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.     

Fetal intra-abdominal umbilical vein varix: what is the clinical significance?

OBJECTIVE: To assess the clinical significance of fetal intra-abdominal umbilical vein (FIUV) varix. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.     

Varix of the fetal intra-abdominal umbilical vein: comparison with normal.

To compare the normal extrahepatic portion of the fetal intra-abdominal umbilical vein (FIUV) with varix of the FIUX, we prospectively measured the diameter of the FIUV in 150 uncomplicated second and third trimester pregnancies and compared these results with retrospective review of nine fetuses with varix of the FIUV as an isolated prenatal sonographic finding. The diameter of the normal FIUV increases linearly from approximately 3 mm at 15 menstrual weeks to approximately 8 mm at term (R = 0.92). The nine fetuses with FIUV varix had a FIUV diameter 6 to 12 standard deviations above the mean for age. Four (44%) of the nine fetuses with FIUV varix subsequently died, including one with trisomy 21. One of the remaining fetuses developed severe hydrops 2 weeks after the initial detection of the FIUV varix. FIUV varix appears to carry an increased risk of adverse fetal outcome, including fetal demise.     

Outcome of pregnancy after prenatal diagnosis of umbilical vein varix.

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.     

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.     

Fetal prognosis in varix of the intrafetal umbilical vein.

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.     

Umbilical cord morphologic characteristics and umbilical artery Doppler parameters in intrauterine growth-restricted fetuses.

OBJECTIVE: To compare prenatal morphometric changes of umbilical cord components in intrauterine growth-restricted fetuses with and without abnormal umbilical artery Doppler parameters. METHODS: Consecutive singleton intrauterine growth-restricted fetuses at a gestational age of older than 20 weeks were compared with matched appropriate-for-gestational-age fetuses. Intrauterine growth restriction was defined in the presence of a sonographic abdominal circumference below the 5th percentile for gestational age at the time of sonography and a birth weight below the 10th percentile. The sonographic examination included pulsed Doppler measurements of the umbilical artery resistance index and measurements of the umbilical cord cross-sectional area and the umbilical cord vessel area. RESULTS: A total of 84 intrauterine growth-restricted fetuses and 168 appropriate-for-gestational-age fetuses were included in the study. All umbilical cord components (umbilical cord cross-sectional area, vein area, artery area, and Wharton jelly area) were smaller in the intrauterine growth-restricted fetuses. The prevalence of lean umbilical cords (cross-sectional area < 10th percentile for gestational age) was significantly higher in intrauterine growth-restricted fetuses compared with appropriate-for-gestational-age fetuses (73.8% versus 11.3%; P < .0001). A significant and progressive reduction of the umbilical vein area corresponding to the degree of umbilical artery Doppler parameter abnormality was found. The umbilical artery area was not related to the hemodynamic changes of the blood flow in the umbilical arteries. CONCLUSIONS: The proportion of lean umbilical cords was higher in intrauterine growth-restricted fetuses than in appropriate-for-gestational-age fetuses. Umbilical vein caliber decreases significantly with worsening of umbilical artery Doppler parameters.     

Umbilical vein blood flow in growth-restricted fetuses.

OBJECTIVE: To determine whether umbilical blood flow is reduced in a subset of growth-restricted (IUGR) fetuses when expressed as flow per kilogram or flow per unit of specific sonographic fetal measurements. DESIGN: Prospective. SUBJECTS: Thirty-seven IUGR fetuses were examined by Doppler ultrasound within 4 h of the last non-stress test prior to delivery. This population was divided into three groups of varying clinical severity according to the characteristics of umbilical arterial pulsatility index (PI) and heart rate. METHODS: Absolute and weight-specific umbilical vein (UV) flow were calculated from measurements of UV diameter and UV mean velocity. Umbilical vein diameter, velocity and UV flow were calculated also per unit head (HC) or abdominal circumference (AC) and correlated with gestational age. RESULTS: Umbilical vein flow (UVf) per kilogram fetal weight was significantly lower in the more severe IUGR fetuses (abnormal umbilical arterial PI) than in normally grown comparable fetuses (P < 0.001). Umbilical vein flow per unit HC was significantly lower in the three groups (P < 0.001) than in the control population. The UV diameter/HC ratio was normal whereas UV velocity/HC ratio was significantly lower in IUGR fetuses than in comparable controls. CONCLUSIONS: The present study clearly establishes that umbilical venous blood flow is reduced in IUGR fetuses on a weight-specific basis. The sonographic growth parameter which best distinguishes umbilical flow differences of IUGR fetuses from normal fetuses is the head circumference.     

Imaging of the ductus venosus in neonates: from patency to closure.

OBJECTIVE: To familiarize the radiologist with the variable sonographic appearance of the involuting ductus venosus in neonates. METHODS: Five sick neonates ranging in age from 24 to 42 weeks had abdominal sonographic examinations to check for intra-abdominal diseases. RESULTS: Doppler sonography showed a patent ductus venosus in 3 neonates and a ductus venosus with thrombosis in 2 neonates. The ductus venosus was identified in the liver between the left portal vein and the inferior vena cava. In 2 patients, follow-up sonography showed that the ductus venosus progressively had thrombosis and then disappeared. CONCLUSIONS: The ductus venosus in a neonate has a variable sonographic appearance depending on its stage of involution. Its detection in a sick neonate can lead to inadvertent diagnosis of a hepatic varix, an abscess, or a tumor.     

Cystic lymphangiomatosis with severe intra‐abdominal bleeding in a newborn: Case report

We report the case of a newborn girl with intestinal cystic lymphangiomatosis who presented with abdominal distension and intra‐abdominal bleeding following a prenatal ultrasound diagnosis of intestinal anomaly. Postnatal abdominal ultrasound revealed disseminated submucosal and intramural cystic dilatations of various sizes in the bowel and intestinal lymphangiomatosis was diagnosed. The presence of severe bleeding diathesis and widespread disease led to conservative treatment. The patient died on postnatal day 7 and postmortem examination confirmed cystic lymphangiomatosis. Detection of intestinal hyperechogenicity and/or dilatation in prenatal ultrasonography and the persistence of these findings during pregnancy are suggestive for pathologies such as meconium ileus, meconium peritonitis, and intestinal atresia. Although rare, intestinal lymphangiomatosis should be kept in mind in patients whose prenatal sonographic findings persist until birth. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2013     

Fetal umbilical vein varix: sonographic appearance and postnatal outcome.

We describe five cases of dilation or varix of the umbilical portion of the left portal vein appearing as a fetal intra-abdominal cyst. Doppler or color flow examination, or both, indicated umbilical venous flow within the cystic area in each case. Only one fetus exhibited transient cardiomegaly, which resolved before birth. This fetus was the only one discovered to have the varix as early as the second trimester. All five infants were delivered at term without sequelae.     

Prenatal sonographic diagnosis of persistent right umbilical vein with varix.

Persistence of the right umbilical vein is variably associated with life-threatening malformations of the fetal genitourinary, gastrointestinal, cardiac, and skeletal systems. Varices of the intraabdominal umbilical vein, although not necessarily associated with other malformations, do seem to carry a risk of fetal mortality, especially if they are large or appear early during the pregnancy. We report the prenatal diagnosis of the rare combination of persistence and varix of the right umbilical vein in an otherwise healthy infant.     

超声对胎儿永存右脐静脉的诊断价值

目的 探讨超声对胎儿永存右脐静脉的诊断价值.方法 回顾分析我院诊断的3例胎儿永存右脐静脉的灰阶及彩色多普勒超声表现及相关临床情况.结果 3例胎儿均可见胆囊位于腹部正中,脐静脉连接门脉右支,门静脉窦凹面朝向胃泡.其中1例合并颅内其他异常并在出生后死亡,另2例不合并其他异常,出生半年后生长发育未见异常.结论 胎儿永存右脐静脉具有特征性超声表现,不合并其他畸形时预后良好,早期检出并给予适当的遗传咨询具有重要的临床意义.     

Prenatal diagnosis of fetal intra-abdominal umbilical vein varix: report of 2 cases

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.     

Ultrasound detection of unusual spontaneous portosystemic shunts associated with uncomplicated portal hypertension

Seven cases of unusual spontaneous portosystemic shunts observed by ultrasonography in the last 8 months are reported, including cases of coronary vein varicocele and patent umbilical vein; two cases of spleno-retroperitoneal anastomosis; omphalo-ilio-caval anastomosis; superior mesenteric vein-inferior vena cava anastomosis; spleno-renal anastomosis; and spleno-portal anastomosis and anastomosis from the splenic vein to the abdominal wall. One of these collateral vessels was also analyzed by pulsed Doppler flowmetry. The patients were either cirrhotic or had pre-hepatic portal hypertension (resulting from chronic pancreatitis) and gave no history of gastrointestinal bleeding or ascites. Two of these patients had previously undergone surgery for problems associated with cholestasis. In both cases, presurgical sonographic studies were used to guide the surgical procedures in the hope of preserving the anomalous connections. Furthermore, ultrasound detection of spontaneous portosystemic shunts was an important factor in interpreting the clinical symptoms of these patients.     

Persistent right umbilical vein: incidence and significance.

OBJECTIVES: To conduct a prospective evaluation of the incidence and neonatal outcome of fetuses with persistent right umbilical vein. This condition had traditionally been considered to be extremely rare and to be associated with a very poor neonatal prognosis, but later evidence has raised some doubts about the veracity of these contentions. METHODS: Between August 1995 and November 1998, 8950 low-risk patients were prospectively evaluated at two medical centers. The sonographic 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 gall bladder was located medially to the umbilical vein. RESULTS: Persistent right umbilical vein was detected in 17 fetuses during the study. Four of them had additional malformations, of which three had been detected antenatally. CONCLUSIONS: We established that the incidence of persistent right umbilical vein 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 echocardiography. When the persistent right umbilical vein is connected to the portal system and other anomalies are ruled out, the prognosis can generally be expected to be favorable.     

Sister Mary Joseph Nodule

A Sister Mary Joseph nodule represents a cutaneous metastasis into the umbilicus. This clinical sign of intra‐abdominal malignancy is frequently overlooked or misinterpreted by both patients and their physicians. We report 4 patients with a Sister Mary Joseph nodule. The umbilical metastases appeared sonographically as hypoechoic masses with irregular margins and small internal hyperechoic foci. Further evaluation revealed disseminated malignancy, and the umbilical nodule was just “a tip of an iceberg.”     

Large cross-sectional area of the umbilical cord as a predictor of fetal macrosomia.

OBJECTIVE: To determine whether a large cross-sectional area of the umbilical cord is a predictor of fetal macrosomia. METHODS: Consecutive patients of > 34 weeks' gestation, who presented for sonographic examination and who delivered within 4 weeks of the examination, were included in the study. The sonographic cross-sectional areas of the umbilical cord, the umbilical vessels and the Wharton's jelly were measured in a free loop of the umbilical cord. Logistic regression analysis was used to determine significant predictors of macrosomia (actual birth weight > 4000 g and > 4500 g). Fetal biometric parameters (biparietal diameter, abdominal circumference and femur length), sonographic estimated fetal weight and umbilical cord area > 95(th) centile for gestational age were used as covariates. RESULTS: During the study period, 1026 patients were enrolled. Fifty-three (5.2%) newborns had a birth weight > 4000 g, and 22 (2.1%) weighed > 4500 g. The proportion of cases with a large umbilical cord was significantly higher in the group of macrosomic compared with non-macrosomic infants (54.7% vs. 8.7%, P < 0.0001). Multiple regression models demonstrated an independent contribution of the large cord in the prediction of birth weight > 4000 g and > 4500 g (odds ratio (95% CI), 20.6 (9.2-45.9) and 4.2 (1.2-17.7), respectively). The sensitivity, specificity and positive and negative predictive values of a sonographic large umbilical cord were 54.7%, 91.3%, 25.4%, and 97.4%, respectively. The combination of abdominal circumference > 95(th) centile and large cord predicted 100% of macrosomic infants. The proportion of umbilical cords with a Wharton's jelly area > 95(th) centile for gestation was significantly higher in macrosomic fetuses of diabetic compared with non-diabetic mothers. CONCLUSIONS: Sonographic assessment of umbilical cord area may improve the prediction of fetal macrosomia.     

多层螺旋CT在评价门脉海绵样变侧支血管中的应用

目的利用64层螺旋CT增强扫描和血管重建对门脉海绵样变的解剖关系及侧支血管的形成情况进行评价。方法对我院2004年8月-2005年5月期间收集的18例门脉海绵样变资料进行分析。结果在门脉闭塞后,引起相应血管的纡曲扩张及侧支血管的形成,其中有门脉周围形成门门侧支循环,胆囊壁静脉的曲张,胃网膜静脉的增粗纡曲,胆管壁静脉的纡曲扩张,腹膜后椎体旁侧支血管的开放,脾肾静脉短路的开放,胃底食管静脉的曲张。结论多层螺旋CT血管重建在诊断门脉海绵样变方面有重要的临床意义。     

Sonographic diagnosis of tumor thrombus formation in the umbilical vein: a case report

There are few reports of tumor thrombus formation in the umbilical vein. We report a case in which sonographic examination revealed tumor thrombus formation in the reopened umbilical vein in a patient with hepatic cirrhosis, primary liver cancer, and portal hypertension.