Persistent intrahepatic right umbilical vein: a prenatal sonographic series without significant anomalies.

Umbilical venous anatomy variation is considered extremely rare. The intrahepatic persistent right umbilical vein represents one version in the spectrum of umbilical vein variations. Prior reports of a prenatally diagnosed persistent right umbilical vein suggest it is strongly associated with severe fetal anomalies including congenital heart disease and gastrointestinal malformations. Our experience contrasts with these findings. Nine cases of intrahepatic persistent right umbilical vein were identified via prenatal sonography between July 1992 and January 1994. The gestational ages of the fetuses ranged from 20 to 36 weeks. Targeted fetal surveys were performed in all nine fetuses and formal fetal echocardiograms were obtained in six. All nine infants have been delivered. In nine cases, the intrahepatic persistent right umbilical vein was an isolated observation with no abnormalities identified pre- or postnatally except one case of hypospadias. Formal fetal echocardiography was normal in all six of the fetuses studied. Given previous reports describing a high risk of congenital malformations in association with the presence of a persistent right umbilical vein, the sonographic finding of this anomaly should still prompt targeted fetal sonography and fetal echocardiography. However, our series indicates that this vascular variant may occur as an isolated abnormality and may not necessarily represent the ominous finding suggested previously.     

Two ductus venosus: a previously unreported anomaly

In early fetal life, the ductus venosus (DV) connects the umbilical vein, carrying nutrient-rich and oxygenated blood from the placenta to the inferior vena cava (IVC). We present the first ever case of a fetus with two ductus venosus. The fetus presented with a four-vessel cord and a dilated bowel at 31 weeks of gestation. Ultrasonography showed a persistent right umbilical vein. Echocardiographic investigation revealed normal cardiac anatomy with no major malformations. Two DVs with slightly different Doppler patterns were visualized anastomosing with the IVC. The baby was born uneventfully at 39 weeks of gestation with stable hemodynamics. Assuming every supernumerary umbilical vein should be connected to a DV for balanced circulation, a fetus with supernumerary umbilical veins lacking a corresponding number of DV connections is likely to be predisposed to complications such as hydrops fetalis and poor perinatal outcomes. The possibility of one or more umbilical veins lacking a DV connection warrants significant attention and regular monitoring from feto-maternal specialists, given the severity of the associated morbidity and mortality.     

Noonan's syndrome and seminoma of undescended testicle

A 26-year-old white man with Noonan's syndrome and a history of lifetime lymphedema had had bilateral orchiopexy for undescended testicles at the age of 12. He noticed increased swelling of the right testicle confirmed by ultrasonography as a solid mass. Computed tomography of the abdomen and pelvis showed multiple enlarged mesenteric and retroperitoneal lymph nodes. At right inguinal orchiectomy, pathologic findings were consistent with seminoma of the right testicle. Postoperatively, he was treated with four cycles of cisplatin and etoposide. A case of nonseminoma in Noonan's syndrome has been reported previously, but this is first case report of seminoma in a patient with Noonan's syndrome.     

超声对胎儿永存右脐静脉的诊断价值

目的 探讨超声对胎儿永存右脐静脉的诊断价值.方法 回顾分析我院诊断的3例胎儿永存右脐静脉的灰阶及彩色多普勒超声表现及相关临床情况.结果 3例胎儿均可见胆囊位于腹部正中,脐静脉连接门脉右支,门静脉窦凹面朝向胃泡.其中1例合并颅内其他异常并在出生后死亡,另2例不合并其他异常,出生半年后生长发育未见异常.结论 胎儿永存右脐静脉具有特征性超声表现,不合并其他畸形时预后良好,早期检出并给予适当的遗传咨询具有重要的临床意义.     

Intrahepatic Persistent Right Umbilical Vein and Associated Outcomes

The aim of this study was to provide a comprehensive review of the current data surrounding an intrahepatic persistent right umbilical vein in the fetus, including associated anomalies and outcomes, and to assist practitioners in counseling and management of affected pregnancies. We performed a MEDLINE, Embase, Cochrane Central Register of Controlled Trials, and Northern Light database search for articles reporting outcomes on prenatally diagnosed cases of a persistent right umbilical vein. Each article was independently reviewed for eligibility by the investigators. Thereafter, the data were extracted and validated independently by 3 investigators. A total of 322 articles were retrieved, and 16 were included in this systematic review. The overall prevalence of an intrahepatic persistent right umbilical vein was found to be 212 per 166,548 (0.13%). Of the 240 cases of an intrahepatic persistent right umbilical vein identified, 183 (76.3%) were isolated. The remaining cases had a coexisting abnormality, including 19 (7.9%) cardiac, 9 (3.8%) central nervous system, 15 (6.3%) genitourinary, 3 (1.3%) genetic, and 17 (7%) placental/cord (predominantly a single umbilical artery). In summary, a persistent right umbilical vein is commonly an isolated finding but may be associated with a coexisting cardiac defect in 8% of cases. Therefore, consideration should be given to fetal echocardiography in cases of a persistent right umbilical vein.     

彩色多普勒超声诊断先天性下腔静脉畸形的临床应用价值

目的分析不同类型先天性下腔静脉畸形的彩色多普勒超声表现及其相应的临床特征,探讨彩色多普勒超声诊断先天性下腔静脉畸形的临床应用价值。方法回顾性分析2009年7月至2012年3月于南京军区福州总医院超声诊断科行腹部彩色多普勒超声检查患者的临床资料,共发现22例先天性下腔静脉畸形,采用分段、多切面、自下而上或自上而下顺序进行超声扫查,结合二维、彩色多普勒、频谱多普勒超声逐段分析并进行最后的诊断,所有病例均为计算机体层扫描静脉造影、数字减影血管造影或临床其他检查所证实。结果22例下腔静脉畸形中包括左下腔静脉5例,超声表现为肾后段腹主动脉左侧上行的下腔静脉与左肾静脉汇合,肾前段跨越腹主动脉前方至右侧下腔静脉。双下腔静脉3例,超声表现为肾后段腹主动脉两侧上行的下腔静脉,肾前段左侧下腔静脉跨越腹主动脉前方经右侧下腔静脉回流入右心房：部分左下腔静脉和双下腔静脉患者伴有左肾静脉压迫。下腔静脉膜性梗阻3例,临床表现均为布-加综合征,超声显示肝后段下腔静脉右心房入口处见隔膜高回声带,彩色多普勒血流成像示肝后段下腔静脉右心房入口处未见血流,静脉腔内见逆心性血流流入腰静脉、腰升静脉。左肾静脉畸形11例,其中腹主动脉后左肾静脉9例,环主动脉型左肾静脉1例,后左肾静脉汇入左髂总静脉1例,9例伴有左肾静脉压迫。结论彩色多普勒超声可清晰显示下腔静脉及其属支的畸形,评价血流动力学的变化及其相应的临床表现,可成为下腔静脉畸形的重要诊断方法。     

Radiofrequency catheter ablation of a coronary sinus-ventricular accessory connection in dextrocardia with complete situs inversus and an anomalous inferior vena cava

An anomalous inferior vena cava (IVC) is often associated with patients with dextrocardia. However, radiofrequency catheter ablation in such a case with that combination has not been reported. We encountered a case of Wolff-Parkinson-White syndrome with dextrocardia associated with complete situs inversus and an azygos continuation of the IVC. A steep angulation at the junction of the azygos vein and superior vena cava precluded the precise mapping of the anatomical right cavity with a femoral vein approach. Successful catheter ablation of an accessory connection between the coronary sinus and left ventricle could be achieved with a right cubital vein approach.     

下腔静脉血经肝回流入右心房：布—加氏综合征的一种特殊类型

目的 回顾性研究一种特殊类型的布－加氏综合征：下腔静脉肝回流入右心房。方法 彩多多普勒超声检查１１４例布－加氏综合征患者,观察下腔静脉和三支肝静脉的走行、入口和血流方向以及肝内外交流支。结果 共５例布－加氏综合征患者呈现特殊循环方式,表现为肝段下腔静脉血经一支肝静脉入肝,通过肝内交通支 另一支肝静脉回流右心房。诊断均经介入手术证实。结果 下腔静脉血经肝加 型布－加氏综合征是一种少见类型,其特点是下     

Persistent right umbilical vein: incidence and significance.

OBJECTIVES: To conduct a prospective evaluation of the incidence and neonatal outcome of fetuses with persistent right umbilical vein. This condition had traditionally been considered to be extremely rare and to be associated with a very poor neonatal prognosis, but later evidence has raised some doubts about the veracity of these contentions. METHODS: Between August 1995 and November 1998, 8950 low-risk patients were prospectively evaluated at two medical centers. The sonographic diagnosis of a persistent right umbilical vein was made in a transverse section of the fetal abdomen when the portal vein was curved toward the stomach, and the fetal gall bladder was located medially to the umbilical vein. RESULTS: Persistent right umbilical vein was detected in 17 fetuses during the study. Four of them had additional malformations, of which three had been detected antenatally. CONCLUSIONS: We established that the incidence of persistent right umbilical vein in a low-risk population is 1 : 526. We believe that the sonographic finding of this anomaly is an indication for conducting targeted fetal sonography and echocardiography. When the persistent right umbilical vein is connected to the portal system and other anomalies are ruled out, the prognosis can generally be expected to be favorable.     

Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion.

OBJECTIVE: The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography. METHODS: We searched our fetal cardiology database for diagnoses of ADV from May 2003 to December 2006. RESULTS: During the study period, we performed 1328 fetal echocardiographic examinations in 990 fetuses. We found 6 cases of ADV (6/1000). Indications for fetal echocardiography were cardiomegaly, dilated umbilical or systemic veins, and extracardiac abnormalities. We identified 5 anatomic variants of ADV. In 2 patients, the umbilical vein connected to the systemic venous circulation by way of the portal sinus: via an abnormal venous channel from the portal sinus to the right atrium (case 1) and presumably via hepatic sinusoids to the hepatic veins (case 2). In the remaining 4 patients, the umbilical vein bypassed the portal sinus and the liver and connected to the systemic venous circulation via an abnormal venous channel: from the umbilical vein to the right atrium (case 3), from the umbilical vein to the inferior vena cava (cases 4 and 5), and from the umbilical vein to the right iliac vein (case 6). All patients survived; 2 required cardiovascular intervention. No intervention was required in 3 patients. CONCLUSIONS: An ADV should be ruled out in a fetus with unexplained cardiomegaly or dilatation of the umbilical vein, systemic veins, or portal sinus. To our knowledge, prenatal diagnosis of an ADV with an abnormal communication between the portal sinus and the right atrium has not been reported previously. The portosystemic communication persisted after birth and required device occlusion.     

Couinaud肝段在CT图像上的划分

为给肝内占位性病变的ＣＴ定位提供解剖学依据，利用２０例腹部连续横断层标本和１０例活体正常肝的ＣＴ图像，研究了Ｃｏｕｉｎａｕｄ肝段在ＣＴ图像上的划分。正中裂为下腔静脉左前壁至肝中静脉或胆囊的连线；左叶间裂的上部为左叶间静脉至下腔静脉左前壁的连线，门静脉左支脐部和肝圆韧带裂是其中、下部的天然标志；肝左静脉行于左段间裂内；右叶间裂为肝右静脉至下腔静脉左前壁的连线；肝门或门静脉右支是右段间裂的标志；背裂为下腔静脉右缘至静脉韧带裂右端或门静脉的连线。本文还探讨了易致错分肝段的一些因素。     

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.     

The prenatal sonographic features of Noonan's syndrome

The sonographic findings in four infants with Noonan's syndrome are described. All four fetuses had cystic hygromata located laterally along the cervical spine and normal karyotypes. Three of the four pregnancies were complicated by polyhydramnios and those three fetuses had associated pleural effusions. One fetus developed frank hydrops and did not survive, a clinical course that appears to be part of the clinical spectrum of Noonan's syndrome. The diagnosis of Noonan's syndrome was given serious consideration prenatally based on the sonographic findings and normal karyotype and confirmed at birth in all four cases. Three of the infants survived.     

Scimitar syndrome; an unusual congenital abnormality occasionally seen in adults

Scimitar syndrome a very rare and variable congenital disorder characterized by an anomalous connection of the pulmonary vein with the IVC. The syndrome is mostly seen in very early infancy, but was now recognized in a 46-year-old woman, who was referred to the outpatient clinic of the department of cardiology with complaints of dizziness. Contrast enhanced computer tomography (CT) showed dextroposition of the heart and a large right pulmonary vein joined the inferior vena cava (IVC) just above the level of the diaphragm. The typical features of the syndrome are discussed.     

Deep vein thrombosis in patient with left-sided inferior vena cava draining into the hemiazygos vein: A case report

BACKGROUNDAbnormalities of the inferior vena cava (IVC) are uncommon, and in many cases they are asymptomatic. Even so, it is vital that clinicians be aware of such anomalies prior to surgery in affected individuals. In the present report, we describe a rare anatomical variation of the IVC.CASE SUMMARYA 66-year-old male was admitted to the hospital due to deep vein thrombosis of the right lower extremity. Upon contrast-enhanced computed tomography imaging, we found that this patient presented with a case of left-sided IVC draining into the hemiazygos vein, while his hepatic vein was directly draining into the atrium.CONCLUSIONCases of left-sided IVC can increase patient susceptibility to thromboembolism owing to the resultant changes in blood flow and/or associated vascular compression.     

复杂先心病合并体静脉畸形的二维及彩色多普勒超声诊断

目的：探讨复杂先天性心脏病合并常见体静脉畸形的超声特征，以便做出正确的诊断。方法：与结果：本文报道经二维和彩色多普勒诊断并经造影或手术证实69例复杂先天性心脏病合并体静脉畸形引流，其中部分患者为多发静脉畸形；69例中合并静脉畸形77例次，包括永存左上腔静脉(LSVC）47例次；左上腔静脉入左房15例次；合并奇静脉异常连接的下腔静脉(IVC)肾上段中断10例次和严重狭窄1例；左无名静脉走行异常4例次。结论：1．心房异构时，体静脉畸形发生率显菩高于心房正位．且异构的性质与体静脉畸形引流类型密切相关；2．为了正确诊断体静脉畸形引流，应进行多切面系统检查，尤其注意对剑下、胸骨上窝和胸骨旁高位切面的观察；3．剑下短轴切面见腹主动脉/下腔静脉与脊柱的对称关系消失，则提示下腔静脉异常；左胸骨旁高位与胸骨上窝切面多能直接显示左上腔静脉，同时左无名静脉非常细小或缺如。如果冠状静脉窦无扩张，则高度怀疑左上腔静脉入左房。左上肢静脉声学造影对左上腔静脉畸形的识别有重要意义。     

下腔静脉先天畸形MSCT表现及临床意义

目的 探讨下腔静脉(IVC)先天畸形的MSCT表现及其临床意义。方法 回顾性分析960例患者中48例IVC先天畸形的腹部增强CT影像资料,观察MSCT动脉期和静脉期IVC后处理图像及其属支的解剖结构。结果 IVC先天畸形的发生率为5.00%(48/960)。左IVC 4例(4/48,8.33%),CT示肾下段IVC位于腹主动脉左侧;双IVC 5例(5/48,10.42%),CT示腹主动脉两侧上行的IVC;主动脉后型左肾静脉12例(12/48,25.00%),CT示左肾静脉走行于腹主动脉后方;环主动脉型左肾静脉26例(26/48,54.17%),CT示左肾静脉分两支环绕腹主动脉,在不同水平分别注入IVC;IVC伴奇静脉延续畸形1例(1/48,2.08%),增强CT示IVC向上延续为奇静脉,肝静脉直接回流至右心房。结论 MSCT可清晰、直观地显示IVC畸形,对诊断与IVC有关的疾病和防止术中血管损伤有重要临床意义。     

超声检查附脐静脉开放腹壁静脉曲张在门静脉高压临床分型的意义

目的应用彩色多普勒超声对门静脉高压附脐静脉开放和腹壁静脉曲张门腔之间侧支循环进行研究,确定门脉高压分型。方法超声检查86例肝硬化门静脉高压、13例布加综合征及6例门静脉主干、脾静脉血栓3组门静脉高压患者的腹壁静脉曲张门腔之间侧支循环吻合情况及血流方向。结果肝硬化门静脉高压组腹壁静脉曲张在脐以上血流流向头端,而脐以下血流流向腹端。布加综合征合并下腔静脉阻塞组,血流均流向上胸端。门静脉主干、脾静脉血栓未见脐静脉开放及腹壁静脉曲张。结论应用彩色多普勒超声判断附脐静脉开放和腹壁静脉曲张门腔之间侧支循环的情况,可明确血管阻塞部位、程度、范围,为肝前、肝内及肝后门静脉高压分型的诊断提供有效依据,对临床制定合理治疗方案具有指导意义。     

Slow pathway ablation for atrioventricular nodal reentry using a right internal jugular vein approach: a case series

BACKGROUND: Inferior venous access to the right heart is not possible in some patients due to congenital or acquired obstruction of the inferior vena cava (IVC). Although right-sided electrophysiology procedures have been performed successfully in patients with a previously placed IVC filter by direct placement of catheters through the filter, an alternative approach is necessary in some patients. METHODS: This case series describes three patients with an IVC filter who underwent successful ablation of the slow pathway for typical atrioventricular (AV) nodal reentrant tachycardia using a superior vena cava (SVC) approach via the right internal jugular (IJ) vein. Two separate introducer sheaths were placed into the IJ vein using separate punctures. This permitted placement of a standard deflectable ablation catheter and an additional catheter in the right atrium to monitor for ventriculoatrial conduction during the junctional rhythm associated with ablation of the slow AV nodal pathway. RESULTS: Catheter ablation was successful in each patient. The number of radiofrequency current applications was 7, 17, and 27. There were no procedural complications and no patient had recurrent tachycardia during follow-up. CONCLUSIONS: Catheter ablation of the slow AV nodal pathway can be performed successfully and safely in patients with inferior venous barriers to the right heart using an SVC approach via the right IJ vein.     

B-TFE与3D DCE-MRA联合应用在布加综合征诊断中的价值

目的探讨B-TFE与3D DCE-MRA联合应用在布加综合征（BCS）诊断中的价值。材料与方法搜集经DSA证实的布加综合征患者32例,其中8例为单纯下腔静脉阻塞,2例为单纯肝静脉阻塞,22例为下腔静脉阻塞伴肝静脉阻塞。32例患者均行B-TFE和3D DCE-MRA检查,分析两种方法及联合应用时对BCS及其侧支循环的显示情况。结果 B-TFE正确诊断8例下腔静脉阻塞、18例下腔静脉阻塞伴肝静脉阻塞和2例肝静脉阻塞,总检出率为87.5%（28/32）;3D DCE-MRA正确诊断8例下腔静脉阻塞、19例下腔静脉阻塞伴肝静脉阻塞和1例肝静脉阻塞,总检出率为93.8%（30/32）。B-TFE共显示个193侧支循环,3DDCE-MRA则显示248个侧支循环。两者结合准确诊断全部BCS及其肝内外侧支循环。结论联合应用B-TFE和3DDCE-MRA是诊断BCS的理想方法。I