A persistent right umbilical vein (PRUV) is an uncommon vascular anomaly which is often detected in utero.
The estimated prevalence is at ~2 per 1000 births 1-2.
In the normal situation, the right umbilical vein begins to obliterate in the ~4th week of gestation and disappears by the 7th week. With a PRUV, the right umbilical vein remains open and the left umbilical vein usually obliterates. A PRUV may also be supernumerary 6.
A PRUV can be intra- or extra-hepatic. The former is much commoner.
Numerous associated have been described (albeit as relatively low rates 3,9). They are commoner with the extra-hepatic type and include:
- single umbilical artery: especially in the extra-hepatic type
- chromosomal anomalies
- Noonan syndrome 8
- situs anomalies
- congenital cardiac anomalies
- congenital renal anomalies
- congenital gastrointestinal anomalies
- vertebral anomalies
It is usually detected in the 2nd to 3rd trimester. Assessment is usually made in the axial plane and colour Doppler is often required. An intra-hepatic persistent right umbilical vein may be seen as an umbilical vein abnormally connected to the right portal vein and the fetal gallbladder is positioned medial to the PRUV.
Treatment and prognosis
When additional anomalies are ruled out, a PRUV in isolation carries a generally favourable outcome 1,9.
If a persistent right umbilical vein is detected, a careful sonographic anatomical survey is generally recommended to rule out more serious congenital malformations 10.
- 1. Wolman I, Gull I, Fait G et-al. Persistent right umbilical vein: incidence and significance. Ultrasound Obstet Gynecol. 2002;19 (6): 562-4. doi:10.1046/j.1469-0705.2002.00678.x - Pubmed citation
- 2. Benacerraf BR. Ultrasound of fetal syndromes. Churchill Livingstone. (2008) ISBN:0443066418. Read it at Google Books - Find it at Amazon
- 3. Nakstad B, Smevik B. Abnormal systemic venous connection possibly associated with a persistent right umbilical vein; a case report. BMC Pediatr. 2004;4 : 7. doi:10.1186/1471-2431-4-7 - Free text at pubmed - Pubmed citation
- 4. Kirsch CF, Feldstein VA, Goldstein RB et-al. Persistent intrahepatic right umbilical vein: a prenatal sonographic series without significant anomalies. J Ultrasound Med. 1996;15 (5): 371-4. J Ultrasound Med (abstract) - Pubmed citation
- 5. Shen O, Tadmor OP, Yagel S. Prenatal diagnosis of persistent right umbilical vein. Ultrasound Obstet Gynecol. 1996;8 (1): 31-3. doi:10.1046/j.1469-0705.1996.08010031.x - Pubmed citation
- 6. Jeanty P. Persistent right umbilical vein: an ominous prenatal finding? Radiology. 1990;177 (3): 735-8. Radiology (abstract) - Pubmed citation
- 7. Ricklan DE, Collett TA, Lyness SK. Umbilical vein variations: review of the literature and a case report of a persistent right umbilical vein. Teratology. 1988;37 (2): 95-100. doi:10.1002/tera.1420370202 - Pubmed citation
- 8. Bradley E, Kean L, Twining P et-al. Persistent right umbilical vein in a fetus with Noonan's syndrome: a case report. Ultrasound Obstet Gynecol. 2001;17 (1): 76-8. doi:10.1046/j.1469-0705.2001.00243.x - Pubmed citation
- 9. Blazer S, Zimmer EZ, Bronshtein M. Persistent intrahepatic right umbilical vein in the fetus: a benign anatomic variant. Obstet Gynecol. 2000;95 (3): 433-6. - Pubmed citation
- 10. Hill LM, Mills A, Peterson C et-al. Persistent right umbilical vein: sonographic detection and subsequent neonatal outcome. Obstet Gynecol. 1994;84 (6): 923-5. - Pubmed citation
- 11. Merz E, Bahlmann F. Ultrasound in obstetrics and gynecology. Thieme Medical Publishers. (2005) ISBN:1588901475. Read it at Google Books - Find it at Amazon