先天性肝内型门–体静脉分流产前及产后表现一例

doi:10.3877/cma.j.issn.1672-6448.2024.06.016

孕妇，29岁，孕3产0，2022年自然受孕。孕妇否认糖尿病、高血压、免疫系统疾病、遗传疾病等，唐氏筛查低风险。孕早期检查未见明显异常。于28⁺⁵周行胎儿超声检查（图1）：肝右叶见范围约2.0 cm×1.8 cm的簇状扩张血管团，追踪右门静脉、左门静脉中支^[2]分别与肝中静脉相通；于肝左叶见范围约1.7 cm×1.4 cm的簇状血管团，追踪左门静脉上支、下支^[2]分别与肝左静脉相通。脉冲多普勒提示肝中静脉阻力明显低于肝左、肝右静脉，左门静脉、右门静脉频谱呈三相波。左心室内局部腱索回声增强。于脐带根部旁胎盘内见大小约2.4 cm×1.6 cm的不均质低回声包块，可见血流信号。超声诊断：（1）肝内异常血管团：考虑门-体静脉分流（肝内型Park Ⅳ型）；（2）胎盘内低回声：考虑胎盘血管瘤；（3）左心室强回声斑。孕妇拒绝羊水穿刺检查。于孕29⁺⁵、30⁺⁴、38周行胎儿超声检查，受累交通支血流信号更加丰富，其余未见显著变化。孕妇于40周分娩一男婴，面、颈、胸见多处皮肤血管瘤，娩出的胎盘见血管瘤。

图1 先天性肝内型门-体静脉分流胎儿期超声图像。图a示胎儿期肝右叶异常回声团二维图像；图b示肝内彩色多普勒图像，可显示血管异常走行：右门静脉、左门静脉中支-肝中静脉分流（黄色箭头），左门静脉上支、下支-肝左静脉分流（红色箭头）；图c，d示胎儿肝右叶二维及彩色多普勒图像，显示右门静脉、左门静脉中支与肝中静脉相交通（黄色箭头）；图e，f示胎儿肝左叶二维及彩色多普勒图像，显示左门静脉上支、下支与肝左静脉相交通（红色箭头）；图g，h为时间-空间相关成像（STIC）图像显示右门静脉及左门静脉中支-肝中静脉分流（黄色箭头），左门静脉上支及下支-肝左静脉分流（红色箭头）；图i ~ k示肝右、肝中、肝左静脉频谱，显示肝中静脉阻力明显低于肝左、肝右静脉；图l示静脉导管频谱形态正常，图m，n示左门静脉、右门静脉频谱形态异常；图o，p示胎盘内血管瘤显示为低回声，彩色多普勒显示其内可见血流信号注：IVC为下腔静脉；RHV为肝右静脉；MHV为肝中静脉；LHV为肝左静脉；UV为脐静脉；PV为门静脉；LPV为左门静脉；ARPV为右门静脉前支；LPVs为左门静脉上支；LPVm为左门静脉中支；LPVi为左门静脉下支

图2 生后第3天患儿皮肤外观、超声检查图像及生后第6天CT检查图像。图a示生后第3天患儿体表多发皮肤血管瘤；图b为生后第6天CT三维重建图像，可见肝右叶扩张的血管团（黄色箭头），肝中静脉与右门静脉、左门静脉分支相通，另见肝左叶扩张的血管团（红色箭头），肝左静脉与左门静脉分支相通；图c，d为生后第3天超声检查图像，图c示肝右叶瘤样扩张的血管团血流多普勒；图d示肝左叶两处扩张血管回声（箭头所示）注：IVC为下腔静脉；RHV为肝右静脉；MHV为肝中静脉；LHV为肝左静脉；UV为脐静脉；PV为门静脉；ARPV为右门静脉前支；LPVs为左门静脉上支；LPVm为左门静脉中支；LPVi为左门静脉下支；SMV为肠系膜上静脉

图3 生后10月龄患儿皮肤外观及超声检查图像。图a示皮肤血管瘤经治疗后明显好转；图b示肝左叶异常扩张血管团消失，相应区域肝实质回声增强；图c示肝右叶异常扩张血管团消失，相应区域肝实质回声增强；图d为彩色多普勒图像示肝右叶血管走行自然，未见明显扩张血管

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Prenatal and postnatal manifestations of congenital intrahepatic portal-systemic venous shunt: a case report

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Prenatal and postnatal manifestations of congenital intrahepatic portal-systemic venous shunt: a case report