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Prenatal diagnosis of fetal intra-abdominal umbilical vein varix: Report of 2 cases
Abstract
Fetal intra-abdominal umbilical vein varix (FIUVV) is a focal aneurysmal dilatation of the umbilical vein. Its clinical importance has not yet been clearly established, but it has been reported to be associated with increased fetal death rate (in nearly 44% of cases) and chromosomal abnormalities (in 12% of cases). We report 2 cases of FIUVV diagnosed via sonography in the third trimester.
... The incidence is low, ranging from 0.4 to 1.1/1000 [1]. It accounts for about 4% of the malformations of the umbilical cord in the fetus [2]. Must be distinguished two forms: the isolated FIUVV and that associated with other malformations, these two forms have a different prognosis. ...
... Current evidence supports the hypothesis that it is a developmental rather than a congenital malformation [8]. 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 [2]. ...
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 birth showed no anomalies. Despite a good prognosis it seems that a close monitoring is essential in antenatal period.
... 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]. ...
... Approximately 100 cases have been reported in the literature to date [4]. The anomaly is rarely diagnosed before 22 weeks of gestation, with a median gestational age at diagnosis of 27 weeks in the series reported by Sepulveda et al [2]. ...
... It is known that umbilical cord anomalies comprise 4% of all fetal malformations, and the prevalence of umbilical vein varix is estimated at 0.4-1.1 in 1000; extra-abdominal varices are even rarer than the intra-abdominal ones. 8,9 Cord varices are often diagnosed between 22 and 33 weeks of pregnancy in women with usually normal previous sonographic examination. 10 Some believe that these anomalies are developmental rather than congenital due to increased intraluminal pressure. ...
A euploid fetus in a partial molar pregnancy can develop umbilical cord abnormalities as pregnancy goes on. So, careful examination of the umbilical cord can determine fetuses at risk for ominous adverse effects. image
... In case of a dilated intra-abdominal umbilical vein, it should be necessary to perform a detailed morphological evaluation of the fetus, verifying the absence of major malformations, soft markers, cardiac anomalies. The ultrasound monitoring is always necessary for the surveillance of all the cases in which there is anemia or fetal distress [8]. İn this study, no accompanying additional anomaly was observed. ...
Fetal ıntra-abdominal umbilical vein (FIUV) varix is a rare and significant clinical situation that requires postnatal monitoring and highlighted by dilatation of the intrahepatic or additional hepatic portion of the intra abdominal section of the umbilical vein. In this case, we presented the fetal intra-abdominal extrahepatic umbilical vein varix at 35 weeks pregnant. There was no other accompanying anomaly and the patient was delivered vaginally of a normal appearing infant with no interventions.
... The fetal intra-abdominal umbilical vein varix (FIUV) is a rare condition characterized by focal dilatation of the umbilical vein of the fetus with a diameter which is at least 50% wider than the adjacent umbilical vein diameter, or an intra-abdominal umbilical vein segment dilated to ≥ nine mm [1,2]. Some authors have defined FIUV as a measurement that is more than two standard deviations above the mean for gestational age [3][4][5]. It is believed that the dilatation of the umbilical vein may result from a structural failure of the vessel's wall, given by a congenital weakness or by a progressive parietal thinning. ...
Fetal umbilical intra-abdominal vein varix (FIUV) is a rare congenital malformation characterized by focal dilatation of the umbilical vein. The authors report a case of pregnant woman at 32 weeks of gestation with a fetus affected by dilatation of an intra-abdominal portion of the umbilical vein. They performed continuous ultrasound and cardiotocographic monitoring, from admission to the delivery. They describe the case and perform a review of the literature.
... 6,8,9,11 Ante la alta frecuencia de muerte fetal intrauterina en las primeras series, se recomendó la monitorización fetal temprana, entre las 28 y 34 semanas y finalización del embarazo luego de alcanzada la madurez pulmonar. 12 A pesar de estos primeros trabajos, en la actualidad la mayoría de los autores reporta series con buenos resultados perinatales en las que recomiendan el seguimiento más estricto si aparecen cambios significativos en el diámetro de la dilatación. 5 No obstante, en términos generales se recomienda una conducta expectante, especialmente en los casos de variz de la vena umbilical intraabdominal aislada no complicados, que suelen representar alrededor de 70% del total. ...
Progress in echographic techniques, particularly, high-resolution echography and color Doppler, as well the higher deep on knowledge and systematization in fetal anatomy exploration, are the main responsible of the increased number of cardiovascular anomalies diagnosed prenatally.
To describe the sonographic findings and perinatal outcomes in cases with prenatal ultrasound diagnosis of intra-abdominal umbilical vein varix.
A descriptive and retrospective study of cases with prenatal ultrasound diagnosis of umbilical vein varix. The diagnosis is performed at the level of the abdominal circumference when the diameter of the vessel is above the established parameters for a certain gestational age. Variables concerning maternal-fetal features, ultrasound findings and perinatal outcomes of affected cases are described.
From August 2008 to August 2012 14 cases of intraabdominal umbilical vein varix were diagnosed in our center with a mean gestational age at diagnosis of 29 weeks. Of the 14 cases, 35% had associated anomalies, mostly cardiovascular anomalies. No chromosomal defects were detected. Perinatal outcomes in newborns were favorable, with mean gestational age at delivery of 38 weeks. Only one case of monochorionic-monoamniotic twin pregnancy required preterm elective termination secondary to a twin-to-twin transfusion syndrome.
Prenatal diagnosis of intra-abdominal umbilical vein varix should be followed by further studies given its possible association with other anomalies, chromosomal defects and cases of stillbirth. However, isolated cases of umbilical vein varix, representing a majority, often evolve favorably with few complications.
Objectives
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).
Methods
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 10 th 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.
Results
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 10 th 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 39 weeks in neither the cohort (38.7 weeks) nor the pooled literature (37.6 weeks).
Conclusion
An i‐FIUVV in singletons is not associated with an increased IUFD risk up to 39 weeks 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 10 th 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 39 weeks of gestation. After 39 weeks of gestation, data on fetuses with i‐FIUVV and their outcomes are lacking.
Las varices intraabdominales de la vena umbilical fetal son entidades poco comunes caracterizadas por la dilatación aneurismática focal. Representan aproximadamente 4% de las anomalías del cordón umbilical y se cree que son una anomalía del desarrollo más que una malformación congénita. Su importancia clínica aún no ha sido establecida claramente. El diagnóstico prenatal es realizado por ecografía convencional en la cual la lesión aparece como una estructura quística redonda o fusiforme dentro del abdomen fetal. Además, es útil para establecer el diámetro y la presencia de otras anomalías fetales asociadas. La evaluación Doppler permite identificar el tipo de flujo intralesional. El pronóstico perinatal es favorable cuando es identificada como un hallazgo aislado. No obstante, en aquellos casos asociados a otras alteraciones anatómicas / estructurales, la resultante fetal y neonatal es variable. Se presenta un caso de diagnóstico prenatal de varice intraabdominal de la vena umbilical fetal.
The 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
Generally the anomalies of the umbilical cord are rare; recently with the wide use of accurate ultrasound techniques and routine scanning for each pregnant women, it becomes possible to diagnose many rare cord anomalies; very rarely MRI is indicated to diagnose precisely some suspected cases; most of this cord anomalies are reported with twins and chromosomal anomalies. Some specific anomalies like cord cyst, haemangioma, knots and tumors were discussed before.
Umbilical vein varix (UVV) is a rare, idiopathic, focal dilatation of the umbilical vein, either within the intraamniotic portion of the umbilical cord or within the fetal abdomen. On ultrasound (US), UVV appears as a round or fusiform anechoic structure that is located either within the umbilical cord or within the fetal abdomen, inferior to the fetal liver and close to the anterior abdominal wall. Color flow, power Doppler, and pulsed wave Doppler US are useful to confirm the vascular nature of this lesion by showing venous flow in its lumen. Although the precise etiology of UVV is unknown, it has been speculated that any condition that increases venous pressure could potentially lead to dilatation of the extrahepatic portion of the umbilical vein because this anatomic region is the weakest area of umbilical circulation. Another suggested mechanism is an intrinsic weakness of the umbilical vein wall that subsequently leads to venous dilatation and the development of the varix. UVV is associated with a high rate of fetal anomalies and a potentially increased risk of fetal demise. The finding of a UVV should prompt a thorough search for other structural malformations and potentially aneuploidy testing. Serial growth measurements and antenatal surveillance are also recommended. There are currently no prenatal or postnatal treatment options for fetuses with UVV; however, due to the association with intrauterine fetal demise, early term delivery should be considered.
Importance:
Fetal umbilical vein aneurysm is an uncommon anomaly that accounts for approximately 4% of umbilical cord abnormalities. The rate of intrauterine fetal death is reported to be approximately 4% to 5%, higher than the background rate of 0.7% that is generally reported during pregnancy.
Objective:
The aim of this study was to review the pathophysiology, diagnosis, and clinical management of fetal umbilical vein aneurysm.
Evidence acquisition:
Advances in high-resolution ultrasound combined with color Doppler and 3-dimensional rendering have contributed to an increased understanding of the fetal venous circulation in recent years.
Results:
When the diagnosis of umbilical vein aneurysm is made, the patient should undergo a detailed ultrasound evaluation of the fetal anatomy, including fetal echocardiography, to exclude associated anomalies. Amniocentesis should be offered when other anomalies are found. Patients should be informed about the potential for an unfavorable outcome of pregnancy and should undergo close ultrasound surveillance to assess the size of the aneurysm, as well as any evidence of thrombosis or signs of hydrops.
Conclusions:
The main prognostic feature associated with a poor outcome of umbilical vein aneurysm seems to be the presence of other anomalies. Early diagnosis is associated with a somewhat worse prognosis, and most fetal deaths have been observed between 27 and 30 weeks of gestation. In the third trimester, it is reasonable to perform serial ultrasound examinations to assess fetal growth, the size of the aneurysm, and the blood flow pattern within the aneurysm.
Objective:
Fetal intraabdominal vein varix (FIUVV) is sonographic finding with unknown prevalence. We aimed to point out this particular abnormality and review possible associations and complications which may arise.
Method:
We performed an unrestricted literature search via PubMed and included all cases diagnosed with FIUVV.
Case presentation:
A 24-year-old, gravida 1 para 0 woman was referred to our clinic with possible diagnosis of FIUVV. We confirmed the diagnosis and detailed sonogram was normal. Beyond the gestational age of 32 weeks, intruterine growth restriction became evident. Close fetal surveillance was performed. We did not detect any thrombus formation within the varix or signs of cardiac decompansation during these visits. Delivery was planned after completion of 37 weeks. A healthy baby weighing 2100 gr was delivered and discharged without any complications.
Conclusion:
It is generally accepted that fetal anatomic survey is necessary after detection of FIUVV. karyotyping could be performed those cases associated with additional structural malformations. Close surveillance of fetal well being and growth is important. Possibility of thrombus formation within the varix should be kept in mind.
Umbilical vein varix (UVV) is defined as a focal enlargement of the umbilical vein and represents approximately 4% of fetal umbilical cord malformations. The reported neonatal outcome of fetuses with UVV varies widely due to its rarity, hence the small sample sizes of the case series in the literature. Earlier studies reported high fetal mortality, but more recent reports have demonstrated no association between UVV and intrauterine fetal death. A recent study has described a possible association between UVV diagnosed prenatally and child developmental delay. The present study of fetuses with UVV was done to evaluate and compare the levels of triple test serum biomarkers used for Down syndrome screening (human chorionic gonadotropin, α-fetoprotein, and unconjugated estriol) between a group of fetuses with uneventful obstetric outcome versus a subgroup of children with developmental delay.
Umbilical vein varix is a rare entity, which can lead to in utero fetal death. We report the case of a women diagnosed with umbilical vein varix on the 31st week of amenorrhea. A close follow-up and the early diagnosis of umbilical vein thrombosis have allowed the patient to give birth to a healthy newborn on the 34th week of amenorrhea. Improved ultrasound imaging as well as systematic study of fetal annexes lead to an early diagnosis of umbilical vein abnormalities. This allows a close follow-up and an early diagnosis of fetal life-threatening complications.
Copyright © 2014 Elsevier Masson SAS. All rights reserved.
Foetal intra-abdominal umbilical vein varix is rare. Colour Doppler ultrasonography helps distinguish this vascular anomaly. A detailed anatomic scan must be performed to exclude associated anomalies: forms associated with additional complications are found in 29 to 35% of the cases. Intra-uterine foetal demise (IUFD) is a complication of umbilical vein varix. However, recent studies are more reassuring. When foetal intra-abdominal umbilical vein varix is isolated, there is no reason to change the management of the pregnancy. Foetal sonographic follow-up is recommended, focusing on an increase in the size of the varix and the appearance of a clot. A particular clinical form, connecting the umbilicus to the extra-hepatic portal vein should be known, because of a high risk of thrombosis. On the basis of this finding, postnatal monitoring by ultrasound is necessary.
La dilatation de la veine ombilicale intra-abdominale (DVOA) est une pathologie rare du système veineux fœtal. Son diagnostic repose sur l’échographie Doppler couleur. Un bilan morphologique fœtal complet est nécessaire afin d’éliminer la présence d’autres lésions, des formes associées étant retrouvées dans 29 à 35 % des cas. Parmi les complications pouvant survenir au cours de la grossesse, la mort fœtale in utéro (MFIU) a, dans les travaux récemment publiés, une fréquence inférieure à ce qui avait été rapporté initialement. Dans les formes isolées, elle ne justifie pas de modifier la prise en charge obstétricale. La surveillance échographique reste cependant recommandée au cours du troisième trimestre à la recherche d’une majoration de la dilatation et surtout de l’apparition d’un thrombus. Une forme clinique particulière, liée à un abouchement anormal de la veine ombilicale dans le système porte infra-hépatique, doit être individualisée en raison d’un risque élevé de thrombose. Elle justifie la réalisation d’une échographie abdominale dans les premiers jours de vie.
Objectives:
The current study aims were to assess the long-term outcomes of children who were diagnosed with umbilical vein varix (UVV) prenatally.
Methods:
The study included fetuses with UVV diagnosed in the community between the years 2005 and 2011. They all have been refereed to our Ultrasound Unit for diagnosis' confirmation. This has been conducted locally by a single operator. After delivery, they were matched by gestational age at birth with a set of newborns from a random list. Developmental delay was assessed by telephone interview using a questionnaire based on Ages and Stages Questionnaire. If the child's score (both in the study and control group) was below the cut-off in one or more domain(s), the families were offered an examination by a child developmental health care team.
Results:
There was no perinatal mortality in both groups. A low-development score was found in 41.7% (15/36) and 3.7% (4/108) in UVV and control group, respectively (P < 0.05). In 10 out of 15 (67%) children in the UVV group with low scores, formal developmental assessment was performed. Four (40%) were diagnosed having developmental delay. Among the four controls with low score, two families refused additional assessment and one child died. The remaining child was found to have normal development.
Conclusions:
A possible association between UVV diagnosed prenatally and child developmental delay was found. However, the clinical implications of these findings are still premature; thus, additional studies are needed.
Objective:
Varix of the fetal intra-abdominal umbilical vein (VFIUV) has been reported to be associated with an increased risk of adverse perinatal outcome and especially with intra-uterine fetal demise (IUFD). Induction of preterm birth, as early as 32-34 weeks gestation has been suggested to minimize this risk. We aimed to evaluate our center experience with the antenatal diagnosis of VFIUV and review the relevant literature.
Methods:
This is a retrospective case series of all cases (between 2004 and 2009) where the sonographic antenatal diagnosis of VFIUV was registered at any gestational age (GA). Ultrasound, maternal and newborn electronic medical records were used. Descriptive statistics were employed as appropriated and correlation coefficient (r) calculated.
Results:
We identified 24 women with fetuses, with isolated VFIUV (excluding one lost-to-follow-up). GA at diagnosis was 30.5 ± 4.4 weeks; 13 (56.5 %) cases were diagnosed <32 weeks. The mean VFIUV diameter was 13 ± 2.9 (range 9-20) mm and turbulent flow was reported in 7 cases (30.4 %). GA at birth was 37 ± 2.5 weeks. The small for gestational age rate was 4 % (1/23), while no case of IUFD occurred. The group induction of labor rate was 65.2 %, while 43 % (10/23) due to the diagnosis of VFIUV alone: 17 % (4/23) preterm and 26 % (6/23) at term. The cesarean rate was 17 % (4/23) and NICU admission was required for five neonates (21.7 %). The preterm induction of birth was related to a significantly increased risk for cesarean and neonatal morbidity (p = 0.015; p = 0.029, respectively). The mode of delivery was not associated with the GA at diagnosis, size/type of flow of VFIUV (r = 0.101; r = 0.727; r = 0.671, respectively) overall (r) = 0.4. All fetuses were live-born with normal follow-up at 2-60 months.
Conclusion:
Isolated VFIUV has a favorable perinatal outcome at term, unrelated to the structural and flow characteristics of VFIUV. We show that follow-up for growth abnormalities with no preterm induction of birth is a safe maternal and neonatal approach.
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.
To present our experience with fetuses with umbilical vein varix (UVV), to investigate possible risk factors and to suggest a management scheme of evaluation.
A study of 14 pregnancies complicated with isolated UVV was performed. Data collected included sonographic characteristics of the UVV, pregnancy outcome including induction of labour, mode of delivery, birthweight, and neonatal complications.
UVV was diagnosed at a median gestational age of 27.5 weeks' gestation (range: 22-34 weeks). The average diameter of the UVV at diagnosis was 10.6 mm (range: 8-15 mm), and the maximal diameter during follow-up was 12.8 mm (range: 10-18 mm). The median gestational age at delivery was 36.1 weeks (range: 34-40 weeks), with an average birthweight of 2834 g (range: 1725-3715 g). Five women underwent emergent cesarean section. In fetuses with turbulent flow in the UVV there was a tendency to larger maximal sizes of the UVV, earlier gestational age at delivery and smaller birthweight. There were no cases of fetal or neonatal demise.
We suggest that fetuses with UVV should be followed weekly from diagnosis to 28 weeks, and twice a week afterwards. Induction of labour should be considered at 36-37 weeks' gestation or at signs of fetal distress.
To assess the clinical significance of varix of the intraabdominal portion of the umbilical vein, we reviewed 10 cases diagnosed prenatally by ultrasonography at a median gestational age of 27 weeks. A comprehensive anatomic survey and serial follow-up scans were performed in each case. All three fetuses with associated anomalies died in utero, and prenatal karyotyping revealed that two of them had a chromosomal abnormality. In six of the seven cases with structurally normal fetuses the pregnancy proceeded uneventfully, and no neonatal complications were attributed to the umbilical vein varix. Our experience and the review of the literature revealed 42 cases with information on fetal outcome. Overall, 24% of the fetuses died, 12% had a chromosomal abnormality, and 5% developed hydrops. We conclude that fetuses with varix of the intrafetal umbilical vein should be considered at risk for poor outcome. However, if no other anomalies are present, the prognosis is generally good.
We report a case of aneurysm of the umbilical vein, causing fetal death at 41 weeks gestation. We conclude that these aneurysms are a complication of congenital thinning of the vessel wall and want to emphasize that in stillbirths the cause of death may only be revealed by careful placental examination, including the umbilical cord.
Varix of the fetal intra-abdominal umbilical vein (FIUV) is a rare entity. We describe an ultrasound diagnosis of this condition together with a review of the literature relating to its prognosis and management. Our conclusion is that close fetal monitoring should be performed, and delivery should be induced when lung maturity has been accomplished or any fetal distress is apparent.
Previous studies of umbilical vein varix diagnosed prenatally have been small, and the results have been contradictory. We wanted to determine whether prenatally diagnosed umbilical vein varix is associated with an increased risk of fetal anomalies or poor perinatal outcomes. We identified all cases of fetal intra-abdominal umbilical vein varix diagnosed on the basis of prenatal ultrasonography at Brigham and Women's Hospital between 1988 and 1998. Cases were reviewed to determine the presence of other sonographic findings as well as pregnancy and neonatal outcomes. We identified 25 cases and included those 23 for which follow-up was available. In 11 cases (48%), pregnancies and neonatal outcomes were normal, with full-term delivery, appropriate birth weight, and no evidence of anomalies. Three cases (13%) had preterm deliveries, and 1 had Kell isoimmunization requiring postnatal transfusion. In the remaining 8 cases (35%), structural anomalies were present. One fetus had a chromosomal abnormality (69,XXX). Prenatal diagnosis of fetal umbilical vein varix appears to be associated with a high rate of fetal anomalies. Detection of an umbilical vein varix should prompt a thorough examination of the fetus, including a fetal survey and echocardiogram. Isoimmunization should be ruled out, and consideration of karyotyping should be discussed if other anomalies are present.
Dilatation of the intra-abdominal portion of the fetal umbilical vein is a rare abnormality. We describe here a new case diagnosed at 31 weeks of gestation and discuss the clinical features and management of this abnormality.
Fetal intra-abdominal umbilical vein (FIUV) varix-a focal dilatation of the umbilical vein-is an uncommon entity. Outcome varies from spontaneous resolution to fetal death. We describe here the experience of our center with this perplexing entity.
Case series review of seven cases of isolated FIUV treated in a tertiary care center from 2000 to 2003.
We describe seven cases of isolated FIUV varix with varying natural history, follow-up strategies, and fetal outcome. In one of the cases, intrauterine fetal demise occurred despite close follow-up.
We suggest that the course of FIUV is unpredictable, with a high fetal mortality rate. Close fetal monitoring with early delivery at 34 weeks' gestation should be advocated.
To evaluate sonographic appearance, natural history, and neonatal outcome of fetal venous anomalies.
We performed an observational study, including all fetuses affected by abnormalities of the venous system diagnosed by ultrasound during the prenatal period.
26 fetuses were identified. Other malformations were present in 5 cases (19.2%), 1 fetus had trisomy 21, and 1 fetus had intrauterine growth retardation (IUGR). Twenty-five pregnancies ended in liveborn infants, and there was 1 case of unexplained intrauterine death in the fetus with IUGR affected by varix of the umbilical vein.
Fetal venous anomalies are very rare and may be associated with fetal malformations or IUGR. Conservative management appears to be an adequate medical practice in the absence of other fetal problems, but in the presence of a varix of the umbilical vein, serial follow-up scans are needed to exclude the onset of hydrops or thrombosis of the varix.
To assess the clinical significance of fetal intra-abdominal umbilical vein (FIUV) varix.
We reviewed all cases of FIUV varix diagnosed in a university hospital from 1994 to 2003 and searched the English literature for cases of prenatal diagnosis of FIUV varix. The FIUV was considered dilated when the measurements were above 2 SD of the mean for gestational age. Cases reported in the literature were included if they met the diagnostic criteria for FIUV varix.
Between 1994 and 2003, 13 fetuses were diagnosed in our hospital as having FIUV varix. Review of the literature revealed an additional 80 cases. Fetal outcome was available for analysis in 91 cases. Additional sonographic abnormalities were detected prenatally in 29 cases (31.9%), most commonly anomalies of the cardiovascular system (including structural and functional abnormalities), hydropic features and anemia. There were nine (9.9%) cases of chromosomal abnormalities. All except one had associated sonographic abnormalities. There were 12 (13%) perinatal losses. Only 54 cases (59.3%) of fetuses with FIUV varix had a normal obstetric outcome. In the 62 cases with isolated FIUV varix, there were five unexplained intrauterine deaths (8.1%) occurring between 29 and 38 weeks of gestation. The incidence of complications, which included intrauterine death, thrombosis of the umbilical vein and abnormal antenatal cardiotocogram, were significantly higher (P = 0.01, Fisher's exact test) if the diagnosis of FIUV varix was made before 26 weeks.
FIUV varix is associated with a high incidence of fetal anomalies and obstetric complications. Detailed sonography is necessary to exclude fetal anomalies. Karyotyping should be offered when additional fetal abnormalities are detected. Intensive surveillance including color Doppler ultrasound should be started from the moment of diagnosis until delivery, especially in those cases presenting early in pregnancy.
Aneurysm of the umbilical vein: case report and review of the literature
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