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Year : 2022  |  Volume : 11  |  Issue : 1  |  Page : 55-57

Umbilical cord with two umbilical veins: A report of two cases

Department of Paediatrics, Andhra Hospitals, Vijayawada, Andhra Pradesh, India

Date of Submission05-Aug-2021
Date of Decision20-Sep-2021
Date of Acceptance21-Sep-2021
Date of Web Publication03-Jan-2022

Correspondence Address:
Bhoojata Gondi
Department of Paediatrics, Andhra Hospitals, Vijayawada, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.jcn_100_21

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Umbilical cord with two umbilical veins is a rarely reported anomaly. Four-vessel cord is known to be associated with other congenital anomalies. Second umbilical vein is usually due to persistence of right umbilical vein. We report two cases of two umbilical veins; one newborn with four-vessel umbilical cord with two umbilical veins, who presented with severe pulmonary arterial hypertension, and another newborn with three-vessel umbilical cord with two umbilical veins, diagnosed to have transposition of great arteries. The presence of two umbilical veins mandates comprehensive workup to rule out other congenital anomalies. However, as highlighted by our first case, the presence of two umbilical veins is not always an ominous sign but could be seen in otherwise normal baby.

Keywords: Congenital anomalies, transposition of great arteries, umbilical veins

How to cite this article:
Gondi B, Laxman S, Rao Paturi VR, Maram KP. Umbilical cord with two umbilical veins: A report of two cases. J Clin Neonatol 2022;11:55-7

How to cite this URL:
Gondi B, Laxman S, Rao Paturi VR, Maram KP. Umbilical cord with two umbilical veins: A report of two cases. J Clin Neonatol [serial online] 2022 [cited 2022 Jan 16];11:55-7. Available from: https://www.jcnonweb.com/text.asp?2022/11/1/55/334727

  Introduction Top

Abnormalities of the umbilical cord related to the number of vessels are not uncommon. Although single umbilical artery, which is the most common umbilical cord anomaly, a four-vessel cord with two umbilical veins is not infrequent in the literature.[1] There are sporadic reports of umbilical cord with two umbilical veins associated with other congenital anomalies. However, the presence of two umbilical veins does not always translate into sinister implications for the baby.[2]

  Case Reports Top

Case 1

A term, female neonate, weighing 2.8 kg, was born by elective cesarean section. The maternal health was uneventful and no risk factors were noted. The baby developed respiratory distress and expiratory grunt after 2 h of life. On arrival to the neonatal unit at 6 h of life, she had severe respiratory distress (respiratory rate 64/min), saturating 84% at room air. Her chest X-ray showed mild cardiomegaly and bilateral patchy infiltrates. Echocardiogram of the heart revealed severe pulmonary arterial hypertension. She was intubated and ventilated for respiratory failure, and umbilical vessels were cannulated for invasive monitoring of arterial oxygen tension and blood pressure as well as administration of parenteral fluids and medications. Close inspection of the umbilical cord showed four vessels, of which two were thin-walled veins, confirmed by blood gas oxygen saturation and by the nonpulsatile back flow, and by the chest and abdominal X-ray [Figure 1]a and [Figure 1]b. There were two thick-walled vessels, of which one was confirmed as umbilical artery based on blood gas oxygen saturations and pulsatile back flow of blood and by the check X-ray. The other thick-walled vessel presumed to be an artery could not be cannulated. She had no other congenital anomalies on workup (cranial ultrasound and abdominal ultrasound).
Figure 1: (a) Chest and abdominal X-ray showing course of two umbilical veins, marked by blue arrows. (b) Umbilical cord showing two veins (blue arrows) and two arteries (red arrows)

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The baby made full recovery after supportive therapy (intravenous fluids, prophylactic antibiotics) and oral sildenafil. A follow-up echocardiogram of the heart after 2 weeks showed normal pulmonary pressures.

Case 2

A 5-day-old, term, male infant, born to a healthy primi mother by normal vaginal delivery had presented with respiratory distress and cyanosis. Maternal health was uneventful without any risk factors. At presentation to the neonatal unit, the baby had an oxygen saturation of 74% at room air and had mild tachypnea (respiratory rate 65/min). He had good central and peripheral pulses and had no murmur on auscultation of the heart. Subsequent workup diagnosed him with transposition of great arteries (TGA). Umbilical vessels were cannulated for parenteral administration of fluids and blood gas monitoring. Inspection of the umbilicus revealed a three-vessel cord, with two umbilical veins and one umbilical cord, diagnosed based on blood gas, pulsatality of blood flow on transducer tracing [Figure 2]a and [Figure 2]b. His cranial ultrasound and abdominal ultrasound were negative for other congenital anomalies.
Figure 2: (a) Chest and abdominal X-ray showing course of two (overlapping) umbilical veins, marked by blue arrows. (b) Umbilical cord showing two veins (blue arrows) and one artery with catheter in situ (red arrow)

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He had an emergency balloon atrial sepstostomy and was discharged to the pediatric cardiology ward for arterial switch procedure.

  Discussion Top

Single umbilical artery is the most common umbilical abnormality reported in the literature.[1] Four-vessel cord with two umbilical veins is reported in few cases and is known to be associated with congenital anomalies.[3],[4],[5] Around 4th week of gestation, the sinus venosus receives blood from the right and left umbilical veins. However, during development, the right umbilical vein obliterates and disappears by 7 weeks of gestation[6] leaving the left umbilical vein connected to the left portal vein in the fetal liver, transporting all the blood. When the right umbilical vein remains open, it carries oxygenated blood to the heart. It may coexist with the left umbilical vein as an intrahepatic supernumerary structure.

A persistent right umbilical vein (PRUV) usually replaces the normal left umbilical vein or is supernumerary.[6] The etiological factors for PRUV include first trimester folic acid deficiency, teratogens such as retinoic acid and, early obstruction or occlusion of the left unbilical vein from external pressure. PRUV is associated with congenital malformations in around 18% of cases such as duodenal atresia, imperforate anus, malrotation, annular pancreas, atrial septal defect, and situs inversus.[5],[7] A literature review noted only seven previously reported cases of a four-vessel cord.[8],[9] In the four-vessel umbilical cord abnormality, the presence of three umbilical arteries is the most common abnormality.[1] However, the most common cause of two umbilical veins is PRUV. Four-vessel cord is associated with a variety of congenital anomalies such as total anomalous venous connection, atrioventricular septal defect, hypertrophic cardiomyopathy trisomy-18, bilateral cleft lip and palate, bifid liver, and malrotation of small bowel.[7] It is considered difficult to diagnose an abnormal number of umbilical vessels in the antenatal scans, even if other congenital abnormalities coexist. While a single umbilical artery can be easily detected by the presence of only one umbilical artery lateral to the fetal bladder, the presence of supernumerary umbilical veins is overlooked, as it is often difficult to check the number of umbilical veins throughout the length of the umbilical cord.

  Conclusion Top

Presence of two umbilical veins is not uncommon and often associated with other congenital anomalies. Our case report highlights the association of two umbilical veins with TGA and also with a neonate with pulmonary hypertension who is otherwise well. The presence of two umbilical veins is not always associated with a poor prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Vrabie SC, Novac L, Manolea MM, Dijmarescu LA, Novac M, Siminel MA. Abnormalities of the umbilical cord congenital anomalies - From the embryo to the neonate. IntechOpen 2018;513:345-62.  Back to cited text no. 1
Pérez-Cosio C, Sheiner E, Abramowicz JS. Four-vessel umbilical cord: Not always a dire prognosis. J Ultrasound Med 2008;27:1389-91.  Back to cited text no. 2
Krzyżanowski A, Swatowski D, Gęca T, Kwiatek M, Stupak A, Woźniak S, et al. Prenatal diagnosis of persistent right umbilical vein - Incidence and clinical impact. A prospective study. Aust N Z J Obstet Gynaecol 2019;59:77-81.  Back to cited text no. 3
Quaak MS, Draaisma JM. A four-vessel umbilical cord. Arch Dis Child Fetal Neonatal Ed 2017;102:F115.  Back to cited text no. 4
Singh N, Rao S, Sobti P, Khurana N. Multiple vessels in the umbilical cord: A report of four cases. Indian J Pathol Microbiol 2012;55:597-8.  Back to cited text no. 5
[PUBMED]  [Full text]  
Rodriguez MA. Four-vessel umbilical cord without congenital abnormalities. South Med J 1984;77:539.  Back to cited text no. 6
Walala BD, Ngeleza NO, Ntakwinja MB, Bosunga KG, Mukengere MD. Partially four-vessel umbilical cord: About a diagnosed case at Panzi Hospital. Int J Reprod Contracept Obstet Gynecol 2020;9:450-2.  Back to cited text no. 7
Murdoch DE. Umbilical-cord doubling. Report of a case. Obstet Gynecol 1966;27:555-7.  Back to cited text no. 8
Kaufmann HJ, Weisser K. Neonatal transumbilical angiography: A preliminary repost. Ann Radiol (Paris) 1964;10:437-44.  Back to cited text no. 9


  [Figure 1], [Figure 2]


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