Four-Vessel Umbilical Cord Associated with Multiple Congenital Anomalies: A Case Report and Literature Review

Abstract

A case is described of a neonate with a four-vessel umbilical cord containing two arteries and two veins. This was due to a rare persistence of the caudal portion of the right umbilical vein. The infant had multiple congenital anomalies including a complete atrioventricular canal, an interrupted inferior vena cava, a double superior vena cava, a left ventricular hypoplasia, dextrocardia, situs ambiguous, and malrotation of the small bowel. The birth of an infant with a four-vessel cord mandates comprehensive work-up for associated anomalies. The literature is reviewed.

A four-vessel umbilical cord

“…The presence of a fourth umbilical structure might often be unnoticed because we stop counting at three. However, this finding can be related to potentially serious congenital anomalies including renal and cardiac malformations and therefore requires additional imaging 4. In our patient, ultrasound examination of the development and position of the heart and intra-abdominal organs did not reveal any abnormalities.…”

Prenatal diagnosis of four‐vessel umbilical cord with supernumerary vein varix: A case report and literature review

“…Normally, the right umbilical vein becomes obliterated at 28-32 days of gestation, disappearing by the seventh week of gestation, and the single umbilical vein present at birth is derived from the primitive left umbilical vein. 9 The presence of two veins could be attributed to the persistence of both the normal left umbilical vein and the caudal part of the right umbilical vein (supernumerary were detected antenatally (Cases 4, 5,7,8,10,11,16), whereas in the other nine cases, it was found postpartum. Fetal age at the time of detection ranged from 21 to 35 gestational weeks.…”

“…A four-vessel umbilical cord (FVUC) with a supernumerary vein is rare and is associated with different anomalies, such as anterior chest wall defects, bilateral cleft lip and palate, placental arteriovenous fistula, edema, heterotaxy syndrome, trisomy 18 and fetal growth retardation. [1][2][3][4][5][6][7] However, its clinical importance has not yet been clearly established. Umbilical vein varix is another anomaly of the umbilical cord and has been related to the high incidence of fetal anomalies and obstetric complications.…”

Study of the Primo-Vascular System and Location-Dependent Oxygen Levels for a Mouse Embryo

“…This may be due to the profusion of blood vessels in the umbilical cord. 12 Based on this, it may be hypothesized that the PVS is less developed in areas where the blood vessel system is highly developed.…”