Umbilical vein varix

Last revised by Arlene Campos on 21 Aug 2024

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:

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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