Umbilical vein varix
Citation, DOI & article data
Umbilical vein varix (UVV) refers to a focal dilatation of the umbilical vein.
UVVs were initially thought to have a high association with other anomalies which include:
- chromosomal anomalies: 5-12% with FIUVV 2,3
- underlying congenital cardiovascular anomaly
Recent literature points to more favorable outcomes when FIUVV is an isolated finding, with significantly lower associations with aneuploidy and mortality than initially reported 14,15.
It tends to favor 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.
Using antenatal ultrasound, an intra-abdominal UVV may be seen as a cyst-like structure in the fetal upper abdomen with venous flow within on color Doppler interrogation. The term umbilical varix is defined when the caliber is greater 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.
Treatment and prognosis
The overall clinical course is variable and unpredictable ranging from favorable in some to unfavorable in others 6. Most cases with an isolated varix have a very good prognosis; most neonates will have a normal outcome.
- development of hydrops fetalis: ~5% 12
- due to thrombosis
- possibly from an associated cardiovascular anomaly
Due to its association with other fetal anomalies and relatively high mortality rates (when not an isolated finding), a careful fetal search for other abnormalities 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.
- 1. Rahemtullah A, Lieberman E, Benson C et-al. Outcome of pregnancy after prenatal diagnosis of umbilical vein varix. J Ultrasound Med. 2001;20 (2): 135-9. J Ultrasound Med (abstract) - Pubmed citation
- 2. Ipek A, Kurt A, Tosun O et-al. Prenatal diagnosis of fetal intra-abdominal umbilical vein varix: report of 2 cases. J Clin Ultrasound. 2008;36 (1): 48-50. doi:10.1002/jcu.20334 - Pubmed citation
- 3. Sepulveda W, Mackenna A, Sanchez J et-al. Fetal prognosis in varix of the intrafetal umbilical vein. J Ultrasound Med. 1998;17 (3): 171-5. J Ultrasound Med (abstract) - Pubmed citation
- 4. Fung TY, Leung TN, Leung TY et-al. Fetal intra-abdominal umbilical vein varix: what is the clinical significance? Ultrasound Obstet Gynecol. 2005;25 (2): 149-54. doi:10.1002/uog.1815 - Pubmed citation
- 5. Byers BD, Goharkhay N, Mateus J et-al. Pregnancy outcome after ultrasound diagnosis of fetal intra-abdominal umbilical vein varix. Ultrasound Obstet Gynecol. 2009;33 (3): 282-6. doi:10.1002/uog.6233 - Pubmed citation
- 6. Valsky DV, Rosenak D, Hochner-celnikier D et-al. Adverse outcome of isolated fetal intra-abdominal umbilical vein varix despite close monitoring. Prenat. Diagn. 2004;24 (6): 451-4. doi:10.1002/pd.897 - Pubmed citation
- 7. Zalel Y, Lehavi O, Heifetz S et-al. Varix of the fetal intra-abdominal umbilical vein: prenatal sonographic diagnosis and suggested in utero management. Ultrasound Obstet Gynecol. 2000;16 (5): 476-8. doi:10.1046/j.1469-0705.2000.00283.x - Pubmed citation
- 8. Mahony BS, Mcgahan JP, Nyberg DA et-al. Varix of the fetal intra-abdominal umbilical vein: comparison with normal. J Ultrasound Med. 1992;11 (2): 73-6. J Ultrasound Med (abstract) - Pubmed citation
- 9. White SP, Kofinas A. Prenatal diagnosis and management of umbilical vein varix of the intra-amniotic portion of the umbilical vein. J Ultrasound Med. 1994;13 (12): 992-4. J Ultrasound Med (citation) - Pubmed citation
- 10. Jeanty P. Fetal and funicular vascular anomalies: identification with prenatal US. Radiology. 1989;173 (2): 367-70. Radiology (abstract) - Pubmed citation
- 11. Panda B, Stiller R, Iruretagoyena I et-al. Prenatal diagnosis of an umbilical vein aneurysm: a case report. Conn Med. 2009;73 (8): 465-7. - Pubmed citation
- 12. Nyberg DA, McGahan JP, Pretorius DH. Diagnostic imaging of fetal anomalies. Lippincott Williams & Wilkins. (2003) ISBN:0781732115. Read it at Google Books - Find it at Amazon
- 13. Mankuta D, Nadjari M, Pomp G. Isolated fetal intra-abdominal umbilical vein varix: clinical importance and recommendations. J Ultrasound Med. 2011;30 (2): 273-6. Pubmed citation
- 14. Lee SW et al. Clinical characteristics and outcomes of antenatal fetal intra-abdominal umbilical vein varix detection. Obstet Gynecol Sci. 2014 May;57(3):181-6. doi: 10.5468/ogs.2014.57.3.181. Epub 2014 May 15. Pubmed citation
- 15. Byers BD, Goharkhay N, et al. Pregnancy outcome after ultrasound diagnosis of fetal intra-abdominal umbilical vein varix. Ultrasound Obstet Gynecol. 2009 Mar;33(3):282-6. doi: 10.1002/uog.6233. Pubmed citation