Umbilical vein varix

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

Location

It tends to favour 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

Associations

UVV's 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 reported14-15.​

Antenatal ultrasound

An intra-abdominal UVV may be seen as a cyst like structure in the fetal upper abdomen with venous flow within on colour Doppler interrogation. The term umbilical varix is defined as being more 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.

Due to its association with other fetal anomalies and relatively high mortality rates (when not an isolated finding), a careful fetal search for other abnormality 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

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

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

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Article information

rID: 13567
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Umbilical venous varix
  • Umbilical vein varix (UVV)
  • Variceal dilatation of the umbilical vein
  • Umbilical vein aneurysm

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