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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.

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 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.

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.

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.