Umbilical vein varix

Last revised by Mostafa El-Feky on 30 May 2020

Umbilical vein varix (UVV) refers to a focal dilatation of the umbilical vein.

UVVs were initially thought to have a high association with other anomalies which include:

Recent literature points to more favorable outcomes when FIUVV is an isolated finding, with significantly lower associations with aneuploidy and mortality than initially reported 14,15.

It tends to favor the intra-abdominal portion of the cord (typically between the abdominal wall and the liver) which is then termed a fetal intra-abdominal umbilical vein varix (FIUVV) or the intra-amniotic portion of the umbilical vein.

Using antenatal ultrasound, an intra-abdominal UVV may be seen as a cyst-like structure in the fetal upper abdomen with venous flow within on color Doppler interrogation. The term umbilical varix is defined when the caliber is greater than 9 mm or with a ratio of more than 50% between the dilated and a more distal normal intra-abdominal portion of the vein.

The overall clinical course is variable and unpredictable ranging from favorable in some to unfavorable in others 6. Most cases with an isolated varix have a very good prognosis; most neonates will have a normal outcome.

  • development of hydrops fetalis: ~5% 12
    • due to thrombosis
    • possibly from an associated cardiovascular anomaly 

Due to its association with other fetal anomalies and relatively high mortality rates (when not an isolated finding), a careful fetal search for other abnormalities as well as close monitoring is recommended if detected prenatally. Follow-up is warranted when an umbilical vein varix is diagnosed (growth and well being), more so if other abnormalities are identified 1,7

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Cases and figures

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