Fetal Intra-Abdominal Umbilical Vein Varix: A Case Report and Literature Review.

Fetal intra-abdominal umbilical vein (FIUV) varix is a rare malformation of the umbilical cord. This is a critical situation due to discrepancies in outcomes varying from normal to high rates of complications and fetal mortalities. We report the observation of a FIUVV vein diagnosed precociously at 22 weeks with a quiet increasing of the diameter by 31 weeks. The outcome was favourable and close monitoring after birth showed no anomalies. Despite a good prognosis it seems that a close monitoring is essential in antenatal period. Figure 1: Longitudinal and axial section of the fetal abdomen showing an intra-abdominal cystic dilatation on path of the umbilical vein. Page 2 of 3 Citation: Bouzid A, Karmous N, Trabelsi H, Mkaouar L, Mourali M (2016) Fetal Intra-abdominal Umbilical Vein Varix: A Case Report and Literature Review. Gynecol Obstet (Sunnyvale) 6: 379. doi:10.4172/2161-0932.1000379 Volume 6 • Issue 5 • 1000379 Gynecol Obstet (Sunnyvale) ISSN: 2161-0932 Gynecology, an open access journal the 167 cases of the literature, E. Beraud et al. reported only 17 cases diagnosis under the term of 22 weeks [3]. The diagnosis of umbilical vein varix justifies a detailed fetal anatomical assessment in a reference center to look for other abnormalities. These associated forms account for 29 to 35% of FIUVV [9] and involves performing a fetal karyotype. In our case we did not realize the karyotype because the FIUVV was isolated. The most common complications of FIUVV described in the literature include rupture of the aneurysm, thrombosis, compression of the umbilical artery and other veins, and cardiac failure due to vascular stealing by the varix and increased preload [10]. The overall frequency is assessed at 10%. Recently published studies modulate the global occurrence of complications and reconsider their relative importance. In our case the diameter of the varix increased quietly but no complication was detected. In all cases, attentive monitoring is recommended, in particular during the third trimester of pregnancy. The frequency of the sonograms varies, depending on the teams, from one examination every two weeks to two examinations per week. The main goal is to detect a thrombus [3]. Valsky et al. [11] and Zalel et al. [12] have advocated early delivery in cases of UVV, on attainment of lung maturity. However, this opinion is controversial because if no other anomalies are present, the prognosis is generally good. No obstetric complications were reported in the literature, independently from the type of delivery, including deliveries at term. Conclusion The significance of antenatal FIUVV detection remains unclear. The discrepancies in outcomes varying from normal to high rates of complications and fetal mortalities could be due to rarity of this anomaly. A recent review showed the good prognosis of isolated form even if diagnosed early in pregnancy. In such cases, antenatal follow-up to term is sufficient. Conflicts of InterestNo conflict of interest.References 1. Mankuta D, Nadjari M, Pomp G (2011) Isolated fetal intra-abdominalumbilicalvein varix: clinical importance and recommenda-tions. J Ultrasound Med 30:273-276. 2. Ipek A, Kurt A, Tosun Ö, Gümüs M, Yazicioglu KR, et al. (2008) Prenataldiagnosis of fetal intra-abdominal umbilical vein varix: report of 2 cases. J ClinUltrasound 36: 48-50. 3. Beraud E, Rozel C, Milon J, Darnault P (2015) Umbilical vein varix: Importanceof anteand post-natal monitoring by ultrasound. Diagnostic and InterventionalImaging 96: 21-26. 4. Mahony BS, McGahan JP, Nyberg DA, Reisner DP (1992) Varix of the fetalintra-abdominal umbilical vein: Comparison with normal. J Ultrasound Med 11:73-76. 5. Sepulveda W, Mackenna A, Sanchez J, Corral E, Carstens E (1998) Fetalprognosis in varix of the intrafetal umbilical vein. J Ultrasound Med 17: 171-175. 6. Allen SL, Bagnall C, Roberts AB, Teele RL (1988) Thrombosing umbilical veinvarix. J Ultrasound Med 17: 189-192. 7. Jeanty P (1989) Fetal and funicular vascular anomalies: identification withprenatal US. Radiology 173: 367-370. 8. Estroff JA, Benacerraf BR (1992) Fetal umbilical vein varix: sonographicappearance and postnatal outcome. J Ultrasound Med 11: 69-73. 9. Byers BD, Goharkhay N, Mateus J, Ward KK, Munn MB, et al. ( 2009)Pregnancy outcome after ultrasound diagnosis of fetal intra-abominal umbilicalvein varix. Ultrasound Obstet Gynecol 33: 282-286.abdominal umbilical vein grows linearly to reach 8 mm at term [4].FIUVV is defined as an intra-abdominal umbilical vein diameter atleast 1.5 times greater than the diameter of the intra-hepatic umbilicalvein [5] or an intra-abdominal umbilical vein diameter exceeding 9mm [6]. Strict quality criteria must be applied to hold the diagnosis.FIUVV is detected as an anechoic, oval-shaped or rounded mass,located between the abdominal wall and the lower edge of the liver [7].It is in continuity with the umbilical vascular axis on sagittal sections.Several differential diagnosis can be evoked in particular choledochalcyst, liver cyst, urachal cyst and mesenteric cyst. The pulsed and colorDoppler modes confirm the vascular nature of the abnormality andreveal a venous type flow. The origins of an FIUVV are not well-defined. Current evidencesupports the hypothesis that it is a developmental rather than acongenital malformation [8]. The most likely etiology and the onlypathologic finding in most cases is thinning of the vessel wall near theanterior abdominal wall due to intrinsic weakness of the umbilical veinwall [2]. A peculiarity in our observation is the too early diagnosis term asthe median gestational age at diagnosis is 27 weeks [5]. In his review ofFigure 2: A 3D acquisition of the intra-abdominal portion of the cord showing no expansionof the structure at this level.Figure 2: A 3D acquisition of the intra-abdominal portion of the cord showingno expansion of the structure at this level. Figure 3: Examination revealed it to be vascular in nature with the umbilical vein leading to it.Figure 3: Examination revealed it to be vascular in nature with the umbilicalvein leading to it. Page 3 of 3Citation: Bouzid A, Karmous N, Trabelsi H, Mkaouar L, Mourali M (2016) Fetal Intra-abdominal Umbilical Vein Varix: A Case Report and LiteratureReview. Gynecol Obstet (Sunnyvale) 6: 379. doi:10.4172/2161-0932.1000379 Volume 6 • Issue 5 • 1000379Gynecol Obstet (Sunnyvale)ISSN: 2161-0932 Gynecology, an open access journal10. Rahemtullah A, Lieberman E, Benson C, Norton ME (2001) Outcome ofpregnancy after prenatal diagnosis of umbilical vein varix. J Ultrasound Med20: 135-139. 11. Valsky DV, Rosenak D, Hochner-Celnikier D, Porat S, Yagel S (2004) Adverseoutcome of isolated fetal intraabdominal umbilical vein varix despite closedmonitoring. Prenat Diagn 24: 451-454. 12. Zalel Y, Lehavi O, Heifetz S, Aizenstein O, Dolitzki M, et al. (2000) Varix ofthe fetal intra-abdominal umbilical vein; prenatal sonographic diagnosis andsuggested in utero management. Ultrasound Obstet Gynecol 16: 476-478. OMICS International: Publication Benefits & Features