胎儿脐-门-体静脉异常分流的产前超声诊断

目的探讨胎儿脐-门-体静脉异常分流(UPSVS)的产前超声图像特征及其临床预后。方法总结了8例UPSVS的超声图像特征及预后,并行追踪随访。结果 8例UPSVS胎儿中,USS型2例,其中1例脐静脉经冠状静脉窦引流入右心房,生后出现膈疝,经手术治疗,目前6个月健康;另1例下腔静脉肝段缺如,脐静脉肝外异常连接于扩张的奇静脉,引产;DVSS型1例,静脉导管直接与右心房相连,合并小脑蚓部发育不良,引产;3例单纯静脉导管缺失,其中1例足月出生,健康,另2例因合并心内畸形,引产;IHPSS型2例,其中1例门静脉右支与肝右静脉相连,失访,另1例肝左静脉与左门静脉沟通,出生1个月未见异常。结论产前超声检查可诊断UPSVS,影响预后的关键因素为是否合并畸形及是否存在完整的IHPVS,产前精确诊断对预后咨询及产后治疗具有重要价值。     

超声时间-空间关联成像技术检测胎儿脐-门静脉系统发育异常

目的应用超声时间-空间关联成像(STIC)技术分析胎儿脐-门静脉系统发育异常(CAPVS)的声像图特点,探讨STIC成像技术的应用价值。方法收集产前超声筛查中心诊断的脐-门静脉系统发育异常(CAPVS)胎儿5例,孕妇年龄25~35岁,胎儿孕周13~28周。应用四维彩色多普勒超声诊断仪检测胎儿发育状况,启动STIC模式扫查胎儿脐-门静脉系统,分析脐-门静脉声像图特征。结果 5例胎儿均诊断为静脉导管缺失,其中3例经下腔静脉直接汇入体循环;1例脐静脉直接汇入右心房;1例未探及正常肝门静脉分支,仅显示一肝门静脉主干。结论二维彩色超声血流显示不清时,STIC成像技术可清晰显示脐-门静脉处血管关系,能及时发现胎儿生长受限或胎儿畸形,以便给予多次随访,改善预后。     

早孕期胎儿静脉导管异常的超声表现及预后分析

目的探讨早孕期胎儿静脉导管异常的超声表现及预后情况。方法回顾性分析我院早孕期经超声检出的26例胎儿静脉导管异常的超声图像,记录静脉导管结构、走行及脐静脉连接情况,追踪随访其预后。结果 26例胎儿均为静脉导管缺如,其中16例脐静脉汇入门静脉,4例脐静脉汇入下腔静脉,2例脐静脉汇入右房,1例脐静脉汇入左房,3例门-肝静脉分流。静脉导管缺如表现为上腹部各切面均未见静脉导管入膈下前庭,正常的脐静脉-左门静脉-静脉导管-下腔静脉之间的序贯连接关系消失,脐静脉绕过肝脏形成脐静脉肝外异位连接或肝内分流。7例合并心内外结构异常,8例合并心外结构异常,2例合并心内结构异常,9例未合并其他结构异常。染色体检查显示,16例染色体正常,1例18-三体,1例21-三体,6例未行染色体检查,2例失访。14例引产,9例出生后暂未见明显异常,1例出生后行室间隔缺损修补术后生长发育缓慢,2例失访。结论早孕期胎儿静脉导管异常的超声表现主要为静脉导管缺如,具有一定特征性;其预后情况主要取决于胎儿是否合并心内外畸形及染色体异常。     

静脉导管缺失的产前超声诊断

目的 探讨胎儿静脉导管缺失的产前超声图像特征及其临床意义.方法 回顾性分析我院诊断的9例静脉导管缺失胎儿病例,总结静脉导管缺失胎儿的产前超声图像特征,并对其相关文献资料进行回顾复习.结果 本组9例静脉导管缺失中5例脐静脉直接回流到右心房;4例脐静脉直接连于下腔静脉.9例中同时合并心内、心外系统结构异常6例,仅合并心外系统异常1例,孤立性静脉导管缺失2例.脐动脉收缩期与舒张期速度比(S/D)增高3例.静脉导管缺失超声图像特征为脐静脉进入胎儿腹腔后走行异常,脐静脉直接回流到右心房或直接入下腔静脉;胎儿上腹部各切面均无静脉导管显示.结论 胎儿静脉导管缺失病例有特征性超声图像,可以产前超声诊断,如继续妊娠,建议行胎儿染色体检查和超声追踪监测.     

静脉导管缺如并脐静脉异常连接产前超声心动图表现

目的 观察静脉导管缺如(ADV)并脐静脉肝内、肝外异常连接的产前超声心动图表现。方法 回顾性分析14胎ADV胎儿的超声心动图表现,观察心脏大小、功能变化,并随访临床预后。结果 ADV的检出率为0.12%(14/11 205),14胎中,5胎脐静脉与门静脉左支、1胎与门静脉右支相连;8胎脐静脉未与门静脉相连,而与肝外静脉系统相连,5胎直接连接于右心房,1胎直接连接于下腔静脉,1胎连接于肝右静脉,1胎连接于冠状静脉窦引流入右心房。结论 产前超声心动图能够明确诊断静脉导管缺如,且可以观察脐静脉肝内、肝外异常连接途径和管道内径。     

产前彩色多普勒超声对胎儿静脉导管异常连接的诊断价值及预后评估分析

目的：分析产前彩色多普勒超声对胎儿静脉导管异常连接的诊断价值及预后评估。方法：选取2019年4月—2022年4月于鄂尔多斯市中心医院超声科进行检查的120例孕妇为研究对象,于产前对所有孕妇进行彩色多普勒超声诊断,然后进行随访。分析典型病例的超声结果,总结静脉导管异常连接胎儿的临床资料以及超声图像表现,记录随访结果中静脉导管异常连接胎儿的结局。结果：典型病例中1例二尖瓣及三尖瓣启闭运动可见静脉导管汇入肝左静脉诊断为胎儿静脉导管走行异常,1例静脉导管汇入肝中静脉属支诊断为胎儿静脉导管走行异常。所有孕妇中共诊断出3例为静脉导管异常连接,连接途径分别为静脉导管-肝中静脉、静脉导管-冠状静脉窦-右房以及静脉导管-肝左静脉,其染色体均为低危;静脉导管-冠状静脉窦-右房患儿妊娠结局为死亡,产后行尸检表明合并主动脉狭窄,终止妊娠;1例静脉导管异常连接肝中静脉的胎儿妊娠结局良好,产后行新生儿常规检查未合并任何心内外畸形,静脉导管结局生后闭锁;另1例静脉导管汇入肝左静脉胎儿妊娠结局为引产,合并脊柱裂畸形,静脉导管生后闭锁,其余各项检查正常,随访暂未见异常。结论：产前彩色多普勒超声对于静脉导管异常连接的诊断...     

胎儿门静脉系统发育异常的产前超声诊断

目的：分析产前诊断为先天性门静脉系统发育异常（CAPVS）的超声特征。方法收集产前超声诊断为 CAPVS 患者的影像学资料,比较Ⅰ型和Ⅱ型 CAPVS 的声像特征差异。结果研究共纳入20例 CAPVS。14例Ⅰ型病例中,11例静脉导管缺失、脐静脉连接异常,3例静脉导管存在肝脏小、门静脉及分支未显示,10例 （71％）合并其他畸形,染色体异常 3例。6例Ⅱ型病例中,1例静脉导管缺失,2例左门静脉消失,3例门-肝静脉吻合,仅 1例合并结构畸形,2例行染色体检查正常。结论产前超声检查可发现和诊断 CAPVS。大部分Ⅰ型 CAPVS 病例合并其他畸形,部分合并染色体异常,而Ⅱ型病例合并畸形者较少。     

胎儿肝内门静脉-体静脉异常连接的产前超声诊断

目的 探讨胎儿肝内门静脉-体静脉异常连接的产前超声诊断价值。资料与方法 回顾分析17例胎儿肝内门静脉-体静脉异常连接的产前超声诊断特征，结合临床资料及追踪随访出生进展状况。结果 Park分型：Ⅰ型0例，Ⅱ型14例（82.35%），Ⅲ型2例（11.77%），Ⅳ型1例（5.88%）。1例合并食道闭锁和静脉导管缺失，1例合并双肾发育不良和双足姿势异常，2例合并静脉导管缺失，1例合并迷走右锁骨下动脉，其余均未见其他合并畸形。1例出现宫内发育迟缓，1例出现心功能不全，其余均未见其他并发症。妊娠结局：2例终止妊娠，1例宫内死亡，14例自然分娩(其中1例异常分流宫内自发闭合)。随访时间6~36个月，9例生后异常分流自发闭合，2例行介入栓塞治疗，1例分流仍持续性存在，1例失访。结论 产前超声对胎儿肝内门静脉-体静脉异常连接具有较高诊断准确性，为出生后临床干预提供重要参考信息。     

胎儿肝内门-体静脉分流产前超声特征分析

目的探讨胎儿肝内门-体静脉分流的产前超声特征。方法总结我院产前超声诊断肝内门-体静脉分流15例胎儿声像图特征,并对产前超声图像及合并症进行分析。结果 (1)产前15例胎儿肝内门-体静脉分流分型:Ⅰ型2例(13.3%),Ⅱ型10例(66.7%),Ⅲ型1例(6.7%),Ⅳ型2例(13.3%)。(2)共同超声表现:二维腹部横切面肝内血管走行紊乱;异常无回声区连接肝静脉与门静脉;受累肝静脉管径增宽,远端显示;异常无回声区内充满彩色血流信号,其血流频谱似门静脉血流频谱。(3)合并心脏增大5例(33.3%),径线小于孕周4例(26.7%),未发现其他异常4例(26.5%)。结论肝内血管紊乱为门-体静脉分流直接征象,心脏增大、胎儿发育偏小为间接征象。     

产前超声诊断胎儿肝外门体静脉分流1例

正孕妇,34岁。于妊娠32周来我院检查,我院产前超声显示胎儿右心增大,心包腔内0.2 cm宽的积液,三尖瓣少量反流。冠状静脉窦内径0.58 cm,肺动脉左侧可见一根血管汇入冠状静脉窦。胎儿脐静脉与肝内门静脉左支吻合后(相当于门静脉主干入肝处)发出一异常血管出肝,沿左肝外侧向后向下走行,横跨腹主动脉前方,与左肾静脉汇合后流入下腔静脉(图1),静脉导管、门静脉主干及肝内门静脉分支可见。诊断:宫内妊娠32周活胎;疑胎儿肝外门体静脉分流、永存左     

Prenatal diagnosis of abnormalities of the fetal venous system.

OBJECTIVE: To present our experience in the prenatal diagnosis of anomalies of fetal veins using high-resolution color Doppler ultrasound. DESIGN: An observational study of 16 fetuses with abnormalities of the umbilical, portal, hepatic and caval venous system being diagnosed at the Division of Prenatal Diagnosis and Therapy (Bonn, Germany) over the past 5 years. The abnormality of the venous system, the underlying embryologic disorder and the outcome of the pregnancy are presented and compared with the literature. RESULTS: In group A, eight fetuses had an abnormal course of the umbilical vein with a patent (n = 3) or absent (n = 5) ductus venosus. No portal veins and absent or abnormal hepatic veins were visualized by color Doppler sonography. Six fetuses (75%) did not have an associated malformation and have survived. Two pregnancies with fetal hydrops due to a small heart and to Turner's syndrome were terminated or ended in fetal demise. In group B, seven of eight fetuses with an abnormal caval system had a situs ambiguus or an atrial isomerism. A cardiac defect was detected in six cases (86%). These six pregnancies ended in four terminations of pregnancy and two infant deaths due to the severity of the congenital cardiac defect. One child with a normal heart and a child with an isolated abnormal course of the lower inferior vena cava are developing well. CONCLUSIONS: In a targeted fetal scan the course of the umbilical vein, ductus venosus, the portal and hepatic veins and inferior vena cava should be carefully examined using color Doppler. Any suspicious finding should be followed by a detailed assessment of the specificity of this abnormality taking into consideration the embryologic development of the fetal venous system together with the associated malformations.     

Ultrasound-Guided Umbilical Venous Catheter Insertion With Alignment of the Umbilical Vein and Ductus Venosus

Previous studies have highlighted the importance of confirming the position of an umbilical venous catheter (UVC) tip by an ultrasound (US) examination. However, methods for preventing insertion into the portal circulation under US guidance have not yet been established. We report 15 cases in which a UVC was successfully passed through the ductus venosus by compressing the upper abdomen near the portal sinus of the liver to align the umbilical vein and ductus venosus under US guidance. The UVC was inserted into the correct position in 14 of the 15 neonates (93%) without complications.     

脐静脉和静脉导管的血流参数与围产儿预后的相关性研究

目的本实验主要研究胎儿静脉导管(DV)血流量与脐静脉(UV)血流量比与围产儿预后的关系。方法通过彩色多普勒超声检测晚期妊娠胎儿的DV与UV的血流参数,分别储存图像、记录数据并计算静脉导管血流量与脐静脉血流量比,进行统计学分析。结果围产儿不良结局时静脉导管的血流量与脐静脉血流量比(DVQ/UVQ)显著增加。结论静脉导管与脐静脉血流参数可作为判断围产儿预后的一项重要指标,对指导围产医学具有一定的临床价值。     

Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification.

OBJECTIVES: Anomalies of the fetal venous system are poorly documented and their pathogenesis is not well understood. The present study was undertaken to review the spectrum of fetal central veins and umbilico-portal system anomalies, and to propose a classification system. METHODS: A 7-year restrospective survey was conducted. RESULTS: Nineteen fetuses showed abnormal connection between central veins and the fetal heart. Three fetuses showed abnormal connections of the cardinal veins, two of which had interruption of the inferior vena cava, and one had isolated persistent left superior vena cava. Anomalies of pulmonary veins were seen in four fetuses: in two with asplenia syndrome, a vertical confluent pulmonary artery was observed. In a further two cases total anomalous pulmonary venous connections were found. Abnormalities of the umbilical vein (UV) were seen in 10 cases; seven had persistent right UV, and three had a spectrum of anomalies: One had abnormal connections of the UV to the left iliac vein associated with agenesis of the ductus venosus (DV) and hydrops fetalis. One case showed in utero occlusion of the DV by echogenic foci that resulted in a persistent left proximal UV and porto-systemic shunt. One case had obliteration of the DV secondary to in utero fetal hepatic fibrosis. Abnormalities of the vitelline veins or portal system were demonstrated in two cases. One had a left porto-systemic shunt which resolved spontaneously at 3 months of age, and one had secondary partial occlusion of the left portal system with liver echogenicities and direct communication of the UV with the right atrium. None of the 19 cases had an abnormal karyotype or evidence of in utero infection. CONCLUSIONS: Detection of various fetal vein anomalies in utero is feasible. The anomalies vary according to embryologic precursors or etiology. Two major mechanisms seem to be involved in the genesis of fetal vein anomalies: in most cases primary maldevelopment of the venous system occurs, while in the minority secondary anomalies from possible thromboembolic events or systemic disease may play a role.     

Absence of the ductus venosus: report of 10 new cases and review of the literature.

OBJECTIVE: To assess the clinical significance of the absence of the ductus venosus. DESIGN: A retrospective study with a review of the literature. METHODS: The archives of our ultrasound laboratory and the English literature were searched for cases with a prenatal diagnosis of absence of the fetal ductus venosus. RESULTS: Between 1985 and 2000, 10 fetuses were diagnosed in our center as having absence of the ductus venosus. The review of the literature revealed 23 cases. Three main patterns of abnormal venous circulation were documented: (1) umbilical vein bypassing the liver and connecting directly to the right atrium (46%); (2) umbilical vein bypassing the liver and connecting to the inferior vena cava mostly through one of the iliac veins (25%); (3) umbilical vein connecting to the portal circulation without giving rise to the ductus venosus (21%). Major anomalies, including chromosomal aberrations, were found in 8/33 (24%) cases. Hydrops developed in 11/33 (33%) cases. Twenty fetuses with isolated absence of the ductus venosus were delivered, and 5 (20%) died. The portal vein was found to be absent in half of the infants examined after birth. CONCLUSIONS: Our results and the review of literature suggest that absence of the ductus venosus is associated with a high incidence of fetal anomalies and adverse outcomes, including associated malformations, chromosomal aberrations, in utero heart failure and absence of the portal vein. Heart failure and absence of the portal vein seem particularly frequent when absence of the ductus venosus is associated with a connection of the umbilical vein to either the inferior vena cava or the right atrium.     

Prenatal diagnosis of intrahepatic communications of the umbilical vein with atypical arteries (A-V fistulae) in two cases of trisomy 21 using color Doppler ultrasound.

We report on two cases of the prenatal diagnosis of arterio-venous communication between the intra-abdominal umbilical vein and atypical arteries. The diagnosis was made by color and spectral Doppler and 'color power angiography'. Both cases presented with hydrops fetalis, one at 14 and the other at 31 weeks of gestation. In the first case, color Doppler demonstrated an atypical arterial vessel connecting the umbilical vein with the aorta; the ductus venosus was patent. Echocardiography showed a so-called atrioventricular canal. In the second case, a complex intrahepatic vascular malformation was found. Color Doppler demonstrated communications between the umbilical vein and the hepatic artery and an atypical artery; the ductus venosus was patent. In the latter case polyhydramnios, duodenal atresia and macroglossia were additionally detected. In both cases, fetal karyotyping revealed trisomy 21. The first case resulted in a missed abortion, the second in a stillbirth. All findings were confirmed on autopsy. Of interest is that both fetuses were affected with trisomy 21. The increasing use of color Doppler in prenatal diagnosis, especially in hemodynamically compromised fetuses, will help to determine the actual incidence of complex vascular malformations of the umbilical vein and to elucidate the impact of such malformations on fetal outcome.     

Prenatal diagnosis of abnormal course of umbilical vein and ductus venosus agenesis: Report of three cases

Ductus venosus connecting the portal and embryonic venous circulation into the inferior vena cava has a crucial role in fetal circulation. The absence of ductus venosus is a rare anomaly, in which the umbilical vein connection to the venous system may be extrahepatic, bypassing the liver or intrahepatic via the portal venous system. We report three cases of ductus venosus agenesis with associated anomalies. In two of them the connection was directly to the right atrium, whereas the umbilical vein drained to the left internal iliac artery in the third case. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2012;     

The anatomy of the umbilical, portal and hepatic venous systems in the human fetus at 14-19 weeks of gestation.

BACKGROUND: Ultrasound imaging of the fetal ductus venosus is becoming increasingly commonplace in clinical practice. The true anatomical relationships of the fetal umbilical and portal venous systems have not been clearly defined due to paucity of published data on the relevant anatomy. This has led to confusing terminology when describing the fetal umbilical, portal and hepatic circulations. The aim of the present study was to examine and document the anatomy of the umbilical, portal and hepatic venous systems and to propose a standardized nomenclature. METHODS: This was a prospective study on 11 fetuses obtained from medical termination of pregnancies between 14 and 19 weeks of gestation. The liver was microdissected to expose the branching pattern and anatomical relations of the umbilical, portal and hepatic venous systems. RESULTS: A wide L-shaped venous confluence at the terminal end of the umbilical vein, termed the portal sinus, was identified. The portal sinus was connected to the right and left hepatic lobes, by the right and left intrahepatic portal veins, respectively. The extrahepatic portal vein drained into the portal sinus just before the origin of the right intrahepatic portal vein. The ductus venosus, a branchless straight vessel, originated from the portal sinus and ascended steeply in the direction of the diaphragm. Numerous small vessels draining the liver converged into three main hepatic veins, which open into the subdiaphragmatic vestibulum. CONCLUSION: Based on detailed sequential anatomical dissection and clear illustrations, the present study documents the anatomy of the umbilical, portal and hepatic venous systems. Taking into account the embryological origin of the vessels, a new anatomically appropriate and simplified nomenclature of these venous systems is proposed. In clinical practice, the consistent use of the suggested terminology would allow collection of comparable data between units and enable operators to be confident of which vessels they are sampling by Doppler ultrasound.     

Congenital absence of portal vein in the fetus: a case report.

Congenital absence of portal vein (CAPV) is a rare abnormality, which may be associated with other abnormalities. We report a case of prenatal diagnosis of absent portal vein confirmed on postnatal ultrasonography and computed tomography scan. The ultrasound features of CAPV include dilated intra-abdominal segment of umbilical vein, dilated inferior vena cava and the presence of hyperechogenic areas in the liver. Blood coming from the umbilical vein directly drains into the inferior vena cava. The dilated intra-abdominal segment of the umbilical vein shows high velocity pulsatile flow, resembling that of ductus venosus. These findings should prompt a careful search for the portal vein and any associated anomalies.