Prenatal diagnosis of abnormalities of the fetal venous system

Hofstaetter, C.; Plath, H.; Hansmann, M.

doi:10.1046/j.1469-0705.2000.00066.x

Cited by 92 publications

(75 citation statements)

References 27 publications

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“…In the extra hepatic variant, the right umbilical vein drains into the systemic circulation and bypasses the liver. The DV is then absent [7][8][9][10][11][12].…”

Section: Embryological Backgroundmentioning

confidence: 99%

Persistent Right-Sided Umbilical Vein and Azygos Continuity associated with Ophthalmological and Pulmonary Malformations. Prenatal Diagnosis of an Exceptional Case

Lepage

Capelle

Emonts

et al. 2014

Gynecol Obstet (Sunnyvale)

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We report on the case of a fetus in whom a prenatal ultrasound performed at 22 weeks of gestation allowed the diagnosis of a double anomaly of the systemic venous return, with a persistent right umbilical vein and the agenesis of the inferior vena cava with azygos continuity.The fetus showed also a large umbilical hernia and a right sided microphtalmia. Fetal growth was normal. Genetic anomalies were excluded by karyotype and CGH array. Cesarean section was performed at 39 weeks of gestational age for breech presentation. The neonate adapted well. Post-natal examination confirmed pre-natal diagnosis. Thoraco-abdominal CT-scan performed at 8 days of age showed additional right upper lobe bronchial atresia with relative hypoplasia of the right pulmonary artery and right hepatic lobe hypotrophy. It also showed a short segmental infra diaphragmatic aplasia of the vena cava with dilation of the azygos vein running along the homolateral diaphragmatic coupola that drained into superior vena cava and malposition of the mesenterial vessels without any intestinal malrotation. Anomalies of the fetal cardinals and umbilical veins are the result of early abnormal venous system development in the embryo, the etiology of which is unknown. Usually, abnormal systemic venous return only affects one vessel. The presence of a double anomaly as described in this case is exceptional.A persistent right umbilical vein is reported in 0.2 to 0.4% of antenatal screening. The variant with intrahepatic transition is the most frequent and isolated form. The exceptional, form with hepatic bypass, is commonly part of a polymalformative syndrome. Agenesis of the inferior vena cava (IVC) with azygos continuity is a rare anomaly (0,2-3% of antenatal screening). Its significance relates to its frequent association with complex congenital heart diseases. The prognosis of anomalies of cardinals and umbilical veins depends on the presence of associated cardiac and extra cardiac malformations that might impact prenatal counseling and parental decision to continue or interrupt pregnancy. Careful repeated pre and post-natal evaluation is necessary to exclude polymalformative syndrome despite of normal molecular genetic examination.

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“…In the extra hepatic variant, the right umbilical vein drains into the systemic circulation and bypasses the liver. The DV is then absent [7][8][9][10][11][12].…”

Section: Embryological Backgroundmentioning

confidence: 99%

Persistent Right-Sided Umbilical Vein and Azygos Continuity associated with Ophthalmological and Pulmonary Malformations. Prenatal Diagnosis of an Exceptional Case

Lepage

Capelle

Emonts

et al. 2014

Gynecol Obstet (Sunnyvale)

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“…The umbilical cord arteries and vein are unlike their counterparts in the remainder of the foetal body as the umbilical cord vein transports oxygenated blood to the foetal heart 22 while the arteries return oxygen‐depleted blood to the placenta. The walls of the umbilical cord artery lack an internal and external elastic lamina and the adventitia found in other arteries is replaced by mucous connective tissue.…”

Section: Umbilical Cord Structurementioning

confidence: 99%

“…, 30 Venous system development results in the obliteration of the right umbilical vein and the development of the ductus venosus between the left umbilical vein and the inferior vena cava (IVC) 22 …”

Section: Umbilical Cord Structurementioning

confidence: 99%

The development, structure and blood flow within the umbilical cord with particular reference to the venous system

Spurway

Logan

Pak

2012

Australas J Ultrason Med

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The umbilical cord is a helical and tubular blood conduit connecting the foetus to the placenta. The umbilical cord achieves its final form by the 12th week of gestation and normally contains two arteries and a single vein, all embedded in Wharton's jelly. The structure of the umbilical cord receives only a cursory glance during many obstetric ultrasound examinations: with imaging limited to documenting the number of vessels within the cord and the insertion sites at the foetus and placenta. Extensive research into blood flow characteristics of the umbilical cord arteries has been undertaken and is now widely applied in contemporary ultrasound practice. In contrast, investigation of umbilical vein blood flow is only instigated in instances of foetal compromise when the spectral waveform of the ductus venosus and pulsations in the vein are scrutinised. The current level of ultrasound imaging of the umbilical vein demonstrates a lack of appreciation and knowledge about a structure that is crucial to sustaining foetal life.The goal of this review is to increase awareness of the importance of the umbilical cord. In addition, this review will provide an information platform for undertaking and critically analysing research into the umbilical cord by providing a summary of cord embryology, structure, foetal venous circulation and mechanisms of blood flow within the umbilical cord vein.

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“…The pathogenesis of CAPV may be attributed to excessive involution of the peri-intestinal vitelline venous loop [10,13,22,69] , or to total failure of the vitelline veins to establish the critical anastomosis with hepatic sinusoids [4] . Behind the abnormalities, the initiative event may be referred to genetic mutation or chromatosome variation as CAPV has been sometimes reported in conjunction with chromosomal disorders [70] , such as translocation (2,10) [21] and turner syndrome (45, XO) [6,71] . The associated extrahepatic portosystemic shunts may occur due to the persistent subcardinohepatic anastomosis with the vitelline veins.…”

Section: Cardiovascular Systemmentioning

confidence: 99%