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中华医学超声杂志(电子版) ›› 2024, Vol. 21 ›› Issue (03) : 257 -267. doi: 10.3877/cma.j.issn.1672-6448.2024.03.003

妇产科超声影像学

胎儿脐-门-体静脉系统异常分流的产前超声诊断及产后结局
朱惠娟1, 邝海燕1,(), 骆迎春1, 邓光祁2, 蒋凌晖3, 汪圣4, 王政5, 孔一凡1, 龙煜1   
  1. 1. 410008 长沙,湖南省妇幼保健院(国家卫生健康委员会出生缺陷研究与预防重点实验室)超声科
    2. 410013 长沙,湖南师范大学医学院
    3. 410200 长沙,湖南省长沙市望城区妇幼保健院超声科
    4. 410000 长沙,湖南省儿童医院放射科
    5. 413000 益阳,湖南省益阳市南县第一人民医院超声科
  • 收稿日期:2023-05-22 出版日期:2024-03-01
  • 通信作者: 邝海燕
  • 基金资助:
    湖南省自然科学基金项目(2019JJ50296); 湖南省卫生健康委卫生科科研课题(W20243133); 湖南省出生缺陷协同防治科技重大专项(2019SK1010); 湖南省卫生计生委科研计划课题项目(C2017054); 湖南省卫生计生委科研计划课题项目(202209023037)

Prenatal ultrasound diagnosis and postpartum outcomes of abnormal umbilical-portal-systemic venous shunt

Huijuan Zhu1, Haiyan Kuang1,(), Yingchun Luo1, Guangqi Deng2, Linghui Jiang3, Sheng Wang4, Zheng Wang5, Yifan Kong1, Yu Long1   

  1. 1. Department of Ultrasound, Hunan Provincial Maternal and Child Health Hospital (Key Laboratory of Birth Defects Research and Prevention, National Health Commission), Changsha 410008, China
    2. College of Medicine, Hunan Normal University, Changsha 410013, China
    3. Department of Ultrasound, Wangcheng District Maternal and Child Health Hospital, Changsha 410200, Hunan Province, China
    4. Department of Radiology, Hunan Children's Hospital, Changsha 410000, China
    5. Department of Ultrasound, the First People's Hospital of Nanxian, Yiyang City, Hunan Province, Yiyang 413000, China
  • Received:2023-05-22 Published:2024-03-01
  • Corresponding author: Haiyan Kuang
引用本文:

朱惠娟, 邝海燕, 骆迎春, 邓光祁, 蒋凌晖, 汪圣, 王政, 孔一凡, 龙煜. 胎儿脐-门-体静脉系统异常分流的产前超声诊断及产后结局[J/OL]. 中华医学超声杂志(电子版), 2024, 21(03): 257-267.

Huijuan Zhu, Haiyan Kuang, Yingchun Luo, Guangqi Deng, Linghui Jiang, Sheng Wang, Zheng Wang, Yifan Kong, Yu Long. Prenatal ultrasound diagnosis and postpartum outcomes of abnormal umbilical-portal-systemic venous shunt[J/OL]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2024, 21(03): 257-267.

目的

探讨胎儿脐-门-体静脉系统异常分流(UPSVS)五大类型的产前超声图像特征及其预后,评价产前超声诊断UPSVS的临床价值。

方法

对2017年1月至2021年1月在湖南省妇幼保健院至少2次以上产前超声检查诊断为UPSVS的病例,共52例进行回顾性分析,应用二维超声联合时间-空间关联成像技术(STIC)将UPSVS分为五类:(1)脐静脉-体静脉分流(USS);(2)静脉导管-体静脉分流(DVSS);(3)门静脉-体静脉分流(PSS),此类型又分为肝内型(IHPSS)和肝外型(EHPSS);(4)先天性肝动脉-门静脉瘘(CHPAVF);(5)孤立性静脉导管缺如或闭锁。分别探讨五大类型UPSVS的产前超声图像特点、主要临床特征及产后结局。

结果

五种类型的UPSVS病例均有各自独特的超声影像学特征;UPSVS各类型产后结局具有差异性,主要表现在活产率、手术干预率、分流血管闭合率3个方面。DVSS、IHPSS、孤立性静脉导管缺如或闭锁组活产率较高(100%、85.0%、83.3%),USS活产率最低(0%);EHPSS与CHPAVF手术干预率最高(均为50.0%);其中DVSS与IHPSS分流血管闭合率较高,CHPAVF分流血管也有自发性闭合的可能。

结论

各类型UPSVS具有特征性的超声图像特征,产前二维超声联合STIC技术可以对UPSVS进行产前诊断及产前分型;UPSVS各类型产后结局具有差异性,产前准确诊断和动态监测对产前咨询及产后治疗具有重要价值。

Objective

To explore the prenatal ultrasound imaging features and prognosis of five types of umbilical-portal-systemic venous shunt (UPSVS) and evaluate the clinical value of prenatal ultrasound diagnosis of UPSVS.

Methods

A retrospective analysis was conducted on 52 cases of UPSVS that was diagnosed by repeat antenatal ultrasonography at Hunan Maternal and Child Health Hospital from January 2017 to January 2021. Two-dimensional ultrasound combined with spatiotemporal image correlation (STIC) technology was used to classify UPSVS into five types: (1) umbilical-systemic shunt; (2) ductus venosus-systemic shunt; (3) portal-systemic shunt; (4) congenital hepatoportal arteriovenous fistula; and (5) isolated absence or closure of the ductus venosus. The prenatal ultrasound imaging characteristics, main clinical features, and postpartum outcomes of each type of UPSVS were investigated.

Results

Each type of UPSVS had its unique ultrasound imaging features. The postpartum outcomes of UPSVS varied among different types, mainly in terms of delivery rate, surgical intervention rate, and shunt vessel closure rate. The types with higher delivery rates were ductus venosus-systemic shunt, intrahepatic portal-systemic shunt, and isolated absence or closure of the ductus venosus (100%, 85.0%, and 83.3% respectively), while the type with the lowest delivery rate was portal-systemic shunt (0%). The types with the highest surgical intervention rate were extrahepatic portal-systemic shunt and congenital hepatoportal arteriovenous fistula (both 50.0%). The ductus venosus-systemic shunt and intrahepatic portal-systemic shunt types had higher shunt vessel closure rates, and spontaneous closure of shunt vessels was also possible in the congenital hepatoportal arteriovenous group.

Conclusion

Each type of UPSVS has characteristic ultrasound imaging features. Prenatal two-dimensional ultrasound combined with STIC technology can be used for prenatal diagnosis and classification of UPSVS. Prenatal accurate diagnosis and dynamic monitoring have important value for prenatal counseling and postpartum treatment of UPSVS.

图1 病例1超声声像图表现。图a为脐静脉入腹腔后的彩色多普勒成像图;图b为下腔静脉增宽二维图;图c为肝内门静脉系统时间-空间关联成像技术图;图d为心脏四腔心切面二维图;图e为肝内门静脉系统能量多普勒成像图;图f为肝外门静脉系统能量多普勒成像图 注:UV为脐静脉,BL为膀胱,IVC为下腔静脉,SP为脊柱,AO为腹主动脉,SWV为肠系膜下静脉,SPV为脾静脉,PV为门静脉主干
图2 病例2超声声像图。图a为脐静脉入腹腔后的彩色多普勒成像图;图b为心脏四腔心切面二维图 图3 病例3超声声像图。图a为静脉导管汇入冠状静脉窦二维图;图b为静脉导管多普勒频谱图 注:UV为脐静脉,LIVER为肝,SP为脊柱;DV为静脉导管,CS为冠状静脉窦,RA为右心房,LA为左心房
图4 病例4超声声像图。图a:静脉导管、左上腔静脉均汇入冠状静脉窦,冠状静脉窦增宽;图b:时间-空间关联成像技术图;图c:静脉导管、脐静脉二维图 注:DV为静脉导管,CS为冠状静脉窦,RA为右心房,LA为左心房,LSVC为永存左上腔静脉,IVC为下腔静脉,DAO为降主动脉,UV为脐静脉
图5 病例5超声声像图。图a:静脉导管汇入下腔静脉右房口处远端二维图像;图b:静脉导管汇入下腔静脉右房口处远端彩色多普勒成像图;图c:静脉导管频谱图像 图6 病例6超声声像图。图a:静脉导管汇入肝左静脉能量多普勒图像;图b:静脉导管频谱图像 注:DV为静脉导管,RA为右心房,IVC为下腔静脉,HV为肝静脉,UV为脐静脉,LHV为肝左静脉,AO为腹主动脉
图7 病例7超声声像图。图a:产前高分辨率血流成像显示异常血管走行(箭头所示);图b:产前时间-空间关联成像技术显示异常血管走行(箭头所示);图c:心脏四腔心切面二维图;图d产后彩色多普勒成像显示异常血管走行(箭头所示);图e产后CT血管成像显示异常血管走行(箭头所示)。门静脉主干入肝后(在发出左、右门静脉之前)发出一异常血管在左肝内(朝向胎儿左侧)走行一段距离后,向后出肝,再折返,向后、向下走行,最后汇入左肾静脉 注:ST为胃泡,PV为门静脉,DAO为主动脉,IVC为下腔静脉,UV为脐静脉,LKV为左肾静脉,SV为脾静脉,LIVER为肝脏
图8 病例8孕妇33岁,孕26+3周,超声声像图示肝内型门静脉-体静脉分流。图a产前高分辨率血流成像显示门静脉右支与肝右静脉末端吻合(吻合处箭头所示);图b产前时间-空间关联成像技术显示吻合处(箭头所示) 注:RHV为肝右静脉,RPV为门静脉右支
图9 病例9超声声像图。图a为产前彩色多普勒成像图;图b为产前高分辨率血流成像注:LPV为门静脉左支,LHA为左肝动脉技术图;图c为产前时间-空间关联成像技术图;图d为产前肝内动脉频谱图,收缩期峰值流速(PSV)为43.95 cm/s,阻力指数(RI)为0.45;图e为产前肝右静脉频谱图;图f为产前四腔心切面二维图;图g 为出生后CT血管成像三维图 注:M为混合回声区,MHV为肝中静脉,RHV为肝右静脉,RPV为门静脉右支,HA为肝动脉,DAO为主动脉,UV为脐静脉,LPV为门静脉左支,LHV为左肝动脉,AO为腹主动脉
图10 病例10超声声像图。图a:产后左肝动脉与门静脉左支瘘二维超声图;图b:产后左肝动脉与门静脉左支瘘彩色多普勒成像图;图c:产后肝门处混合回声区彩色多普勒超声成像图;图d:左肝动脉与门静脉左支汇合处频谱多普勒超声成像,左肝动脉与门静脉左支汇合口处收缩期峰值流速约121 cm/s,阻力指数为0.37 注:LPV为门静脉左支,LHA为左肝动脉
图11 孤立性静脉导管闭锁病例超声声像图。图a为静脉导管(DV)闭锁留下的痕迹。图b为DV所在位置未探及能量多普勒血流信号
表1 胎儿脐-门-体静脉异常分流合并其他结构畸形汇总
表2 各类脐-门-体静脉异常分流的产后结局汇总[%(例/例)]
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