Anomalies of the Fetal Venous System: A Report of 26 Cases and Review of the Literature

Viora, E.; Sciarrone, Andrea; Bastonero, S.; Errante, G.; Mortara, G.; Chiappa, Enrico; Campogrande, M

doi:10.1159/000078997

Cited by 29 publications

(22 citation statements)

References 25 publications

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“…Patients with FIUVV should be closely monitored with sonography and should be informed about the possible adverse outcomes. 1,7,8 Delivery should be induced at 34-36 gestational weeks, when the fetal lung is mature. …”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of fetal intra‐abdominal umbilical vein varix: Report of 2 cases

İpek

Kurt

Tosun

et al. 2007

J of Clinical Ultrasound

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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. 1,2 With the development of sonography and Doppler sonography, diagnosis has become easier than in the past.The clinical importance of FIUVV 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).1-3 We report 2 cases of FIUVV diagnosed via sonography in the third trimester. CASE REPORTS Case 1A 31-year-old healthy woman, gravida 2, para 1, was referred to our radiology department for sonographic examination. Her first pregnancy was normal. A second-trimester sonographic examination at another institution was unremarkable. The examination was performed with a Voluson 730 Expert (GE Medical Systems, Milwaukee, WI) equipped with a 2-5-MHz curved-array probe. A normal intrauterine fetus of nearly 29 weeks' gestational age was present according to the patient's last menstrual period and sonographic measurements. The biparietal diameter was 69 mm, the femur length was 55 mm, and the abdominal circumference was 248 mm. The estimated fetal weight was 1,300 g with normal amniotic fluid index, umbilical artery, and uterine artery pulsatility index. However, intra-abdominal umbilical vein varix with transverse diameter of 11 mm over an 18-mm segment was diagnosed near the anterior abdominal wall ( Figure 1A). Color Doppler analysis also showed turbulent flow in the varicose segment ( Figure 1B). Otherwise, the fetus was structurally normal. The patient declined chromosomal investigation. Weekly sonographic monitoring was recommended, but the patient did not return for follow-up examinations. She delivered a healthy girl weighing 2,600 g via cesarean section peformed at 35 gestational weeks at another institution. Case 2A 29-year-old woman, gravida 1, para 0, was referred for sonographic examination at approximately 36 weeks' gestation according to her last menstrual period and sonographic measurements. The patient had a second-trimester examination at another institution that was reportedly

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Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of fetal intra‐abdominal umbilical vein varix: Report of 2 cases

İpek

Kurt

Tosun

et al. 2007

J of Clinical Ultrasound

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“…The recent advent of modern, high‐resolution ultrasound machines combined with color Doppler has enhanced our ability to detect and describe venous anomalies in utero 1–5. Although rare, UPVS anomalies are often associated with structural anomalies, in particular cardiac defects6, as well as various genetic syndromes, such as Noonan and Goldenhar syndromes7, 8, and associations with aneuploidy and chromosomal aberrations have been found2, 9. The association with Down syndrome was first reported in 1992 by Kieran et al 10, who described a newborn with trisomy 21 and a direct communication between the right portal vein and the inferior vena cava (IVC).…”

Section: Introductionmentioning

confidence: 99%

Umbilical vein anomaly in fetuses with Down syndrome

Achiron

Gindes

Gilboa

et al. 2010

Ultrasound in Obstet & Gyne

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“…Persistence of the right umbilical vein in the fetus is a variant of the usual intra‐abdominal umbilical venous connection. The estimated prevalence of an intrahepatic persistent right umbilical vein is 1 per 786 births 1 – 9 . However, this number may be underestimated in populations that do not undergo targeted sonographic examinations, as the variation in anatomy can be subtle.…”

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confidence: 99%

Intrahepatic Persistent Right Umbilical Vein and Associated Outcomes

Lide

Lindsley

Hale

et al. 2016

J of Ultrasound Medicine

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The aim of this study was to provide a comprehensive review of the current data surrounding an intrahepatic persistent right umbilical vein in the fetus, including associated anomalies and outcomes, and to assist practitioners in counseling and management of affected pregnancies. We performed a MEDLINE, Embase, Cochrane Central Register of Controlled Trials, and Northern Light database search for articles reporting outcomes on prenatally diagnosed cases of a persistent right umbilical vein. Each article was independently reviewed for eligibility by the investigators. Thereafter, the data were extracted and validated independently by 3 investigators. A total of 322 articles were retrieved, and 16 were included in this systematic review. The overall prevalence of an intrahepatic persistent right umbilical vein was found to be 212 per 166,548 (0.13%). Of the 240 cases of an intrahepatic persistent right umbilical vein identified, 183 (76.3%) were isolated. The remaining cases had a coexisting abnormality, including 19 (7.9%) cardiac, 9 (3.8%) central nervous system, 15 (6.3%) genitourinary, 3 (1.3%) genetic, and 17 (7%) placental/cord (predominantly a single umbilical artery). In summary, a persistent right umbilical vein is commonly an isolated finding but may be associated with a coexisting cardiac defect in 8% of cases. Therefore, consideration should be given to fetal echocardiography in cases of a persistent right umbilical vein.

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Anomalies of the Fetal Venous System: A Report of 26 Cases and Review of the Literature

Cited by 29 publications

References 25 publications

Prenatal diagnosis of fetal intra‐abdominal umbilical vein varix: Report of 2 cases

Prenatal diagnosis of fetal intra‐abdominal umbilical vein varix: Report of 2 cases

Umbilical vein anomaly in fetuses with Down syndrome

Intrahepatic Persistent Right Umbilical Vein and Associated Outcomes

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