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Objective To investigate the incidence and concomitant findings of persistent right umbilical vein (PRUV). Methods The study was conducted by retrospective review of the data of 1856 patients who admitted to the Clinic of Obstetrics and Gynecology of Fırat University between December 2018 and December 2019 for the gestational examination between 14 and 28 weeks of gestation. The obstetric characteristics of the patients such as age, number of pregnancy, abortion, parity and body mass index were recorded. The diagnosis of PRUV was established in the conditions where portal vein reaches to stomach abnormally (roughly, course towards stomach instead of parallel course), fetal gall bladder locates in the medial of umbilical vein or umbilical vein connects to right portal vein abnormally instead of left portal vein. In the cases diagnosed with PRUV, the isolated persistent right umbilical vein or its association with other anomalies was recorded. Ultrasonography findings (minor markers) were also recorded in these cases. Results During the study period, 1856 pregnant women were evaluated for gestational examination. Five cases were established with the diagnosis of PRUV. Accordingly, PRUV prevalence was 0.27%. The PRUV incidence in the study population was 1/370 in the study population. No chromosomal anomaly was found in PRUV cases, but one case had echogenic intracardiac focus, one case had echogenic intestine, and one case had short nasal bone. When organ systems were evaluated, renal cyst was found in one case. No additional finding was found in one case. Conclusion PRUV is the most common form among fetal venous system anomalies. If detected, fetal examination is required in terms of the anomalies that may accompany. Chromosome analysis is not necessary if it is isolated, and it should be evaluated as a variant of normal anatomy.

Persistent extrahepatic right umbilical vein without ductus venosus is a rare anomaly of systemic venous drainage and is reported to be associated with cardiac anomalies. We report the case of an 8-year-old boy diagnosed to have an ostium secundum atrial septal defect, in whom an abnormal vascular channel and its opening in the right atrium in close relationship to the inferior vena cava and coronary sinus opening was identified at operation. Post-operative evaluation of this vascular channel was diagnosed to be an extrahepatic persistent right umbilical vein.

Identify structural anomalies and adverse pregnancy outcomes accompanying single umbilical artery (SUA) and persistent right umbilical vein (PRUV) and to investigate whether SUA and PRUV are associated with chromosomal abnormalities and if these defects warrant invasive antenatal diagnosis.We retrospectively analyzed pregnancies with an antenatal diagnosis of SUA/PRUV from the International Peace Maternity and Child Health Hospital (IPMCHH) database. Data of structural malformations, fetal karyotyping, and pregnancy outcomes were analyzed.Results revealed that 13.9 and 7% of SUA and PRUV cases, respectively, had malformations. Furthermore, 60% of the malformations accompanying SUA were isolated congenital heart disease (CHD), and 50% of the malformations accompanying PRUV were multiple malformations including CHD. All primarily diagnosed isolated PRUV (iPRUV) cases and 92.6% of primarily diagnosed iSUA cases had normal fetal echocardiography. Cases of iSUA and iPRUV with indications of invasive antenatal diagnosis showed no chromosomal abnormalities. The emergency cesarean section rate did not differ between the study population and the IPMCHH general population (p = .184).Patients of SUA and PRUV diagnosed in secondary hospitals should be referred to tertiary medical center for further ultrasonography. SUA and PRUV are not indicators for invasive antenatal diagnosis and selective cesarean section.

A case of Persistent Right Umbilical Vein (PRUV) is reported. Prenatal diagnosis was made on gray scale and color Doppler at the time of routine anomaly scan. The management is briefly discussed.

Introduction: To appraise the incidence and value of intrahepatic persistent right umbilical vein (PRUV). Methods: This was a single-center study. Records of all women with a prenatal diagnosis of intrahepatic PRUV were reviewed. The inclusion criteria were women with gestational age greater than 13 weeks of gestation. Exclusion criteria were fetuses with situs abnormalities, due to the hepatic venous ambiguity, and extrahepatic PRUV. The primary outcome was the incidence of intrahepatic PRUV in our cohort. The secondary outcomes were associated malformations. Results: 219/57,079 cases (0.38%) of intrahepatic PRUV were recorded. The mean gestational age at diagnosis was 21.8 ± 2.9 weeks of gestations. PRUV was isolated in the 76.7%, while in 23.3% was associated with other major or minor abnormalities. The most common associated abnormalities were cardiovascular abnormalities (8.7%), followed by genitourinary abnormalities (6.4%), skeletal abnormalities (4.6%), and central nervous system abnormalities (4.1%). Within the cardiovascular abnormalities, the most common one was ventricular septal defect (six cases). Conclusion: In most cases PRUV is an isolated finding. Associated minor or major malformations are presented in the 23.3% of the cases, so this finding should prompt detailed prenatal assessment of the fetus, with particular regard to cardiovascular system.

Objective Our objective was to evaluate the incidence of a persistent right umbilical vein in isomerism versus situs solitus. Methods For this retrospective, observational, nonrandomized report, we identified fetuses with confirmed right umbilical veins from all patients referred for fetal echocardiography in Southern Nevada between January 2006 and January 2019. Results For the period January 2006 to January 2019, we identified 89 fetuses with situs solitus and a right umbilical vein from 16 320 women undergoing prenatal cardiac evaluation, resulting in a right umbilical vein incidence of 0.5% in situs solitus. For the same period, we identified 36 fetuses with isomerism and confirmatory umbilical vein imaging. Of the 36, 15 (42%) had right umbilical veins. Of the 15, four of 11 (19%) had left isomerism, and 11 of 15 (73%) had right isomerism (P = .006). Conclusions A right umbilical vein is rare with situs solitus and common with isomerism. A right umbilical vein is more common in right isomerism than left isomerism and should alert the clinician to check for isomeric situs, especially right isomerism.

Objectives A fetal persistent intrahepatic right umbilical vein has been linked to anomalies and genetic disorders but can be a normal variant. We conducted a retrospective review to determine other sonographic findings that can stratify fetuses for further evaluation. Methods A total of 313 fetuses had a persistent intrahepatic right umbilical vein identified on 17- to 24-week sonography. The outcome was any major congenital anomaly or an adverse neonatal outcome, which was defined as aneuploidy, fetal demise, or neonatal death. Results A total of 217 patients (69.3%) had a normal neonatal outcome. Sixty-nine patients (22.0%) were lost to follow-up. Five fetuses (2.1%) had aneuploidy; 4 of the 5 had additional sonographic findings, and 1 had an isolated persistent intrahepatic right umbilical vein. Twenty-four fetuses had a major anomaly in association with the persistent right umbilical vein; 26 additional fetuses had soft sonographic markers associated with karyotypic abnormalities but were chromosomally normal. Of those with adverse neonatal outcomes, 12 had a congenital heart defect (57%). An additional sonographic finding with a persistent intrahepatic right umbilical vein was predictive of a congenital anomaly or an adverse neonatal outcome (P < .001), with a positive predictive value of 44.0% (95% confidence interval, 30.0%–58.7%). An isolated persistent intrahepatic right umbilical vein had a 0.4% risk for a congenital anomaly or an adverse neonatal outcome. Conclusions A persistent intrahepatic right umbilical vein should prompt an extended anatomic survey and a fetal cardiac evaluation. If the survey and cardiac anatomy are reassuring, no further follow-up is needed. If additional findings are identified, genetic counseling and invasive testing should be considered.

Key Clinical Message Gastroschisis is a full‐thickness congenital abdominal wall defect usually occurring to the right of the umbilicus. About twenty cases of left‐sided gastroschisis have been reported, without reference to the laterality of the umbilical vein. This first case highlights the importance of considering and reporting this association by the perinatal team.

The objective of our study was to appraise the incidence and significance of persistent right umbilical vein (PRUV), the most common foetal venous aberration. Based on a south Indian antenatal cohort, we identified 23 cases of PRUV amongst 20,452 foetuses of consecutive pregnancies, from 2009 to 2014, yielding an incidence of 1 in 889 total births (0.11%). The median maternal age was 24 (IQR, 22-26) years, and median gestational age at diagnosis was 23 (IQR, 22-24) weeks. Intrahepatic drainage of PRUV was seen in 91.3% cases. In three cases (13%), ductus venosus was absent. In 52.2% of the cases, additional major abnormalities were observed - predominantly cardiovascular (39.1%). The common minor marker was single umbilical artery (13%). The karyotype was found to be normal in six cases (26%) which underwent invasive testing. When associated anomalies were inconsequential or absent, the postnatal outcome was good, which reflected in 60.9% of our cases.

There are various numerical abnormalities of umbilical cord vessels known in the literature, the single umbilical artery being the most prevalent. A four-vessel umbilical cord is found less frequently, and moreover, is less well-known in daily practice. A persistent right umbilical vein, however, can be associated with potentially serious congenital defects. A case of a four-vessel umbilical cord containing two arteries and two veins is presented. The literature on this subject reports both a differentiation between the two variants, intrahepatic and extrahepatic, which can be distinguished during antenatal ultrasound screening, and a possible association with congenital abnormalities, some of which can carry substantial morbidity and mortality. Although the incidence of a four-vessel umbilical cord is low, its presence should be considered both during routine antenatal ultrasound screening and on physical examination of any neonate.

A case is described of a neonate with a four-vessel umbilical cord containing two arteries and two veins. This was due to a rare persistence of the caudal portion of the right umbilical vein. The infant had multiple congenital anomalies including a complete atrioventricular canal, an interrupted inferior vena cava, a double superior vena cava, a left ventricular hypoplasia, dextrocardia, situs ambiguous, and malrotation of the small bowel. The birth of an infant with a four-vessel cord mandates comprehensive work-up for associated anomalies. The literature is reviewed.

Persistent right umbilical vein (PRUV) is a vascular pathology in which the left umbilical vein becomes occluded while the right umbilical vein persists and remains open. When observing fetal anatomy during a routine 18-week scan, it is important to notice the direction of the vasculature for possible fetal anomalies such as PRUV. In this case, a full detailed sonogram of the fetus was conducted and an extrahepatic PRUV was observed using 2D imaging and further confirmed using color Doppler to identify the type of PRUV. Following the identification of the PRUV, the fetus was thoroughly assessed for the possibility of other associated anomalies. Clearly, when an anomaly is observed, it is crucial to assess the fetus for associated congenital variances to identify risk and potential outcome.

Three cases of persistent right umbilical vein associated with left-sided gallbladder are presented. This poorly understood, rare variant is due to obliteration of the left umbilical vein during embryonic development. The left-sided gallbladder, associated with persistent right umbilical vein, is not anomalous positioning of the gallbladder but an embryologic abnormality of the umbilical vein that causes the gallbladder to migrate to the left of the ligament teres. Findings and clinical relevance are discussed.

Persistent right umbilical vein (PRUV) is a common anomaly of the venous system. Although candidates for future PRUV were expected to occur more frequently in earlier specimens, evaluation of serial horizontal sections from 58 embryos and fetuses of gestational age 5–7 weeks found that only two of these embryos and fetuses were candidates for anomalies. In a specimen, a degenerating right umbilical vein (UV) joined the thick left UV in a narrow peritoneal space between the liver and abdominal cavity, and in the other specimen, a degenerating left UV joined a thick right UV in the abdominal wall near the liver. In these two specimens, the UV drained into the normal, umbilical portion of the left liver. These results strongly suggested that, other than the usual PRUV draining into the right liver, another type of PRUV was likely to consist of the right UV draining into the left liver.

Objective: To evaluate outcomes of fetuses with antepartum sonographic diagnoses of persistent intrahepatic right umbilical veins. Methods: A detailed fetal sonographic examination was done in 30,240 consecutive pregnancies at 14–26 weeks’ gestation. High- and low-risk pregnancies were included and persistent right umbilical veins specifically were recorded. Results: Sixty-nine fetuses had persistent intrahepatic right umbilical veins, of which 60 had no additional sonographic abnormalities, four had transient nuchal findings, and four had minor anomalies or anatomic variants. Only one of the 69 fetuses had a major anomaly (diaphragmatic hernia), and died after surgery. The remaining 68 fetuses were normal and healthy after birth. Conclusion: Persistent intrahepatic right umbilical vein is a fetal anatomic variant that is not rare and usually associated with a favorable outcome.

Persistent right umbilical vein is a rare entity, with a prevalence of about 0.22%.1 The intrahepatic variant is observed most frequently, and 72% of the fetuses are normal.2,3 In a very limited number of cases, the persistent right umbilical vein terminates directly into the right atrium, without passage into the liver. The majority of fetuses with this variety have multiple congenital malformations.2 We report a case of persistent right umbilical vein draining directly into the right atrium in association with trisomy 18. Although particular sonographic markers related to Edwards syndrome were present in our case, we would recommend fetal karyotyping in all cases of persistent right umbilical vein. CASE REPORT

We present a case of persistent right umbilical vein with Noonan's syndrome. Persistence of the right umbilical vein with no intrahepatic portion is an uncommon finding with a review of the literature revealing only five other cases. In these cases drainage was directly into the right atrium, inferior vena cava (IVC) or IVC via the iliac vein. Of the reported cases Noonan's syndrome was confirmed in one case and suspected in a second. This report represents a third case of persistent right umbilical vein with confirmed Noonan's syndrome.

The persistence of a right umbilical vein is an uncommon finding, with only a dozen cases reported since 1826. The persistent right umbilical vein may replace the normal left umbilical vein or be supernumerary. The anomaly is associated with numerous and occasionally lethal malformations. In this series, only three of six fetuses (and another two in the literature) had no associated anomalies. All the others had a variety of associated lesions ranging from minor to lethal. The appearance at ultrasound is easy to recognize: The intrahepatic portion of the umbilical vein is lateral to the gallbladder, and the portal vein curves toward the stomach, instead of parallel to it. Since the recognition of the persistent right umbilical vein is simple and does not require additional scanning (it is visible in the section used to measure the abdominal perimeter), the author suggests using it as an indicator for more in-depth scanning.

Gastroschisis, a ventral body wall defect, is a continuing challenge and concern to researchers, clinicians, and epidemiologists seeking to identify its cause(s) and pathogenesis. Concern has been renewed in recent years because, unlike most other birth defects, rates of gastroschisis are reportedly increasing in many developed and developing countries. No tenable explanation or specific causes have been identified for this trend. Rates of gastroschisis are particularly high among pregnancies of very young women. Such an intriguing association, not observed to this degree with other birth defects, may afford clues to the defect's cause. Understanding the causes of gastroschisis may provide insight to the defect's origin. In pursuing such causal studies, it would be helpful to understand the embryogenesis of gastroschisis. To date, four main embryologic hypotheses have been proposed: (1) Failure of mesoderm to form in the body wall; (2) Rupture of the amnion around the umbilical ring with subsequent herniation of bowel; (3) Abnormal involution of the right umbilical vein leading to weakening of the body wall and gut herniation; and (4) Disruption of the right vitelline (yolk sac) artery with subsequent body wall damage and gut herniation. Although based on embryological phenomena, these hypotheses do not provide an adequate explanation for how gastroschisis would occur. Therefore, we propose an alternative hypothesis, based on well described embryonic events. Specifically, we propose that abnormal folding of the body wall results in a ventral body wall defect through which the gut herniates, leading to the clinical presentation of gastroschisis. This hypothesis potentially explains the origin of gastroschisis as well as that of other developmental defects of the ventral wall. © 2007 Wiley-Liss, Inc.

A persistent right umbilical vein is thought to be a rare anomaly, frequently associated with other life-threatening malformations. Eight cases of prenatal diagnosis of persistent right umbilical vein are presented. All cases were discovered on a routine second-trimester sonogram in an unselected population. Of the seven babies who were delivered, one had a dextrocardia and right-sided descending aorta, but none had other associated major malformations. The outcome was favorable in all cases and all seven infants are well and thriving with up to a year and a half of follow-up. Our findings suggest that persistent right umbilical vein might not be as rare as the paucity of reports in the literature implies. Provided that no other malformations are present, this anomaly is probably of little prognostic significance, requiring no further evaluation or follow-up.

Objectives: Our aim is to describe the ultrasound findings, maternal and perinatal variables in cases with a prenatal diagnosis of persistence of right umbilical vein. Methods: A retrospective analysis of fetuses with ultrasound prenatal diagnosis of PRUV attending in two terciary centers, in Madrid. We describe clinical maternal-perinatal and fetal variables in all cases. Results: A total of 20426 deliveries were attending in both centers during the period of recruitment. Twenty-two cases (0.1%), with PRUV were detected. All cases were intrahepatic type a Ductus venosus with a normal Doppler waveform was presented in all cases. Nine fetuses (40.9%) were proved to have additional ultrasound anomalies. None of these non-isolated cases had chromosomal abnormalities. Cardiovascular malformations were the most frequently associated congenital anomalies. Conclusions: Based on our results and a literature review, PRUV has a potential association with other fetal malformations and a very low rate of chromosomal abnormalities. After prenatal diagnosis of persistent right umbilical vein a detailed anatomical evaluation and an echocardiography should be performed in order to rule out other structural malformations. Indication for fetal karyotype study has to be individualized considering persistence right umbilical vein type and other ultrasound findings.

Objectives Our purpose was to conduct the first prospective evaluation of the incidence and neonatal outcome of fetuses with persistent right umbilical vein (PRUV). This condition had traditionally been considered to be extremely rare and to forebode grave consequences for the babies, but later evidence raised some doubt upon the veracity of these contentions. Methods Between August 1995 and November 1998, 8950 low-risk patients were prospectively evaluated at two medical centers. The ultrasonographic 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 gallbladder was located medially to the umbilical vein. Results Seventeen cases of fetuses with PRUV were detected during the study period among the 8950 study participants. Four of them had additional malformations of which three had been detected antenatally. Conclusions We established that the incidence of PRUV 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 fetal echocardiography. When the PRUV is connected to the portal system and after other anomalies are ruled out, the prognosis can generally be expected to be favorable.

AIM To detect the anomaly in the persistent right umbilical vein (PRUV) of portal vein (PV) with deviation of the ligamentum tere and left-sided gallbladder. METHODS A total of 5783 candidates for routine analysis were evaluated for hepatic vascular abnormalities by ultrasonography. RESULTS Ten candidates (0.17%) had a portal vein anomaly with a rightward-deviated ligamentum tere. The blood-flow velocity in the PRUV of the portal vein (17.7+/-3.0 cm/s) of the 10 cases was similar to that of the right anterior portal trunk (17.6+/-4.1 cm/s). However, the vessel diameter of the PRUV (eta12.4+/-4.4 mm) was larger than the right anterior portal trunk (eta6.1+/-0.9 mm). Therefore, flow volume in the anomalous portion (0.97+/-0.30 L/min) was more than that in the right anterior portal trunk (0.18+/-0.05 L/min). CONCLUSION The anomaly plays an important role in intra-hepatic PV flow.

Background Abnormal venous connections involving a persistent right umbilical vein are rare. In a minority of cases the liver is entirely bypassed and the condition is associated with multiple congenital malformations. Case presentation The described case illustrates a systemic venous drainage that was severely abnormal in a newborn girl with a truncus arteriosus type II congenital heart defect. Injection of contrast medium through the umbilical vein catheter revealed a very peculiar venous connection that passed anterio-laterally through the right hemithorax before crossing in an oblique fashion towards the superior vena cava. Conclusions This venous drainage may be the result of a persistent right umbilical vein connecting with the superior vena cava.

A case of a ligamentum teres formed from an obliterated right umbilical vein is described. It passed to the right branch of the portal vein. The quadrate and left lobes of the liver were not separated by the usual fissure. Very few cases of anomalous umbilical veins or persistent right umbilical veins have been recorded. Of these, several have been recorded only in the umbilical cord, while in others the persistent right umbilical vein has been found intra-abdominally, in an extrahepatic position, and passing directly to the right atrium or to the inferior vena cava. Its presence is generally associated with severe congenital abnormalities, in contrast with the present case. In view of the high incidence of congenital defects associated with aberrant or accessory umbilical veins, when these are detected either in the umbilical cord or in the abdomen by umbilical phlebography, it is suggested that the patient should be carefully investigated for other congenital abnormalities.

An abnormal umbilical vein that terminated in the right atrium, which was described by Mende in 1826, is considered to be an example of persistence of the right umbilical vein in man. A similar abnormality has been found in rat fetuses following the administration of certain teratogens to females during pregnancy.

Of the two umbilical veins formed in young rat embryos, the right regresses early while the left persists until birth. In a number of rat fetuses from mothers subjected to a transitory deficiency of pteroylglutamic ("folic") acid during pregnancy, this pattern was reversed and the right, instead of the left, umbilical vein persisted until birth. In such cases, the ductus venosus was always absent. No record of persistent right umbilical vein has been found in the literature.

Umbilical vein collaterals frequently occur in patients with portal hypertension, with the vessels usually connected to the left portal system. An umbilical vein collateral draining the right intrahepatic portal system was found in a patient with portal hypertension. This observation has not been previously reported and probably represents persistence of the embryonic right umbilical vein.