What are the other Names for this Condition? (Also known as/Synonyms)

• Supernumerary Umbilical Cord Vessels

What is Four Vessel Umbilical Cord? (Definition/Background Information)

• The placenta is an organ that connects the developing fetus to the uterine wall. It is a disc-shaped reddish-brown structure that connects the fetus to the mother through the umbilical cord
  • The normal function of placenta is to supply nutrients and oxygen to the fetus from the mother’s blood and remove wastes from the fetal body
  • The umbilical cord contains two umbilical arteries and one umbilical vein, providing a connection between maternal and fetal blood circulation
• In Four Vessel Umbilical Cord, there is either an extra umbilical artery or umbilical vein. The condition is also known as Supernumerary Umbilical Cord Vessels. It is thought to arise from persistent embryonic vein (persistent right umbilical vein) or vitelline vessels (vessels in the ovum)
• In some cases, Four Vessel Umbilical Cord occurs as an isolated abnormality. However, sometimes, it may be associated with certain congenital conditions including heart defects, such as ectopia cordis and tetralogy of Fallot, pulmonary valve stenosis, cleft lip and palate, liver malformation, situs inversus, and gastroschisis
• Complications of Four Vessel Umbilical Cord are usually observed in the background of the associated congenital conditions and defects. In such cases, a close monitoring of the pregnancy is important, followed by treatment of the congenital associated abnormalities
• Currently, there are no preventative measures available for Four-Vessel Umbilical Cord. However, the prognosis is generally good when Four-Vessel Umbilical Cord is not associated with any congenital abnormalities

Who gets Four Vessel Umbilical Cord? (Age and Sex Distribution)

• Only a few cases of a Four-Vessel Umbilical Cord have been reported worldwide in medical literature and the condition is extremely rare
• Women who are pregnant with both male and female fetuses can be affected
• No racial or ethnic preference is noted

What are the Risk Factors for Four Vessel Umbilical Cord? (Predisposing Factors)

There are no known risk factors for Four-Vessel Umbilical Cord; but, in some cases, it may be associated with the following congenital defects:

• Bifid liver
• Cleft lip and palate
• Ectopia cordis
• Gastroschisis
• Pulmonary valve stenosis
• Situs inversus
• Tetralogy of Fallot

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Four Vessel Umbilical Cord? (Etiology)

The exact cause of formation of Four-Vessel Umbilical Cord is unknown. It is reported that an abnormal development of the umbilical cord and its vessels during pregnancy may be a possible cause. The condition is also known as Supernumerary Umbilical Cord, since two arteries and two veins are noted.

• An extra vein occurs when there is a failure of the right umbilical vein to involute during the sixth week of pregnancy
• An extra artery occurs during the third week when there is an abnormality known as allantoic artery development

What are the Signs and Symptoms of Four Vessel Umbilical Cord?

• Four-Vessel Umbilical Cord is associated with a variety of fetal congenital anomalies that may be revealed on a prenatal imaging scan
• The signs and symptoms are dependent on the associated congenital anomalies

How is Four Vessel Umbilical Cord is Diagnosed?

It is reported that isolated cases of Four-Vessel Umbilical Cord are often difficult to diagnose. In general, the diagnosis may involve the following tests and exams:

• Physical examination along with an evaluation of one’s medical history: The healthcare provider may check for any signs of fetal distress such as non-reassuring fetal heart rate
• Prenatal abdominal ultrasound may reveal the condition and other associated fetal abnormalities, if any
• Amniocentesis to detect chromosomal anomalies
• A careful examination of the placenta by a pathologist after birth of the child, to macroscopically (gross visual exam) and microscopically analyse the placental tissue may help establish a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Four-Vessel Umbilical Cord?

Most isolated cases (cases that are not observed in a background of congenital abnormalities) of Four-Vessel Umbilical Cord are not known to present any significant complications. However, if the condition is present with associated congenital abnormalities, the following may be noted:

• When persistent right umbilical vein is associated with absence of ductus venosus, it may cause volume overload and severe hemodynamic effects leading to fetal hydrops
• In terminal cases, certain complications, such as polyhydramnios (increased amniotic fluid), cardiomegaly (heart enlargement), and fluid accumulation, may occur

It is more common for the extrahepatic type of persistent right umbilical vein to have associated anomalies than the intrahepatic type of persistent right umbilical vein.

How is Four Vessel Umbilical Cord Treated?

• The treatment depends on whether Four Vessel Umbilical Cord is an isolated occurrence or is noted against a background of other congenital anomalies
• A careful and periodic monitoring of the pregnancy is necessary, if Four-Vessel Umbilical Cord is associated with certain congenital anomalies, which may sometimes lead to hemodynamic stress in the fetus

Treatment of associated abnormalities may be necessary on a case by case basis.

How can Four Vessel Umbilical Cord be Prevented?

• Currently, there are no definitive methods available to prevent Four Vessel Umbilical Cord
• A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Four Vessel Umbilical Cord? (Outcomes/Resolutions)

• The outcome of Four Vessel Umbilical Cord is usually good in most isolated cases
• However, when it is observed in association with certain fetal congenital anomalies, the outcomes depend on the type of congenital anomalies and severity of signs and symptoms
• An appropriate and timely management of the associated abnormality helps improve outcomes. The presence of Four Vessel Umbilical Cord does not always mean that the prognosis is poor

Additional and Relevant Useful Information for Four Vessel Umbilical Cord:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/pregnancy-related-disorders/