Extra-abdominal umbilical vein varix in a newborn

Abstract

Umbilical vein varices are rare fetal anomalies typically found intra-abdominally and identified on ultrasound prior to birth. Intra-abdominal umbilical vein varices account for approximately 4% of umbilical cord abnormalities and are thought to be a developmental abnormality rather than a congenital malformation. The umbilical vein varix anomaly been shown to be associated with a higher incidence of adverse perinatal outcomes and there is evidence of a relationship between this and chromosomal abnormalities. There have been few case reports of extra-abdominal varices. This case reviews a multiparous Hispanic female who delivered a baby with an extra-abdominal umbilical vein varix who was admitted to the neonatal intensive care unit but had an uncomplicated hospital course. The report reviews strategies for antenatal testing and surveillance of identified varices.

Current Perspectives of Prenatal Sonography of Umbilical Cord Morphology

“…Umbilical varix is defined when the caliber is greater than 9 mm or with a ratio of >50% between the dilated and a more distal normal intraabdominal portion of the vein (Figure 8). [180][181][182] The incidence of this condition is relatively rare, occurring between 0.4 and 1.1/1000 deliveries and is associated with additional fetal anomalies in one-third of cases, [183][184][185]…”

Severe fetal anemia as a consequence of extra‐abdominal umbilical vein varix: A case report and review of the literature

“…12 Table 1. [13][14][15][16][17][18][19][20][21][22][23][24][25][26] Including our case, 11 cases (79%) have been diagnosed antenatally. The average of diameter of the EAUVV was 3.4 cm (range: 1-5 cm).…”

“…A search of the English literature on PubMed and Google Scholar using “umbilical vein varix” and “umbilical cord anomalies” as keywords (from 1980 to 2020) identified only 13 case reports describing EAUVV thus far. The clinical and sonographic characteristics and outcomes of the pregnancies with EAUVV are described in Table 1 13‐26 . Including our case, 11 cases (79%) have been diagnosed antenatally.…”

Massive Extra-Abdominal Umbilical Vein Varix: A Case Report

“…The case above represents the largest known case of a fetal extra-abdominal umbilical vein varix, and it ranks among only 14 other reported cases [4][5][6][7][8]. These varices are often difficult to diagnose prenatally, and even once a diagnosis has been established there is little data to inform methods of surveillance and management.…”

“…No such standards have been proposed to identify extra-abdominal umbilical vein varices because of their rarity and the difficulty of sonographic diagnosis since many extra-abdominal varices resemble umbilical cord cysts on ultrasound. Indeed, in many reported cases, the diagnosis was only established by postnatal or postmortem examination of the neonate [5][6][7][10][11][12]. In cases where an extra-abdominal varix is suspected, fetal MRI may be a helpful adjunct to ultrasonography in clarifying the diagnosis (Fig.…”