Umbilical Vein Varix Research Articles

Spontaneous echo contrast and decreased umbilical vein blood flow may predict thrombus formation in fetal intra-abdominal umbilical vein varix.

Fetal intra-abdominal umbilical vein varix (FIUVV) can cause thrombosis, fetal growth restriction (FGR), and intrauterine fetal death (IUFD). However, its management and evaluation to avoid fetal risks have not been elucidated. The aim of this study was to develop a novel method to evaluate fetal risks, including FGR and fetal dysfunction via frequent ultrasound examinations. A 28-year-old pregnant woman was diagnosed with FIUVV via ultrasound at 26weeks of gestation and admitted to our hospital. Ultrasound examinations were performed two to three times weekly to evaluate size and shape of the FIUVV and umbilical vein blood flow at the inflow and outflow sites of the FIUVV. The outflow site of the FIUVV was constricted and collapsed, and the blood flow velocity at the inflow site of the FIUVV was decreased. At 32weeks of gestation, spontaneous echo contrast (SEC), which indicates increased echogenicity, appeared. At 35weeks of gestation, the patient noticed decreased fetal movement, and CTG showed non-reassuring fetal status. SEC in the FIUVV was remarkable. Fetal movement could not be confirmed at ultrasound. Cesarean section was performed and a 1,854-g healthy infant was delivered with an umbilical cord arterial pH of 7.266. The echographic changes, such as decreased umbilical vein blood flow and SEC, in FIUVV observed in this case could indicate thrombus formation, which can lead to fetal dysfunction. Frequent ultrasound examinations can help determine the timing of delivery and improve the neonatal prognosis.

Isolated fetal umbilical vein varix and the association with intrauterine fetal death and fetal growth restriction: A systematic review, meta-analysis, and nested retrospective cohort study.

To assess the risk of intrauterine fetal death (IUFD) and fetal growth restriction (FGR) in fetuses with an isolated fetal intra-abdominal umbilical vein varix (i-FIUVV). A retrospective cohort study combined with a systematic review and meta-analysis of the literature was performed. In the retrospective cohort study, all singleton fetuses with an i-FIUVV in the fetal medicine units of the Amsterdam UMC (between 2007 and 2023) were analyzed. The primary outcome measures were IUFD and FGR. The sample proportions of IUFD and FGR were depicted as risk percentages. The IUFD proportion was compared to the regional reference population and the FGR proportion was compared to the reported proportions in Europe. The secondary outcome measures were gestational age at diagnosis, initial and maximal FIUVV diameter, fetal monitoring in pregnancy, turbulent flow in the varix, thrombus formation in the varix, induction of labor, gestational age at birth, and birthweight centile. The proportion of fetuses with a birthweight below the 10th centile was compared with that of the regional reference population. The systematic review included all cases from eligible literature published between 2007 and 2023 supplemented by the data of our retrospective cohort study. In the systematic review and meta-analysis, the pooled proportions of IUFD and FGR were assessed in fetuses with i-FIUVV. The retrospective cohort included 43 singletons with an i-FIUVV. The IUFD risk was 0% [Confidence Interval, CI: 0%-8.2%], which did not differ significantly from 0.3% in the reference population, p=1.0. The risk of FGR was 16.3% [CI: 6.8%-30.7%] in the studied population, which is higher than the reported incidence of FGR in Europe ranging from 5%-10%. The proportion of fetuses with birthweights below the 10th centile was higher in our cohort compared with the reference population (23.3 vs. 9.9%, p<0.01). The systematic review included 12 articles, three abstracts, and our current cohort. In total, 513 cases with an i-FIUVV were included. The pooled risk was 0.4% [CI: 0.1%-1.7%] for IUFD and 5.2% [CI: 1.1%-21.3%] for FGR. The mean gestational age at birth did not exceed 39weeks in neither the cohort (38.7weeks) nor the pooled literature (37.6weeks). An i-FIUVV in singletons is not associated with an increased IUFD risk up to 39weeks of gestation but is possibly associated with FGR. The incidence of FGR in our cohort was higher than in the pooled literature (16.3% vs. 5%) but FGR definitions in the included studies varied. The proportion of birthweights below the 10th percentile in our cohort was significantly higher than in the reference group. Thus, based on these findings, we suggest conducting sonographic growth assessments while simultaneously assessing the i-FIUVV. No further monitoring and follow-up are indicated up to 39weeks of gestation. After 39weeks of gestation, data on fetuses with i-FIUVV and their outcomes are lacking.

Fetal extra-abdominal umbilical vein varix: Two cases of a rare malformation

Fetal umbilical vein varix is a rare malformation of the umbilical cord. Most of the varices arise intraabdominally and there have been multiple studies about the diagnosis and management of fetal intra-abdominal umbilical vein varices. However fetal extra-abdominal umbilical vein varices are much rarer and up to date there are no clear criteria for the diagnosis or the clinical management of the condition. We saw two cases within a month in our ultrasound department in 2022 and had a favorable outcome in both pregnancies. In the first case the diagnosis was only established at 39 weeks of gestation, and we performed a cesarean section the next day. The second case was diagnosed at 31 weeks of gestation. The patient was admitted to hospital, and we administered Betamethasone for RDS prophylaxis. At 34 weeks of gestation there were fetal heartbeat abnormalities, and a cesarean section was performed. We also reviewed the literature of previous cases and our two cases included, so far only 19 cases of fetal extra-abdominal umbilical vein varix have been described.

Isolated fetal intra-abdominal umbilical vein varix: A population-based review of obstetrical and neonatal outcomes.

To assess obstetrical and neonatal outcomes associated with isolated fetal intra-abdominal umbilical vein varix (FIUVV).

Foetal death due to extensive extra-abdominal umbilical vein Varix with umbilical vein thrombosis: a case report

BackgroundFoetal anaemia and umbilical vein thrombosis are rare pregnancy complications that can increase the risk of perinatal adverse events, which, in severe cases, can lead to foetal death. During pregnancy, umbilical vein varix (UVV) commonly occurs in the intra-abdominal part of the umbilical vein and is associated with an increased risk of foetal anaemia and umbilical vein thrombosis. However, UVV occurring in the extra-abdominal part of the umbilical vein is rare, especially when accompanied by thrombosis. In this case report, we describe a rare case of an extensive extra-abdominal umbilical vein varix (EAUVV), which ultimately resulted in foetal death due to umbilical vein thrombosis.Case presentationIn this report, we describe a rare case of an extensive EAUVV that was discovered at 25 weeks and 3 days of gestation. During the examination, there were no abnormalities in foetal haemodynamics. The estimated weight of the foetus was only 709 g. In addition to refusing to be hospitalized, the patient refused close monitoring of the foetus. As a result, we were limited to choosing an expectant therapy. The foetus died 2 weeks after diagnosis and was confirmed to have EAUVV with thrombosis after the induction of labour.ConclusionIn the case of EAUVV, lesions are extremely rare, and it is very easy for thrombosis to form, which may result in the death of the child. When determining the next step in the treatment of the condition, the degree of UVV, possible complications, gestational age, foetal haemodynamics, and other relevant factors are strongly connected to the clinical therapy decision, and these factors should be considered comprehensively when making a clinical decision. We recommend close monitoring with hospital admission (to facilities capable of handling extremely preterm foetuses) after variability in delivery for worsening haemodynamic status.

Thrombosed umbilical vein varix in newborn with congenital syphilis.

Thrombosed umbilical vein varix in newborn with congenital syphilis.

Intra-abdominal umbilical vein varix in a neonate with polysyndactyly.

Intra-abdominal umbilical vein varix in a neonate with polysyndactyly.

VP20.06: Fetal intra‐abdominal umbilical vein varix: retrospective study and review of literature

To investigate the ultrasound characteristics, associations and obstetric outcome in pregnancies with intra-abdominal umbilical vein varix of the fetus. It was a retrospective cohort study conducted over two years i.e., from March 2019- February 2021. Data was collected from ultrasound records, antenatal and intra- partum records. For the neonatal outcome patients and respective consultants were followed up by telephonic calls. The data collected was demographic characteristics, varix characteristics (gestational age at diagnosis, size, progression, turbulence, thrombus formation, associated malformations and genetic), obstetric complications (fetal growth restriction/ IUFD) and neonatal outcome. Descriptive statistics (means, standard deviations, and percentages [%]) were calculated using SPSS, version 21.0 (SPSS Inc, Chicago, USA). Over the period of 2 years six cases of FIUVV were diagnosed making the incidence of FIUVV of 5/10000 pregnancies. Incidence of isolated FIUVV was 4.4/10000 cases. The mean gestational age at diagnosis was 25 weeks 3days with earliest diagnosis at 19w1d. Aneuploidy screening was done in five of cases with low risk for trisomy 21, 18 & 13 as outcome. The mean varix size was 11.85mm±2.28 mm (range 8.9mm-14.8 mm). No incidence of turbulence or thrombus formation within the varix was seen in the current study. One case had absent DV with anomalous connection of umbilical vein with IVC. One Perinatal death after planned LSCS at term & sudden IUFD at 33 weeks were noted in this study. Detailed anatomical scan and counselling should be done after FIUVV diagnosis. The fetal surveillance should be increased to look for major dilatation thombus or FGR. Longer analysis on the large population is needed to study the association of controls with the outcome. Also, long term studies are needed to for any delayed consequences affecting the adult life of these fetuses.

Fetal Intra-abdominal Umbilical Vein Varix Assessed by the Novel Doppler Ultrasound

Fetal Intra-abdominal Umbilical Vein Varix Assessed by the Novel Doppler Ultrasound

OC13.09: Intra‐amniotic umbilical vein varix: a case report and review of the literature

OC13.09: Intra‐amniotic umbilical vein varix: a case report and review of the literature

VP02.24: Umbilical vein varix: does it really need stringent monitoring?

Fetal intraabdominal umbilical vein varix (FIUVV) is a focal dilatation of the intraabdominal but extrahepatic portion of the umbilical vein. Here we present two cases where FIUVV was detected in the third trimester – one at 29 weeks and the other at 37 weeks. The detailed structural analysis was normal. Monitoring was done in the form of daily kick count, fortnightly antenatal visits and four weekly ultrasounds. We did not observe any increase in the size of the varix, turbulence in the flow or thrombosis during follow up scans. One was delivered at 37 weeks due to severe FGR and other at 38 weeks due to reduced fetal movements. FIUVV mandates a detailed anatomical survey. Invasive testing is recommended only if there are associated abnormalities. There is no or minimal increased risk of complications during pregnancy. The frequency of monitoring during pregnancy is still not established. Later the diagnosis of the varix, less frequent is the monitoring and better is the outcome. Supporting information can be found in the online version of this abstract Supporting Information Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Comparison of Doppler Indices in Patients With Umbilical Vein Varix (UVV) to Standard Doppler Indices [08O

INTRODUCTION: There is little consensus on antenatal management of umbilical vein varices (UVV). Our study reviewed biophysical profile (BPP) scores, Doppler indices, and maternal/neonatal demographics. METHODS: Following approval by our Institutional Review Board, we retrospectively reviewed singleton pregnancies with UVV from 1/1/2012 to 6/1/2019. Of those with Doppler studies, we compared middle cerebral artery (MCA), umbilical artery (UA), and ductus venosus (DV) values to the 50th percentile of standard indices. We reviewed BPP scores, fetal abnormalities, mode of delivery, maternal comorbidities, neonatal intensive care unit (NICU) admission, and APGARs. Basic descriptive statistics were conducted. RESULTS: Overall, 84 women were identified with UVV at an average gestational age of 32 weeks. Notably, 40.5% had used tobacco. Of the fetuses, 13.1% had intrauterine growth restriction, 6% tricuspid regurgitation, 31% anatomic abnormalities. No abnormal BPPs and fetal demises were identified. Sixty-two percent had an abnormal doppler value, with the MCA Doppler differing most from standard indices. Average gestational age at time of delivery was 37 weeks and the cesarean section rate was 26% with a minority due to non-reassuring fetal status (27%). There were three indicated preterm deliveries secondary to umbilical vein thrombus. For the neonates, 31% were admitted to the NICU. Only one had a 5-minute APGAR score &lt;5. CONCLUSION: Doppler abnormalities are common in pregnancies affected by UVV with MCA being the most commonly abnormal. None had abnormal BPPs and few required interventions such as indicated cesarean section due to fetal status. This raises the question: is antenatal testing and Doppler testing of any utility?

Lessons from Umbilical Vein Varix

ABSTRACTUmbilical vein varix is an uncommon vascular anomaly. The varix may be the initial presentation of an underlying congenital portosystemic shunt adversely affecting the fetus as we demonstrate in this case. To be able to label a varix as an isolated finding necessitates a thorough evaluation of the fetal venous system as well. Serial ultrasound of the fetus can help in detecting changes in the varix, thrombosis, growth restriction, as well as heart failure if there is another underlying venous anomaly. Early postnatal imaging is essential to exclude other associations to optimize the neonatal outcome. The prognosis is good if it is an isolated finding.How to cite this article:Sharma KA, Kour Isher H, Dadhwal V,et al. Lessons from Umbilical Vein Varix. Int J Infertil Fetal Med 2020;11(1):30–32.

Perinatal outcomes of fetal intra-abdominal umbilical vein varix: a multicenter cohort study

Objectives To investigate the perinatal outcomes of fetuses with antenatal diagnoses of intra-abdominal umbilical vein varix (UVV). Methods A multicenter retrospective study was conducted in four countries on fetuses diagnosed with intra-abdominal UVV between 2012 and 2019. Collected data included gestational age (GA), UVV maximum diameter at diagnosis and delivery, associated anatomical and chromosomal anomalies, birth weight, and neonatal outcomes. Results Twenty fetuses were identified, of which 20% had associated chromosomal and/or anatomical abnormalities, most resulting in poor outcomes (either intrauterine fetal death or pregnancy termination). Mean maternal age was 34.1 ± 7.0 years, UVV was diagnosed at 26.5 ± 4.5 weeks of GA on average with a maximum diameter of 12.0 ± 4.0 mm. Mean GA at delivery was 35.4 ± 5.6 weeks. Survival rate was 85%. Conclusion Our study shows a satisfactory outcome when intra-abdominal UVV is an isolated finding, with minimal obstetrical and perinatal consequences. The prognosis is worse when UVV is associated with other anomalies.

P05.04: Visualisation of intrahepatic portosystemic shunt and intra‐abdominal umbilical vein varix by fetal echocardiography with HDliveFlow imaging

P05.04: Visualisation of intrahepatic portosystemic shunt and intra‐abdominal umbilical vein varix by fetal echocardiography with HDliveFlow imaging

Antenatal sonographic features of persistent extrahepatic vitelline vein aneurysm confused with umbilical vein varix

Background: The persistent vitelline vein is a portal venous system malformation arising during the embryonic period. These abnormal blood vessels frequently thrombose and can lead superior mesenteric vein obstruction or portal hypertension. Case report: We visualized a fetal intra-abdominal cystic mass with turbulent flow on prenatal ultrasound at 28 weeks’ gestation. Initially diagnosed as an umbilical vein varix, it was later determined to be an extrahepatic persistent vitelline vein with an internal thrombus by postnatal ultrasound. It was successfully surgically excised. Conclusion: When an abnormal abdominal vascular structure near the umbilicus is found during prenatal ultrasonography, the persistent vitelline vein should be included in the differential diagnosis to allow prompt evaluation and treatment after birth.

Sonographic Evaluation of Umbilical Vein

To evaluate intraabdominal course of umbilical vein, its relation with portal vein and ductus venosus and its anomalies. All pregnant women were examined during midtrimester anomaly scan between January 2015 and December 2017. With evaluation of umbilical vein in its intraabdominal course, size of umbilical vein, its relation to the stomach and the gallbladder and origin of ductus venosus were studied in middle and upper abdominal axial and sagittal planes by two dimensional and colour doppler sonography. Anastomosis of umbilical vein with portal vein and its further course was examined and detailed anatomical survey was made to look for associated anomalies. Fetal and neonatal outcome was assessed after delivery. Twelve cases of umbilical vein abnormalities were evaluated. Of them, there were three cases of umbilical vein varix, four cases of persistent right umbilical vein and five cases of abnormal umbilical–portal–systemic venous shunt were noted. Examination of the intraabdominal course of umbilical vein and ductus venosus should be a part of second trimester anomaly scan. Proper knowledge of umbilical portal venous system is mandatory.

Is umbilical vein varix associated with changes in cerebroplacental ratio?

Objective: We aimed to review a single-center experience in follow-up and management of fetuses with umbilical vein varix (UVV) and to assess the effect of UVV on fetal Doppler parameters.Methods: We reviewed retrospectively maternal antenatal records, delivery records, and newborn records to identify cases of UVV. Further, we retrospectively compared 25 fetuses with isolated UVV and available cerebroplacental ratio (CPR) analysis with 75 matched controls.Results: We identified 67 cases of UVV. The median gestational age (GA) at diagnosis was 34 weeks (range: 26–41 weeks). The average diameter of UVV at diagnosis was 10.1 mm (range: 9–14 mm). The median GA at delivery was 36 + 6 (range: 33–41 weeks), with an average birth weight of 2918 g (range: 1278–4140 g). There was a single case of intrauterine death at 35 weeks. CPR was 2.13 ± 0.62 in isolated UVV group compared with 1.84 ± 0.61 in the control group (p < .05). Other Doppler parameters did not differ between fetuses with UVV compared with controls.Conclusions: CPR was significantly increased in the UVV group compared with control fetuses. This finding suggests that UVV is not associated with chronic fetal oxygen deprivation; it, therefore, may contribute to our understanding of the pathophysiology explaining abnormal pregnancy outcome in cases with UVV.

Perinatal Outcome of Fetal Umbilical Vein Varix: A Multicenter Cohort Study [29P

INTRODUCTION: Umbilical vein varix (UVV) is a focal dilation of the fetal umbilical vein. Clinical significance is not clearly established but it has been associated with adverse outcomes. This study investigates the clinical outcomes of UVV diagnosed during pregnancy. METHODS: We performed a multicenter retrospective (USA, Chile, Italy and Brazil) study by reviewing 19 fetuses diagnosed with UVV between 2012 and 2017. We investigated gestational age at diagnosis and at delivery, UVV maximum diameter, fetal gender, associated anatomical anomalies, intrauterine growth restriction, fetal demise, type of delivery, birth weight, Apgar scores. RESULTS: UVV was diagnosed at 26.4+/- 4.4 weeks of gestation. Maximum diameter of the defect at diagnosis typically was 13.1+/- 5mm. The majority of fetuses were male (n=12, 63%). Growth restriction was diagnosed in 26% (n=5) of the fetuses. Seventeen (89%) infants survived. Fetal demise occurred in 2 (11%) patients; one was associated with structural anomalies, and the other fetus did not have any other associated anomaly. Vaginal delivery was performed in 10 (53%) patients; median weight at birth was 2870 g (270-4960 g). Median Apgar scores were 8 and 9 on 1st and 5th minutes consecutively (ranging from 0 to 10). Genetic and anatomical anomalies were present in 21% (n=4) of the fetuses and included: karyotype 47,XY+13, cleft lip and palate, cerebellar hypoplasia, aortic hypoplasia, cardiomegaly, Blake's pouch cyst, ambiguous genitalia, campomelic dysplasia with SOX9 mutation. CONCLUSION: UVV is a rare congenital condition that is usually associated with low frequency of other structural/genetic anomalies and with good prognosis. ACKNOWLEDGEMENTS: Waldo Sepulveda, Edward Araujo Junior, Mari Charisse Trinidad, and Heron Werner

Case 253: Thrombosed Umbilical Venous Varix in an Infant

History A 3-month-old previously healthy girl presented to an outside institution with a 4-day history of low-grade fever, irritability, and a tender "knot" in the upper abdomen. Ultrasonography (US) was performed at an outside hospital. US images were not available for review; however, they showed a mass in the left hepatic lobe, per the outside report, and the patient was referred to our institution for further evaluation. Her parents reported a normal full-term pregnancy, with regular prenatal care and normal prenatal US findings. The baby was born after an uncomplicated gestation. She was delivered at term via an uncomplicated cesarean section due to a maternal history of cesarean section. The perinatal course was uncomplicated, and there was no history of umbilical catheterization, per the parents. On arrival at our institution, the patient had a temperature of 38.2°C. All other vital signs were normal. Palpation revealed a tender and firm mass in the periumbilical region; otherwise, physical examination findings were normal. Results of laboratory work-up were normal, except for elevated white blood cell count (26 600/mm3 [26.6 × 109/L]; normal, 6000-17 500/mm3 [6-17.5 × 109/L]). The patient underwent US followed by intravenous contrast material-enhanced (10 mL ioversol, Optiray 320; Medtronic, Santa Rosa, Calif) computed tomography (CT) on the same day.