Single umbilical artery
- Martin L Gimovsky, MD
Martin L Gimovsky, MD
- Clinical Professor
- Department of Obstetrics, Gynecology and Reproductive Science
- Mount Sinai School of Medicine
- Eva Tejero Rosa, MS, CGC
Eva Tejero Rosa, MS, CGC
- Supervisor/Lead Genetic Counselor
- Atlantic Health System
- Waldo Sepulveda, MD
Waldo Sepulveda, MD
- Director, FETALMED
- Maternal-Fetal Diagnostic Center, Santiago, Chile
- Section Editors
- Deborah Levine, MD
Deborah Levine, MD
- Section Editor — Imaging
- Professor of Radiology
- Director of Ob/Gyn Ultrasound
- Department of Radiology
- Beth Israel Deaconess Medical Center
- Louise Wilkins-Haug, MD, PhD
Louise Wilkins-Haug, MD, PhD
- Section Editor — Prenatal Diagnosis and Genetics
- Professor of Obstetrics, Gynecology, and Reproductive Biology
- Harvard Medical School
The umbilical cord typically contains two arteries and one vein. Single umbilical artery (SUA) refers to a variation of umbilical cord anatomy in which there is only one umbilical artery. It may be an isolated finding, or associated with aneuploidy or other congenital anomalies.
Three mechanisms have been proposed to explain the development of SUA :
●Secondary atresia or atrophy of a previously normal umbilical artery
●Primary agenesis of one of the umbilical arteries
●Persistence of the original single allantoic artery of the body stalk
- Abuhamad AZ, Shaffer W, Mari G, et al. Single umbilical artery: does it matter which artery is missing? Am J Obstet Gynecol 1995; 173:728.
- Altshuler G, Tsang RC, Ermocilla R. Single umbilical artery. Correlation of clinical status and umbilical cord histology. Am J Dis Child 1975; 129:697.
- Persutte WH, Hobbins J. Single umbilical artery: a clinical enigma in modern prenatal diagnosis. Ultrasound Obstet Gynecol 1995; 6:216.
- Blackburn W, Cooley W. Umbilical cord. In: Human malformations and related anomalies, Stevenson RE, Hall JG (Eds), Oxford University Press, New York 1993. Vol 2nd, p.1275.
- Rembouskos G, Cicero S, Longo D, et al. Single umbilical artery at 11-14 weeks' gestation: relation to chromosomal defects. Ultrasound Obstet Gynecol 2003; 22:567.
- Granese R, Coco C, Jeanty P. The value of single umbilical artery in the prediction of fetal aneuploidy: findings in 12,672 pregnant women. Ultrasound Q 2007; 23:117.
- Thummala MR, Raju TN, Langenberg P. Isolated single umbilical artery anomaly and the risk for congenital malformations: a meta-analysis. J Pediatr Surg 1998; 33:580.
- Leung AK, Robson WL. Single umbilical artery. A report of 159 cases. Am J Dis Child 1989; 143:108.
- Bryan EM, Kohler HG. The missing umbilical artery. I. Prospective study based on a maternity unit. Arch Dis Child 1974; 49:844.
- Bianchi DW, Cromblehome TM, D’Alton ME. Fetology: Diagnosis and management of the fetal patient, McGraw Hill, New York 2000. p.827.
- Naeye RL. Disorders of the umbilical cord. In: Disorders of the placenta, fetus and neonate, Mosby-Year Book Inc, St Louis 1992. p.92.
- Bernirschke K, Driscoll SG. The pathology of the human placenta, Springer, New York 1967. p.232.
- Heifetz SA. Single umbilical artery. A statistical analysis of 237 autopsy cases and review of the literature. Perspect Pediatr Pathol 1984; 8:345.
- Klatt J, Kuhn A, Baumann M, Raio L. Single umbilical artery in twin pregnancies. Ultrasound Obstet Gynecol 2012; 39:505.
- Stout MJ, Odibo AO, Longman R, et al. The incidence of isolated single umbilical artery in twins and adverse pregnancy outcomes. Prenat Diagn 2013; 33:269.
- Victoria A, Mora G, Arias F. Perinatal outcome, placental pathology, and severity of discordance in monochorionic and dichorionic twins. Obstet Gynecol 2001; 97:310.
- AIUM practice guideline for the performance of obstetric ultrasound examinations. http://files.aium.org/guidelines/obstetric/imageresources.pdf (Accessed on March 03, 2011).
- Lamberty CO, de Carvalho MH, Miguelez J, et al. Ultrasound detection rate of single umbilical artery in the first trimester of pregnancy. Prenat Diagn 2011; 31:865.
- Lubusky M, Dhaifalah I, Prochazka M, et al. Single umbilical artery and its siding in the second trimester of pregnancy: relation to chromosomal defects. Prenat Diagn 2007; 27:327.
- Geipel A, Germer U, Welp T, et al. Prenatal diagnosis of single umbilical artery: determination of the absent side, associated anomalies, Doppler findings and perinatal outcome. Ultrasound Obstet Gynecol 2000; 15:114.
- Blazer S, Sujov P, Escholi Z, et al. Single umbilical artery--right or left? does it matter? Prenat Diagn 1997; 17:5.
- Budorick NE, Kelly TF, Dunn JA, Scioscia AL. The single umbilical artery in a high-risk patient population: what should be offered? J Ultrasound Med 2001; 20:619.
- Proctor LK, Fitzgerald B, Whittle WL, et al. Umbilical cord diameter percentile curves and their correlation to birth weight and placental pathology. Placenta 2013; 34:62.
- Suzuki S, Kato M. Clinical Significance of Pregnancies Complicated by Velamentous Umbilical Cord Insertion Associated With Other Umbilical Cord/Placental Abnormalities. J Clin Med Res 2015; 7:853.
- Sepúlveda WH. Antenatal sonographic detection of single umbilical artery. J Perinat Med 1991; 19:391.
- Sepulveda W, Peek MJ, Hassan J, Hollingsworth J. Umbilical vein to artery ratio in fetuses with single umbilical artery. Ultrasound Obstet Gynecol 1996; 8:23.
- De Catte L, Burrini D, Mares C, Waterschoot T. Single umbilical artery: analysis of Doppler flow indices and arterial diameters in normal and small-for-gestational age fetuses. Ultrasound Obstet Gynecol 1996; 8:27.
- Sepulveda W, Bower S, Flack NJ, Fisk NM. Discordant iliac and femoral artery flow velocity waveforms in fetuses with single umbilical artery. Am J Obstet Gynecol 1994; 171:521.
- Sepulveda W, Nicolaidis P, Bower S, et al. Common iliac artery flow velocity waveforms in fetuses with a single umbilical artery: a longitudinal study. Br J Obstet Gynaecol 1996; 103:660.
- Catanzarite VA, Hendricks SK, Maida C, et al. Prenatal diagnosis of the two-vessel cord: implications for patient counselling and obstetric management. Ultrasound Obstet Gynecol 1995; 5:98.
- Benirschke K, Kaufmann P. Umbilical cord and major fetal vessels. In: Pathology of the Human Placenta, 2nd ed, Springer-Verlag, New York 1990. p.180.
- Rosenak D, Meizner I. Prenatal sonographic detection of single and double umbilical artery in the same fetus. J Ultrasound Med 1994; 13:995.
- Sepulveda W, Dezerega V, Carstens E, Gutierrez J. Fused umbilical arteries: prenatal sonographic diagnosis and clinical significance. J Ultrasound Med 2001; 20:59.
- Dolkart LA, Reimers FT, Kuonen CA. Discordant umbilical arteries: ultrasonographic and Doppler analysis. Obstet Gynecol 1992; 79:59.
- Sepulveda W, Flack NJ, Bower S, Fisk NM. The value of color Doppler ultrasound in the prenatal diagnosis of hypoplastic umbilical artery. Ultrasound Obstet Gynecol 1994; 4:143.
- Petrikovsky B, Schneider E. Prenatal diagnosis and clinical significance of hypoplastic umbilical artery. Prenat Diagn 1996; 16:938.
- Aoki S, Hata T, Ariyuki Y, et al. Antenatal diagnosis of aberrant umbilical vessels. Gynecol Obstet Invest 1997; 43:232.
- Raio L, Ghezzi F, Di Naro E, et al. The clinical significance of antenatal detection of discordant umbilical arteries. Obstet Gynecol 1998; 91:86.
- Sepulveda W, Reyes M, Gonçalves LF. Two uncommon umbilical vessel anomalies in a fetus with trisomy 18. Prenat Diagn 1998; 18:1098.
- Nyberg DA, Mahony BS, Luthy D, Kapur R. Single umbilical artery. Prenatal detection of concurrent anomalies. J Ultrasound Med 1991; 10:247.
- DeFigueiredo D, Dagklis T, Zidere V, et al. Isolated single umbilical artery: need for specialist fetal echocardiography? Ultrasound Obstet Gynecol 2010; 36:553.
- Hua M, Odibo AO, Macones GA, et al. Single umbilical artery and its associated findings. Obstet Gynecol 2010; 115:930.
- Dagklis T, Defigueiredo D, Staboulidou I, et al. Isolated single umbilical artery and fetal karyotype. Ultrasound Obstet Gynecol 2010; 36:291.
- Martínez-Frías ML, Bermejo E, Rodríguez-Pinilla E, et al. 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.
- Prefumo F, Güven MA, Carvalho JS. Single umbilical artery and congenital heart disease in selected and unselected populations. Ultrasound Obstet Gynecol 2010; 35:552.
- Smith DW. Recognizable patterns of human malformation, 6th, WB Saunders, Philadelphia 2006.
- Chen K, Akoma U, Anderson A, et al. Prenatally diagnosed single umbilical artery: The role and relationship of additional risk factors in the fetus for congenital heart disease. J Clin Ultrasound 2016; 44:113.
- Deshpande SA, Jog S, Watson H, Gornall A. Do babies with isolated single umbilical artery need routine postnatal renal ultrasonography? Arch Dis Child Fetal Neonatal Ed 2009; 94:F265.
- Van den Hof MC, Wilson RD, Diagnostic Imaging Committee, Society of Obstetricians and Gynaecologists of Canada, Genetics Committee, Society of Obstetricians and Gynaecologists of Canada. Fetal soft markers in obstetric ultrasound. J Obstet Gynaecol Can 2005; 27:592.
- Parilla BV, Tamura RK, MacGregor SN, et al. The clinical significance of a single umbilical artery as an isolated finding on prenatal ultrasound. Obstet Gynecol 1995; 85:570.
- Khong TY, George K. Chromosomal abnormalities associated with a single umbilical artery. Prenat Diagn 1992; 12:965.
- Danish Guidelines 2015. Nordic Federation of Societies of Obstetrics and Gynecology. www.nfog.org/guidelines.shtml (Accessed on February 16, 2016).
- Bryan EM, Kohler HG. The missing umbilical artery. II. Paediatric follow-up. Arch Dis Child 1975; 50:714.
- Murphy-Kaulbeck L, Dodds L, Joseph KS, Van den Hof M. Single umbilical artery risk factors and pregnancy outcomes. Obstet Gynecol 2010; 116:843.
- Wiegand S, McKenna DS, Croom C, et al. Serial sonographic growth assessment in pregnancies complicated by an isolated single umbilical artery. Am J Perinatol 2008; 25:149.
- Society for Maternal-Fetal Medicine. Single umbilical artery: What you need to know. Reaffirmed February 1, 2013. https://www.smfm.org/publications/104-single-umbilical-artery-what-you-need-to-know (Accessed on February 16, 2016).
- Sepulveda W, Dezerega V, Carstens E, et al. Risks of funipuncture in fetuses with single umbilical arteries. Obstet Gynecol 2000; 95:557.
- Abdel-Fattah SA, Bartha JL, Kyle PM, et al. Safety of fetal blood sampling by cordocentesis in fetuses with single umbilical arteries. Prenat Diagn 2004; 24:605.
- Froehlich LA, Fujikura T. Follow-up of infants with single umbilical artery. Pediatrics 1973; 52:6.
- Gornall AS, Kurinczuk JJ, Konje JC. Antenatal detection of a single umbilical artery: does it matter? Prenat Diagn 2003; 23:117.
- Burshtein S, Levy A, Holcberg G, et al. Is single umbilical artery an independent risk factor for perinatal mortality? Arch Gynecol Obstet 2011; 283:191.
- Battarbee AN, Palatnik A, Ernst LM, Grobman WA. Association of Isolated Single Umbilical Artery With Small for Gestational Age and Preterm Birth. Obstet Gynecol 2015; 126:760.
- Mailath-Pokorny M, Worda K, Schmid M, et al. Isolated single umbilical artery: evaluating the risk of adverse pregnancy outcome. Eur J Obstet Gynecol Reprod Biol 2015; 184:80.
- Voskamp BJ, Fleurke-Rozema H, Oude-Rengerink K, et al. Relationship of isolated single umbilical artery to fetal growth, aneuploidy and perinatal mortality: systematic review and meta-analysis. Ultrasound Obstet Gynecol 2013; 42:622.
- Ashwal E, Melamed N, Hiersch L, et al. The impact of isolated single umbilical artery on labor and delivery outcome. Prenat Diagn 2014; 34:581.
- Lacro RV, Jones KL, Benirschke K. The umbilical cord twist: origin, direction, and relevance. Am J Obstet Gynecol 1987; 157:833.
- Raio L, Ghezzi F, Di Naro E, et al. Prenatal assessment of Wharton's jelly in umbilical cords with single artery. Ultrasound Obstet Gynecol 1999; 14:42.
- Chetty-John S, Zhang J, Chen Z, et al. Long-term physical and neurologic development in newborn infants with isolated single umbilical artery. Am J Obstet Gynecol 2010; 203:368.e1.
- PREVALENCE AND EPIDEMIOLOGY
- RISK FACTORS
- CLINICAL PRESENTATION
- Normal three-vessel cord
- Single umbilical artery
- Differential diagnosis
- - Fused arteries at the insertion site
- - Hypoplasia of one umbilical artery
- POSTDIAGNOSTIC EVALUATION
- Assessment for associated abnormalities
- Fetal genetic studies
- PREGNANCY MANAGEMENT
- Monitoring for growth restriction
- Antepartum fetal monitoring
- Special issues
- - Cordocentesis
- - First-trimester diagnosis
- PREGNANCY OUTCOME
- Preterm delivery
- Small for gestational age and birth weight
- Perinatal outcome
- Long-term outcome in offspring
- SUMMARY AND RECOMMENDATIONS