胎儿静脉导管先天缺如的产前超声表现及预后随访分析

Prenatal ultrasound characteristics of fetal congenital absence of the ductus venosus and follow- up analysis of the prognosis

目的探讨胎儿先天性静脉导管缺如的产前超声表现及其对胎儿预后的影响。方法回顾性分析2013年12月至2017年6月11例胎儿静脉导管缺如超声声像图特点，并追踪胎儿预后情况。结果11例静脉导管缺如中7例为脐静脉直接回流到右心房；1例为脐静脉直接连于下腔静脉；1例为脐静脉直接汇入髂静脉；2例为脐静脉与门静脉相连接。11例中孤立性静脉导管缺如4例，出生后暂未见异常；同时合并胎儿心内、心外系统结构异常5例，其中1例为双绒毛膜双羊膜双胎妊娠之一胎儿（之二胎儿未见明显异常），自然分娩后死亡，另4例均于孕28周前终止妊娠；仅合并胎儿心外系统异常2例，其中1例合并严重消化道异常，分娩后死亡；另1例合并单脐动脉及羊水多，出生后暂未见异常。结论彩色多普勒超声检查是产前筛查胎儿静脉导管缺如的重要手段。孤立性静脉导管缺如未合并胎儿心功能异常者预后良好；静脉导管缺如若合并严重心内、心外畸形者预后不良。静脉导管缺如与胎儿染色体异常有相关性，如需继续妊娠，应建议先进行胎儿染色体检查，并利用超声密切随访胎儿心功能。

ObjectiveTo investigate the sonographic findings of fetal congenital absence of the ductus venosus （ADV） and its effect on the prognosis of fetus.MethodsThe ultrasonographic features of 11cases of fetal ADV deficiency diagnosed from December 2013 to June 2017 were retrospectively analyzed, and the fetal prognosis was followed up.ResultsOf the 11 cases, umbilical vein flew directly back to the right atrium in 7 cases; umbilical vein connected with the inferior vena cava in 1 case; umbilical vein directly flowed into the iliac vein in 1 case; umbilical vein and portal vein connection in the other 2 cases. Of the 11 cases, 4 cases were solitary and had no abnormality after birth; 5 cases were complicated with abnormal structure of intracardiac and extracardiac system; 2 cases were only followed with abnormal extracardiac system. Of the 5 complicated cases, 1 case was double chorionic twin amniotic membrane pregnancy （the second fetuse had no obvious abnormality） and died after birth; the other 4 cases were terminated before 28 weeks of gestation. Of the 2 complicated cases, 1 case was followed with severe digestive tract abnormalities and died after birth, and the other one was followed with single umbilical artery and hydramnion.ConclusionsColor Doppler ultrasonography is an important means for prenatal screening of fetal ADV.The prognosis of patients with solitary ADV without fetal cardiac dysfunction is good.The prognosis is poor to patients with severe intracardiac and extracardiac abnormality. There is a correlation between the ADV and fetal chromosomal abnormalities. If pregnancy is necessary, fetal chromosome examination should be recommended first and cardiac function should be followed up closely by ultrasound.