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Persistent Right Umbilical Vein
Roya Sohaey, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Persistent right umbilical vein (PRUV)
              • Definitions

                • PRUV: Embryologic right umbilical vein (UV) remains open and left UV usually obliterates
                  • Normal: Right UV obliterates and left UV remains open
                • 2 types of PRUV
                  • Intrahepatic PRUV (most common)
                    • Right UV joins at sinus venosus and proceeds to ductus venosus (DV)
                  • Extrahepatic PRUV (rare)
                    • PRUV bypasses liver and portal system
                      • Drains directly into right atrium, inferior vena cava, or iliac vein
                    • DV often absent
                      • If left UV remains patent, then DV may be present

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  • Reporting Tips

                                    Selected References

                                    1. Toscano P et al: Intrahepatic persistent fetal right umbilical vein: a retrospective study. J Matern Fetal Neonatal Med. 1-4, 2019
                                    2. Achiron R et al: Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance. Ultrasound Obstet Gynecol. 47(6):739-47, 2016
                                    3. Lide B et al: Intrahepatic persistent right umbilical vein and associated outcomes: a systematic review of the literature. J Ultrasound Med. 35(1):1-5, 2016
                                    4. Martinez R et al: Perinatal outcome after ultrasound prenatal diagnosis of persistent right umbilical vein. Eur J Obstet Gynecol Reprod Biol. 168(1):36-9, 2013
                                    5. Weichert J et al: Persistent right umbilical vein: a prenatal condition worth mentioning? Ultrasound Obstet Gynecol. 37(5):543-8, 2011
                                    6. Hoehn T et al: Persistent right umbilical vein associated with complex congenital cardiac malformation. Am J Perinatol. 23(3):181-2, 2006
                                    7. Nakstad B et al: Abnormal systemic venous connection possibly associated with a persistent right umbilical vein; a case report. BMC Pediatr. 4(1):7, 2004
                                    8. Wolman I et al: Persistent right umbilical vein: incidence and significance. Ultrasound Obstet Gynecol. 19(6):562-4, 2002
                                    9. De Catte L et al: Persistent right umbilical vein in trisomy 18: sonographic observation. J Ultrasound Med. 17(12):775-9, 1998
                                    10. Ariyuki Y et al: Antenatal diagnosis of persistent right umbilical vein. J Clin Ultrasound. 23(5):324-6, 1995
                                    11. Hill LM et al: Persistent right umbilical vein: sonographic detection and subsequent neonatal outcome. Obstet Gynecol. 84(6):923-5, 1994
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Persistent right umbilical vein (PRUV)
                                                • Definitions

                                                  • PRUV: Embryologic right umbilical vein (UV) remains open and left UV usually obliterates
                                                    • Normal: Right UV obliterates and left UV remains open
                                                  • 2 types of PRUV
                                                    • Intrahepatic PRUV (most common)
                                                      • Right UV joins at sinus venosus and proceeds to ductus venosus (DV)
                                                    • Extrahepatic PRUV (rare)
                                                      • PRUV bypasses liver and portal system
                                                        • Drains directly into right atrium, inferior vena cava, or iliac vein
                                                      • DV often absent
                                                        • If left UV remains patent, then DV may be present

                                                IMAGING

                                                • General Features

                                                  • Ultrasonographic Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  • Image Interpretation Pearls

                                                                    • Reporting Tips

                                                                      Selected References

                                                                      1. Toscano P et al: Intrahepatic persistent fetal right umbilical vein: a retrospective study. J Matern Fetal Neonatal Med. 1-4, 2019
                                                                      2. Achiron R et al: Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance. Ultrasound Obstet Gynecol. 47(6):739-47, 2016
                                                                      3. Lide B et al: Intrahepatic persistent right umbilical vein and associated outcomes: a systematic review of the literature. J Ultrasound Med. 35(1):1-5, 2016
                                                                      4. Martinez R et al: Perinatal outcome after ultrasound prenatal diagnosis of persistent right umbilical vein. Eur J Obstet Gynecol Reprod Biol. 168(1):36-9, 2013
                                                                      5. Weichert J et al: Persistent right umbilical vein: a prenatal condition worth mentioning? Ultrasound Obstet Gynecol. 37(5):543-8, 2011
                                                                      6. Hoehn T et al: Persistent right umbilical vein associated with complex congenital cardiac malformation. Am J Perinatol. 23(3):181-2, 2006
                                                                      7. Nakstad B et al: Abnormal systemic venous connection possibly associated with a persistent right umbilical vein; a case report. BMC Pediatr. 4(1):7, 2004
                                                                      8. Wolman I et al: Persistent right umbilical vein: incidence and significance. Ultrasound Obstet Gynecol. 19(6):562-4, 2002
                                                                      9. De Catte L et al: Persistent right umbilical vein in trisomy 18: sonographic observation. J Ultrasound Med. 17(12):775-9, 1998
                                                                      10. Ariyuki Y et al: Antenatal diagnosis of persistent right umbilical vein. J Clin Ultrasound. 23(5):324-6, 1995
                                                                      11. Hill LM et al: Persistent right umbilical vein: sonographic detection and subsequent neonatal outcome. Obstet Gynecol. 84(6):923-5, 1994