Fetal intra-abdominal umbilical vein varix (FIUVV) is a focal aneurysmal
dilatation of the umbilical vein. Its clinical importance has not
yet been clearly established, but it has been reported to be associated
with increased fetal fetal malformations, chromosomal abnormalities
and increased death rate (1 ; 5).
Case report :
We describe one case of dilation or varix of the umbilical vein
appearing as a fetal intra-abdominal cyst at 28 weeks in a 21-year-old
female G2 P1. Doppler or color flow examination indicated umbilical
venous flow within the cystic area in the case. This infant was
delivered at term without sequelae.
The umbilical vein was considered dilated when the measurement was
above 2 standard deviation of the mean for gestational age (3 ;
In literature an association has been reported between the presence
of FIUVV and fetal anomalies and/or obstetrical complications. (3).
Additional sonographic abnormalities were detected prenatally in
31.9%, most commonly anomalies of the cardiovascular system (including
structural and functional abnormalities), hydropic features and
anemia. Chromosomal abnormalities were detected in 12% and recently
it has been suggested that this prenatal finding should be considered
as a soft marker for aneuploidy. Mortality associated with the FIUVV
has been reported between 24-44%(4).
In presence of FIUVV, Fetal echocardiography and detailed US study
of fetal anatomy is needed to exclude associated anomalies. Isoimmunization
should be ruled out, and consideration of karyotyping should be
discussed. Serial follow-up scans are needed to exclude the onset
of hydrops or thrombosis of the varix (5). A close fetal monitoring
by serial color Doppler and ultrasonographic examinations should
be performed. (3 ; 4; 6).
Sepulveda.. (7) conclude that fetuses with varix of the intrafetal
umbilical vein should be considered at risk for poor outcome. However,
if no other anomalies are present, the prognosis is generally good.(7)
Differential diagnosis includes other abdominal
cysts such as choledochal, mesenteric or urachal cysts. The
presence of intra-abdominal umbilical vein varix can be confirmed
by color Doppler finding of turbulent flow in the cystic mass. Prognosis
A varix of intrafetal umbilical vein is considered a poor prognostic
sign; if no associated anomalies are present, the prognosis is generally
1: Ipek A, Kurt A, Tosun O, Gumus M, Yazicioglu KR, Asik E, Tas
I. Prenatal diagnosis of fetal intra-abdominal umbilical vein varix:
Report of 2 cases. J Clin Ultrasound. 2007 Apr 13;
2: Mulch AD, Stallings SP, Salafia CM. Elevated maternal serum alpha-fetoprotein,
umbilical vein varix, and mesenchymal dysplasia: are they related?
Prenat Diagn. 2006 Aug;26(8):659-61
3: Volpe G, Resta L, Volpe P, Stefanelli R, Minervini M, Volpe N,
Buonadonna L, Gentile M. [Varix of the extra-hepatic portion of
the fetal intra-abdominal umbilical vein: pathogenesis, prenatal
sonographic diagnosis, and perinatal outcome] Minerva Ginecol. 2006
4 : Fung TY, Leung TN, Leung TY, Lau TK. Fetal intra-abdominal umbilical
vein varix: what is the clinical significance? Ultrasound Obstet
Gynecol. 2005 Feb;25(2):149-54.
5: Viora E, Sciarrone A, Bastonero S, Errante G, Mortara G, Chiappa
E, Campogrande M. Anomalies of the fetal venous system: a report
of 26 cases and review of the literature. Fetal Diagn Ther. 2004
6: Rahemtullah A, Lieberman E, Benson C, Norton ME. Outcome of pregnancy
after prenatal diagnosis of umbilical vein varix. J Ultrasound Med.
7: Sepulveda W, Mackenna A, Sanchez J, Corral E, Carstens E. Fetal
prognosis in varix of the intrafetal umbilical vein. J Ultrasound
Med. 1998 Mar;17(3):171-5.
8: Estroff JA, Benacerraf BR. Fetal umbilical vein varix: sonographic
appearance and postnatal outcome. J Ultrasound Med. 1992 Mar;11(3):69-73.