为金标准。Objective To evaluate the imaging characteristics of congenital portosystemic shunt(PSS)in children.Methods Data of children diagnosed with congenital PSS during Mar 2008 to Dec 2022 in Children’s Hospital of Fudan University were collected.The clinical and imaging findings were retrospectively analyzed.The morphology of the portal vein and its branches,the route of PSS,and the distribution of collateral vessels were observed by two radiologists independently.Results Twenty-six children with congenital PSS were enrolled.CTA showed extrahepatic PSS in 16 cases(Ⅰb=4,Ⅱ=12),draining into inferior vena cava in 2 cases,left renal vein in 5 cases,internal iliac vein in 6 cases,and gastric coronary vein in 3 cases.The patients with shunts into internal iliac vein and gastric coronary vein all presented with upper or lower gastrointestinal bleeding.The remaining 10 cases were intrahepatic PSS(Ⅰ=5,Ⅱ=2,Ⅲ=2 andⅣ=1).The development of portal vein and intrahepatic branches were normal.Two cases of extrahepatic typeⅡdiagnosed by digital subtraction angiography(DSA)were misdiagnosed as extrahepatic typeⅠb,because the CTA did not clearly show the branches of the intrahepatic portal vein.Two children with extrahepatic PSS typeⅡunderwent magnetic resonance angiography(MRA)at the same time,and the findings were consistent with CTA,but the fine branches of the intrahepatic portal vein were poorly displayed.Conclusion The anatomy of congenital PSS is diverse and CTA/MRA is an accurate imaging technique.However,for extrahepatic typeⅠPSS,DSA should be taken as the golden standard.

关 键 词：先天性门体分流 CT血管成像(CTA) 磁共振血管成像(MRA) 数字减影血管造影(DSA) 儿童 

分 类 号：R725.4[医药卫生—儿科] R816[医药卫生—临床医学]