Fetal Intra-abdominal 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.

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).

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

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

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.

Fetal intra-abdominal umbilical vein varix: retrospective cohort study and systematic review and meta-analysis.

To investigate the ultrasound characteristics and outcome of pregnancies with fetal intra-abdominal umbilical vein varix (FIUVV). Cases of FIUVV managed at our tertiary university hospital over an 8-year period were reviewed. Information retrieved included gestational age and diameter of the umbilical varix at diagnosis, increase in varix diameter, associated ultrasound or chromosomal anomalies and pregnancy outcome. Furthermore, a systematic review and meta-analysis of series of FIUVV in the literature was performed to assess the incidence of chromosomal anomalies, small-for-gestational age infants and intrauterine fetal demise (IUFD), and to pool odds ratio (OR) estimates on the relationship between the incidence of these outcomes and the presence of additional associated ultrasound anomalies. Thirteen cases of FIUVV were included in the cohort study. Additional ultrasound anomalies were found in two (15.4%) of 13 cases. One case of IUFD was observed and no case of chromosomal anomaly or thrombosis of varix was recorded. A total of five studies comprising 254 cases met the inclusion criteria of the systematic review. FIUVV was associated with additional ultrasound anomalies (non-isolated FIUVV) in 19% (95% CI, 10.9-29.1%) of cases. No case of chromosomal abnormality or IUFD was reported in fetuses with isolated FIUVV. In contrast, in the group of non-isolated FIUVV, the incidence of chromosomal anomalies was 19.6% and that of IUFD was 7.3%, with ORs of 14.8 (95% CI, 2.9-73.0) and 8.2 (95% CI, 1.05-63.1), respectively, when compared with the group of isolated FIUVV. When isolated, the outcome of cases affected by FIUVV is usually favorable. In about 20% of cases, additional ultrasound anomalies are found, which are associated with an increased risk for chromosomal abnormalities and IUFD. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Fetal Intra-abdominal Umbilical Vein Varix: A Case Report and Literature Review

Fetal intra-abdominal umbilical vein (FIUV) varix is a rare malformation of the umbilical cord. This is a critical situation due to discrepancies in outcomes varying from normal to high rates of complications and fetal mortalities. We report the observation of a FIUVV vein diagnosed precociously at 22 weeks with a quiet increasing of the diameter by 31 weeks. The outcome was favourable and close monitoring after birthshowed no anomalies. Despite a good prognosis it seems that a close monitoring is essential in antenatal period.

Intra-Abdominal Umbilical Vein Varix – A Case Report

AbstractThe incidence of the fetal intra-abdominal umbilical vein varix condition is very rare and has been associated with fetal hydrops, IUGR and still birth A 26-year-old primigravida was referred for routine antenatal scan. The scan at 30 weeks showed an intra-abdominal ovoid structure superior to the fetal bladder. Color flow Doppler revealed venous flow in continuity with the umbilical vein. A diagnosis of umbilical varix was made. The venous flow was present throughout the lesion, suggesting the absence of thrombi. There was no evidence of fetal hydrops. Subsequent scans at regular intervals showed no increase in size of the umbilical varix. The patient had an uneventful elective cesarean section at 39 weeks. Postnatal assessment and a follow-up neonatal cardiac echo scan were normal. Our case supports the new emerging evidence that pregnancy outcome in cases of isolated fetal umbilical vein varix is generally good. Caution must be exercised against unnecessary early induction and costly preterm births

Fetal Intra-abdominal Umbilical Vein Varix Complicated with Patent Ductus Venosus and Atrial Septal Defect

Umbilical vein varix has diverse clinical features and an unpredictable course during the pregnancy and/or perinatal period. We report a rare case of isolated fetal varix of the intra-abdominal umbilical vein, which was associated with fetal cardiomegaly. After birth, the umbilical vein varix remained with continuous blood flow through the patent ductus venosus. In addition, persistent cardiomegaly was complicated with an atrial septal defect.

Clinical characteristics and outcomes of antenatal fetal intra-abdominal umbilical vein varix detection.

ObjectiveThis study reviewed clinical characteristics of fetal intra-abdominal umbilical vein (FIUV) varices that were detected during antenatal ultrasound examinations.MethodsBetween January 2006 and January 2012, 121 cases of FIUV varices were detected and 7 cases were lost to follow-up. We retrospectively reviewed the medical records of 114 patients and neonates.ResultsFrom a total 96,553 ultrasound examinations in 43,995 pregnancies, 121 cases of FIUV varices were identified (2.8 per 1,000 pregnancies). Gestational age at diagnosis was 32.0 ± 2.9 weeks (range, 20.1-36.3 weeks), the mean diameter of the FIUV varix was 12.6 ± 2.1 mm (range, 8.0-21.0 mm) at initial diagnosis and the mean maximal diameter was 13.1 ± 2.3 mm (range, 8.0-21.0 mm) during follow-up. The most severe pregnancy complications included one case of intrauterine fetal death and another case of fetal hydrops. Associated fetal anomalies (n = 11, 9.6%) detected by ultrasonography included bilateral renal pelvis dilatation, ventriculomegaly, cryptorchidism, incomplete renal duplication and pulmonary sequestration. A total of 104 cases (91.2%) were delivered at term and 10 cases (8.8%) were preterm deliveries before 37 weeks of gestation.ConclusionFIUV varices that are not associated with fetal anomalies based on ultrasound examination during prenatal care have favorable pregnancy outcomes. Nevertheless, close fetal monitoring is recommended to decrease perinatal complications.

Placental mesenchymal dysplasia associated with androgenetic/biparental mosaicism

Placental mesenchymal dysplasia associated with androgenetic/biparental mosaicism

Fetal intra-abdominal umbilical vein varix: a case report

The incidence of the fetal intra-abdominal umbilical vein varix condition is very rare. Its significance in pregnancy is still unknown, but it has been associated with fetal hydrops, IUGR and still birth. However, in most isolated cases, the outcome is good. A 31-year-old primigravida was referred to the antenatal clinic at 28 weeks of gestation as her uterus was measuring small for gestational age. Her anomaly scan at 20 weeks was normal. The scan at 28 weeks showed growth below the lower 10th centile with normal liquor volume and umbilical Doppler wave velocity. However, an intra-abdominal ovoid structure measuring 28 mm × 10 mm was seen superior to the fetal bladder. Color flow Doppler revealed venous flow in continuity with the umbilical vein. A diagnosis of umbilical varix was made. The venous flow was present throughout the lesion, suggesting the absence of thrombi. There was no evidence of fetal hydrops. Subsequent scans at 32, 34 and 36 weeks showed no increase in size of the umbilical varix and progressive fetal growth, although growth remained below the lower 10th centile with normal Doppler results and liquor volume. The patient had an uneventful emergency cesarean section at 37 weeks for failed induction of labor and reduced fetal movements. Postnatal assessment and a follow-up neonatal cardiac echo scan were normal. Our case supports the new emerging evidence that pregnancy outcome in cases of isolated fetal umbilical vein varix is generally good. Caution must be exercised against unnecessary early induction and costly preterm births.

Three- and four-dimensional sonographic diagnosis of fetal intra-abdominal umbilical vein varix.

Fetal intra-abdominal umbilical vein (FIUV) varix is a rare sonographic finding of a focal dilatation of the umbilical vein. Diagnostic criteria are as follows: an FIUV varix diameter [50 % wider than the adjacent umbilical vein’s diameter, and an intra-abdominal umbilical vein diameter [9 mm [1]. We present antenatal three(3D) and fourdimensional (4D) sonographic diagnoses of FIUV varix. A 30-year-old pregnant Japanese woman, gravida 1, para 0, was referred to our ultrasound clinic at 20 weeks and 3 days of gestation due to a sonographic finding of bilateral choroid plexus cysts. A thorough two-dimensional (2D) sonographic anatomical scan confirmed the presence of bilateral choroid plexus cysts, but there were no other abnormalities. Anatomical re-evaluation at 27 weeks and 3 days revealed the disappearance of the previously noted choroid plexus cysts. However, a dilatation of the intraabdominal portion of the umbilical vein (11.5 mm) was identified between the fetal abdominal wall and inferior edge of the liver. 3D power Doppler (3D HD-flow) with the glass-body mode (Voluson E8; GE Medical Systems, Milwaukee, Wi, USA; curved array transabdominal transducer, 2–8 MHz) revealed the widening and course of the FIUV varix and spatial relationships between the FIUV varix and surrounding vessels (Fig. 1). The 3D inversion mode highlighted the presence of the dilated portion of the vessel in comparison with the adjacent normal segment (Fig. 2). Several follow-up examinations were conducted until term, and a decrease in the size of the cystic portion to a normal diameter was confirmed. A healthy female infant weighing 3,648 g was delivered vaginally at 41 weeks and 3 days of gestation, with an umbilical artery pH of 7.229, and an Apgar score of 7/9 at 1 and 5 min, respectively. No cord or other abnormalities were identified. Image reconstruction of the intra-abdominal anechoic mass positively identified the diagnostic criteria for FIUV varix in the present patient. The 3D/4D color/power Doppler modality, even though diagnostically useful, effectively supports the sonographic diagnosis. However, 2D sonography is difficult to interpret in some cases based on the characteristics of varices. In the abdominal area, this vascular anomaly can be differentiated from other cystic lesions using 2D color/power Doppler sonography [1]. 3D/ 4D color/power Doppler clarified the entire varix and affected vein’s shape and contour. The 3D inversion mode provides data on the anatomy and pathology of fetal fluidfilled structures (digital casts) not possible with standard 2D sonography [2]. This, to our knowledge, is the first report of a FIUV varix imaged using the 3D inversion mode as a sonographic adjunct to 3D/4D color/power Doppler. 3D/4D technology has allowed sonologists to more effectively visualize fetal structures employing multiplanar reconstruction, not previously achievable with conventional 2D sonography. The additional information it yields aids physicians and couples by supporting the diagnosis, allowing better visualization, and improving understanding of and counselling for fetal anomalies. It must be remembered that 3D/4D technology is a supplementary modality, whose use does not ultimately alter the diagnosis, outcome, or counseling provided. B. S. Austria U. Hanaoka M. Sato T. Hata (&) Department of Perinatology and Gynecology, Kagawa University School of Medicine, 1750-1 Ikenobe, Miki, Kagawa 761-0793, Japan e-mail: toshi28@med.kagawa-u.ac.jp

Four cases of fetal intra-abdominal umbilical vein varix: A single centre's approach to management

Fetal intra-abdominal umbilical vein (FIUV) varix is a rare prenatal abnormality characterised by a focal intrahepatic or extrahepatic dilatation of the intra-abdominal portion of the umbilical vein. Usually, it is an isolated finding, but in some cases it can be associated to other fetal anomalies. Thrombosis is a possible complication of FIUV varix and it can lead to poor fetal or neonatal outcome. We describe four consecutive cases of FIUV varix diagnosed in our Unit and managed with low-dose aspirin (LDA) prophylaxis until the 35th week of gestation. None of the fetuses developed thrombosis of the varix and the neonatal outcomes were good in all the cases.

Intra-abdominal Umbilical Vein Varix Thrombus

Fetal intra-abdominal umbilical vein varix (FIUVV) is an uncommon finding with poorly understood clinical significance. Numerous fetal complications are associated with this anomaly. This case report demonstrates FIUVV in which thrombus develops leading to early induction of labor for successful delivery. The literature regarding FIUVV is reviewed and discussed with implications for sonographic follow-up during pregnancy and patient management.

Ultrasonographic Evidence of Intra-Abdominal Umbilical Vein Dilatation: Is It a True Varix?

Fetal intraabdominal umbilical vein (FIUV) dilatation, or varix, is a rare ultrasonographic (US) finding of focal dilatation of the umbilical vein. This article describes FIUV tortuosity in cases with suspected varix and provides ultrasonographic criteria for its diagnosis. Cases of suspected FIUV varix referred to our unit for final diagnosis and follow-up were studied. Each woman underwent comprehensive US evaluations that included basic grayscale scan and color Doppler scan. In 12 singleton pregnancies, primary grayscale scan confirmed FIUV dilatation. Supplementary color Doppler scans, however, revealed linear bidirectional blood flow and FIUV tortuosity in all cases. Color Doppler scans did not depict true FIUV dilatations or varix but rather a tortuous course of the vein. A normal pregnancy outcome can be expected in these cases.

Two‐ and three‐dimensional sonographic diagnosis of fetal intra‐abdominal umbilical vein varix: A case report

Fetal intra-abdominal umbilical vein varix is a rare condition characterized by focal dilatation of the umbilical vein of the fetus. We report a case of fetal intra-abdominal umbilical vein varix associated with additional sonographic abnormalities and detected at 31 weeks' gestation. Several follow-up sonographic examinations were performed, and the prenatal findings were confirmed on postnatal ultrasound. The diagnosis was facilitated by the use of three-dimensional power Doppler sonography.

Another "double bubble" sign or "double bladder" sign: fetal intra-abdominal umbilical vein varix.

Fetal intra-abdominal umbilical vein varix increases the incidence of fetal/neonatal morbidity and mortality. Its early diagnosis may be clinically important. For a case of this disorder, we have proposed the new name of "double bladder" sign; "another double bubble" sign may also illustrate this disorder.

Isolated Fetal Intra-Abdominal Umbilical Vein Varix

This series describes a single center's experience in follow-up and management of fetuses with an isolated fetal intra-abdominal umbilical vein varix. All cases with a fetal intra-abdominal umbilical vein varix that were diagnosed or referred to our medical center over 15 years were followed and managed. The definition of a fetal intra-abdominal umbilical vein varix used was a segment dilated to 9 mm or greater or at least 50% wider than the diameter of the adjacent umbilical vein. Over the 15-year period, our center had approximately 65,000 births with 28 cases of isolated fetal intra-abdominal umbilical vein varices: a prevalence rate of 1 case per 2300 births. Three of the 28 cases (10.7%) had intrauterine growth restriction. Five of 30 fetuses (17%) showed turbulent flow in the varix. We had no cases of intrauterine fetal death, and 27 of the 28 neonates had good outcomes. In contrary to earlier reports, we found that when a fetal intra-abdominal umbilical vein varix is isolated, a good fetal outcome is expected. On the basis of our experience, we have changed our policy and do not recommend inducing preterm labor. Nevertheless, close fetal surveillance until delivery is warranted.

Favorable Obstetric Outcome in a Fetus Diagnosed With Umbilical Vein Varix at 22 Weeks' Gestation

Umbilical vein varix (UVV) is a rare diagnosis during early gestation, and most cases diagnosed early have a poor obstetric outcome. We report a case of intraabdominal extrahepatic UVV diagnosed at 22 weeks of gestation by ultrasonography, with a favorable obstetric outcome. This case shows that early diagnosis of UVV during pregnancy does not necessarily correlate with poor outcome. Pregnancy termination is thus not the only option, and an affected pregnancy can be carried to term under close monitoring if there are no other associated anomalies. Variceal dilatation of the umbilical vein is rare and accounts for approximately 4% of malformations of the umbilical cord [1]. UVV has been defined variously; Sepulveda et al [2] defined fetal intra-abdominal UVV (FIUVV) as an intra-abdominal umbilical vein diameter at least 1.5 times greater than the diameter of the intrahepatic umbilical vein, while Allen et al [3] defined the anomaly as an intra-abdominal umbilical vein diameter exceeding 9 mm. Syphilis, degenerative changes, decreased resistance due to icterus, and congenital thinning have been proposed as etiologies of FIUVV. The most likely etiology and the only pathologic finding in most cases is thinning of the vessel wall near the anterior abdominal wall due to intrinsic weakness of the umbilical vein wall [4]. FIUVV is associated with a fetal death rate of up to 44%, and with karyotype abnormalities in 12% of cases (trisomy 21, 18 and 9, and triploidy 69,XXX). Structural malformations and hydrops fetalis have been diagnosed in up to 35% of cases each [2,5,6]. Our case of UVV is unique in having a favorable obstetric outcome, despite being diagnosed at an early gestational age of 22 weeks. A 24-year-old primigravida attended a routine antenatal ultrasound at 22 weeks of gestation. A cystic structure (15 × 14 mm) was detected in the fetal abdomen, just beneath the abdominal wall at the level of liver (Figure A). Color Doppler examination revealed it to be vascular in nature with the umbilical vein leading to it, suggesting a diagnosis of extrahepatic FIUVV (Figure B). Doppler examination allowed differentiation of UVV from other cystic lesions, including choledochal cyst, liver cyst, urachal cyst and mesenteric cyst. A careful scan failed to detect any other associated anomalies. Fortnightly ultrasound scans were performed to monitor growth of the fetus until 34 weeks’ gestation. During this period, there was no change in the size of the cyst. The patient was temporarily lost to follow-up. She was then reported again after a normal term delivery by a trained nurse at home. The perinatal period was uneventful. There was no family history of any similar anomaly or any features of isoimmunization. Because of the absence of any associated anomalies, karyotyping was not performed. Ultrasound examination at 3 months was normal. The child was healthy after 1 year. UVV presents in approximately 4% of malformations of the umbilical cord [1], and is more common in the umbilical cord than in the fetus [7]. In intraabdominal UVVs, extrahepatic varices are more common than intrahepatic ones [6]. Approximately 100 cases have been reported in the