To assess the clinical significance of varix of the intraabdominal por
tion of the umbilical vein, we reviewed 10 cases diagnosed prenatally
by ultrasonography at a median gestational age of 27 weeks. A comprehe
nsive anatomic survey and serial follow-up scans were performed in eac
h case. All three fetuses with associated anomalies died in utero, and
prenatal karyotyping revealed that two of them had a chromosomal abno
rmality. In six of the seven cases with structurally normal fetuses th
e pregnancy proceeded uneventfully, and no neonatal complications were
attributed to the umbilical vein varix. Our experience and the review
of the Literature revealed 42 cases with information on fetal outcome
. Overall, 24% of the fetuses died, 12% had a chromosomal abnormality,
and 5% developed hydrops. We conclude that fetuses with varix of the
intrafetal umbilical vein should be considered at risk for poor outcom
e. However, if no other anomalies are present, the prognosis is genera
lly good.