Single Umbilical Artery, Supernumerary Vessels, Segmental Thinning of the Umbilical Cord Vessels and Vascular Calcifications in Umbilical Vessels

  • Margaret J. EvansEmail author


Single umbilical artery (SUA) is diagnosed in approximately 0.2–2% of all pregnancies though some series quote rates of up to 5% depending on the population studied and the mode of examination (gross examination v microscopic). Absence of the left artery is more frequent than the absence of the right artery, being identified in 73% of cases of SUA. Supernumerary vessels (SNV) are extremely rare in humans, and persistence of the right umbilical vein with patency of the left appears more commonly than an extra artery. Both SUA and SNV can be discerned on examination of cross sections of the cord. Segmental thinning of umbilical vessels occurs in approx 1.5% of all pregnancies; the aetiology is unknown. Vascular calcifications in umbilical vessels are an extremely rare lesion with two distinct patterns recognised. The aetiology varies depending on the lesion. This chapter describes the embryology of these lesions, outlines the diagnostic pitfalls and briefly references related disorders.


Umbilical cord Single umbilical artery Supernumerary vessels Persistent right umbilical vein Segmental thinning Vascular calcification 


  1. 1.
    Moore KL, Persaud TVN. The placenta and fetal membranes. In: Moore KL, Persaud TVN, editors. The developing human: clinically oriented embryology. 6th ed. Philadelphia: W.B. Saunders; 1998. p. 129–62.Google Scholar
  2. 2.
    Geipel A, Germer U, Welp T, Schwinger E, Gembruch U. Prenatal diagnosis of single umbilical artery: determination of the absent side, associated anomalies, Doppler findings and perinatal outcome. Ultrasound Obstet Gynecol. 2000;15:114–7.CrossRefGoogle Scholar
  3. 3.
    Martínez-Frías ML, Bermejo E, Rodríguez-Pinilla E, Prieto D, ECEMC Working Group. Does single umbilical artery (SUA) predict any type of congenital defect? Clinical-epidemiological analysis of a large consecutive series of malformed infants. Am J Med Genet A. 2008;146A:15–25.CrossRefGoogle Scholar
  4. 4.
    Akoi S, Hata T, Ariyuki Y, Makihara K, Hata K, Kiato M. Antenatal diagnosis of aberrant umbilical vessels. Gynecol Obstet Investig. 1997;43:232–5.CrossRefGoogle Scholar
  5. 5.
    Khong TY, Dilly SA. Calcification of umbilical artery: two distinct lesions. J Clin Pathol. 1989;42:931–4.CrossRefGoogle Scholar
  6. 6.
    Qureshi F, Jacques SM. Marked segmental thinning of the umbilical cord vessels. Arch Pathol Lab Med. 1994;118:826–30.PubMedGoogle Scholar
  7. 7.
    Heifetz SA. Single umbilical artery: a statistical analysis of 237 autopsy cases and review of the literature. Perspect Pediatr Pathol. 1984;8:345–78.PubMedGoogle Scholar
  8. 8.
    Lilja M. Infants with single umbilical artery studied in a national registry. General epidemiological characteristics. Paediatr Perinat Epidemiol. 1992;5:27–36.CrossRefGoogle Scholar
  9. 9.
    Abuhamad AZ, Shaffer W, Mari G, Copel JA, Hobbins JC, Evans AT. Single umbilical artery: does it matter which artery is missing? Am J Obstet Gynecol. 1995;173:728–32.CrossRefGoogle Scholar
  10. 10.
    DeFigueiredo D, Dagklis T, Zidere V, Allan L, Nicolaides KH. Isolated single umbilical artery: need for specialist fetal echocardiography? Ultrasound Obstet Gynecol. 2010;36:553–5.CrossRefGoogle Scholar
  11. 11.
    Dagklis T, DeFigueiredo D, Staboulidou I, Casagrandi D, Nicolaides KH. Isolated single umbilical artery and fetal karyotype. Ultrasound Obstet Gynecol. 2010;36:291–5.CrossRefGoogle Scholar
  12. 12.
    Gutvirtz G, Walfisch A, Beharier O, Sheiner E. Isolated umbilical artery is an independent risk factor for perinatal mortality and adverse outcomes in term neonates. Arch Gynecol Obstet. 2016;294:931–5.CrossRefGoogle Scholar
  13. 13.
    Mailath-Pokorny M, Worda K, Schmid M, Polterauer S, Bettelheim D. Isolated umbilical artery: evaluating the risk of adverse pregnancy outcome. Eur J Obstet Gynecol Reprod Biol. 2015;184:80–3.CrossRefGoogle Scholar
  14. 14.
    Battarbee AN, Palatnik A, Ernst L, Grobman WA. Association of isolated single umbilical artery with small for gestational age and preterm birth. Obstet Gynecol. 2015;126:760–4.CrossRefGoogle Scholar
  15. 15.
    Kim HJ, Kim JH, Chay DB, Park JH, Kim MA. Association of isolated single umbilical artery with perinatal outcomes: systemic review and meta-analysis. Obstet Gynecol Sci. 2017;60:266–73.CrossRefGoogle Scholar
  16. 16.
    Karatza A, Tsamandas A, Varvarigou A, Davlouros P, Pavlou V, Mantagos S. Supernumerary umbilical vein in a hydropic neonate with hypertrophic cardiomyopathy. Fetal Pediatr Pathol. 2011;30:173–6.CrossRefGoogle Scholar
  17. 17.
    Painter D, Russell P. Four-vessel umbilical cord associated with multiple congenital anomalies. Obstet Gynecol. 1977;50:505–7.PubMedGoogle Scholar
  18. 18.
    Kaufmann HJ, Weisser K. Neonatal transumbilical angiography. A preliminary report. Ann Radiol. 1964;7:437.PubMedGoogle Scholar
  19. 19.
    Rehder H. Anomalies of the portal and umbilical veins [in German]. Vichows Arch A Pathol Anat. 1971;352:50–60.CrossRefGoogle Scholar
  20. 20.
    Pérez-Cosio C, Sheiner E, Abramowicz JS. Four-vessel umbilical cord: not always a dire prognosis. J Ultrasound Med. 2008;27:1389–91.CrossRefGoogle Scholar
  21. 21.
    Du X, Yuan Q, Li Z, Li Y. Three umbilical arteries resulting in a four-vessel umbilical cord in a stillbirth. Int J Clin Exp Med. 2015;8:4682–5.PubMedPubMedCentralGoogle Scholar
  22. 22.
    Weichert J, Hartge U, Germer R, Axt-Fliedner R, Gembruch U. Persistent right umbilical vein: a prenatal condition worth mentioning. Ultrasound Obstet Gynecol. 2011;37:543–8.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of PathologyEdinburgh Royal InfirmaryEdinburghUK
  2. 2.Centre for Comparative PathologyUniversity of EdinburghEdinburghUK
  3. 3.Department of Health SciencesCollege of Life Sciences, University of Leicester, Centre for MedicineLeicesterUK

Personalised recommendations