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 be also be supernumerary 6.
A PRUV can be intra or extra-hepatic. The former is much more 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 a 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.
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- 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
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