Retroperiton

Umbilical vein varix

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Introduction
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.

Discussion
The umbilical vein was considered dilated when the measurement was above 2 standard deviation of the mean for gestational age (3 ; 4).
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
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 good.(7)

Reference:
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 Feb;58(1):17-23.
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 Sep-Oct;19(5):440-7.
6: Rahemtullah A, Lieberman E, Benson C, Norton ME. Outcome of pregnancy after prenatal diagnosis of umbilical vein varix. J Ultrasound Med. 2001 Feb;20(2):135-9.
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.