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儿童先天性肝内门体分流的临床特征及影像学表现

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目的 分析儿童先天性肝内门体分流的临床特征及影像学表现.方法 纳入2017年6月至2022年6月收治的先天性肝内门体分流患儿40例,分析其临床特征及影像学表现.结果 40例患儿中,伴有乙肝肝硬化23例(57.5%).临床分型为Ⅲ型23例(57.5%),分流部位以门静脉右后支-下腔静脉为主17例(42.5%),并伴随ALT、AST、总胆红素升高.CT表现示,病灶位于肝左叶15例,右叶25例;可见分流处肝静脉明显增宽33例(82.5%),未见明显增宽7例(17.5%).超声示,病变均位于肝实质内近肝脏表面或叶段边缘,二维超声呈管型、囊状及不规则无回声;由 CDFI技术检查可见自门静脉分支至肝静脉的红蓝交替的血流信号,无搏动性.结论 儿童先天性肝内门体分流部位以门静脉右后支-下腔静脉为主,多伴有乙肝肝硬化病史,临床分型常见Ⅲ型,并伴随多项肝功能指标改变.
Clinical and imaging profiles of congenital intrahepatic portosystemic shunts in pediatrc patients
Objective To elucidate the clinical characteristics and imaging findings associated with congenital intrahepatic portosystemic shunts in pediatric patients.Methods From June 2017 to June 2022,we examined the clinical and imaging features of 40 pediatric patients diagnosed with congenital intrahepatic portosystemic shunts at our hospital.Results In our cohort of 40 pediatric patients with congenital intrahepatic portosystemic shunts,a significant proportion(23 cases,57.5%)had a history of hepatitis B cirrhosis.The predominant clinical classification was type Ⅲ shunts(23 cases,57.5%),with the shunt site most commonly identified at the right posterior branch of the portal vein inferior vena cava(17 cases,42.5%).These patients exhibited alterations in liver function markers,including elevated alanine aminotransferase(ALT),glutamic oxaloacetic aminotransferase(AST),and total bilirubin(TBil)levels.CT imaging revealed that lesions were predominantly located in the right hepatic lobe(25 cases,62.5%)and the left hepatic lobe(15 cases,37.5%).A majority(33 cases,82.5%)showed significant expansion of hepatic veins at the shunt site,whereas 7 cases(17.5%)did not.Ultrasound findings indicated that lesions were situated near the surface or lobe edge within the parenchyma,appearing as tubular,cystic,or irregular shaped structures without echo on two-dimensional ultrasound.Color Doppler ultrasound demonstrated non-pulsatile,alternating red and blue blood flow signals from the portal vein branch to the hepatic vein.Conclusion In pediatric patients,congenital intrahepatic portosystemic shunts exhibit distinct clinical and radiological profiles,often correlated with a history of hepatitis B cirrhosis.Predominatly classified as type Ⅲ,these shunts frequently occur at the right posterior branch of the portal to the inferior vena cava,eliciting significant alterations in liver function parameters.These diagnostic features and their clinical implications offer valuable reference points for effective management in clinical settings.

ChildrenCongenital intrahepatic portosystemic shuntClinical characteristicsImaging manifestations

马连骏、郝引、李旭雪

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610000 四川成都四川省中西医结合医院放射科

四川省骨科医院放射科

儿童 先天性肝内门体分流 临床特征 影像学表现

四川省科技计划项目

2020YFS0497

2024

肝脏
上海市医学会

肝脏

CSTPCD
影响因子:0.71
ISSN:1008-1704
年,卷(期):2024.29(4)
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