Current Perspectives of Prenatal Sonographic Diagnosis and Clinical Management Challenges of Complex Umbilical Cord Entanglement

Abstract Diagnosis of potential umbilical cord compromise, namely, true knots of the umbilical cord and nuchal cords has been enabled with increasing accuracy with current enhanced prenatal sonography. Often an incidental finding at delivery, the incidence of true knots of the umbilical cord has been estimated at between 0.04% and 3% of deliveries. This condition has been reported to account for a 4 to 10-fold increase of stillbirth and perinatal morbidity of 11% of cases. Nuchal cords, commonly observed at the delivery of uncompromised, non-hypoxic non-acidotic newborns occur more frequently with single nuchal cords noted in between 20% and 35% of all deliveries at term. Multiple nuchal cords are considerably less frequent, with decreasing frequencies inverse to the number of nuchal cord loops. While clearly single (and likely double) nuchal cords are almost uniformly associated with favorable neonatal outcomes, emerging data suggest that cases of ≥3 loops of nuchal cords are more likely to be associated with an increased risk of adverse perinatal outcome (either stillbirth or compromised neonatal condition at delivery). We define cases of a true knot of the umbilical cord, cases of ≥3 loops of nuchal cords, any combination of a true knot and nuchal cord, or any umbilical cord entanglement (nuchal or true knot) in the presence of a single umbilical artery, in singleton gestations as complex umbilical cord entanglement. Two concurrent developments, the increase in accuracy of prenatal sonographic diagnosis of complex umbilical cord entanglement and recent data confirming fatal compromise of the umbilical circulation in approximately 20% of cases of stillbirth, suggest that establishing governing body guidelines for reporting of potential umbilical cord compromise, and recommendation of consideration for early-term delivery of select cases, may be warranted. This commentary will address current perspectives of prenatal diagnosis and clinical management challenges of complex umbilical cord entanglement.

[1]  Medically Indicated Late-Preterm and Early-Term Deliveries: ACOG Committee Opinion Summary, Number 818. , 2021, Obstetrics and gynecology.

[2]  C. Spong Indicated Late‐Preterm and Early‐Term Deliveries , 2020 .

[3]  D. Sherer,et al.  Current Perspectives of Prenatal Sonographic Diagnosis and Clinical Management Challenges of Nuchal Cord(s) , 2020, International journal of women's health.

[4]  D. Sherer,et al.  Current Perspectives of Prenatal Sonographic Diagnosis and Clinical Management Challenges of True Knot of the Umbilical Cord , 2020, International journal of women's health.

[5]  G. Saade,et al.  Umbilical Cord Abnormalities and Stillbirth. , 2020, Obstetrics and gynecology.

[6]  W. Sepulveda Antenatal course and perinatal outcome after ultrasound detection of triple nuchal cord: a case series , 2021, The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians.

[7]  I. Kumar,et al.  Structured evaluation and reporting in imaging of placenta and umbilical cord , 2020, Acta radiologica.

[8]  W. Henrich,et al.  Sextuple rings of nuchal cord by breech presentation: a warning sign. , 2019, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[9]  T. Biron-Shental,et al.  Adverse pregnancy outcomes and multiple nuchal cord loops , 2019, Archives of Gynecology and Obstetrics.

[10]  AIUM–ACR–ACOG–SMFM–SRU Practice Parameter for the Performance of Standard Diagnostic Obstetric Ultrasound Examinations , 2018, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[11]  Tsuyoshi Saito,et al.  Multiple part umbilical cord entanglement and neonatal outcomes. , 2018, Taiwanese journal of obstetrics & gynecology.

[12]  E. Nkwabong,et al.  Risk factors for nuchal cord entanglement at delivery , 2018, International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics.

[13]  F. Souki,et al.  One long umbilical cord, four nuchal cord loops and a true knot , 2017, BMJ Case Reports.

[14]  D. Sherer,et al.  Coexisting true umbilical cord knot and nuchal cord: possible cumulative increased risk of adverse perinatal outcome , 2017, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[15]  D. Sherer,et al.  Persistent quadruple nuchal cord throughout the third trimester associated with decelerating fetal growth , 2017, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[16]  J. Laine,et al.  Causes of Stillbirth in Turku, Finland, 2001–2011 , 2017, Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society.

[17]  A. Zaltz,et al.  All Tied Up and Nowhere to Go: Report of a Figure‐eight Umbilical Cord Complex True Knot and Triple Nuchal Cord Detected on Antenatal Sonography , 2016, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[18]  J. Laine,et al.  Causes of stillbirth in Turku, Finland, 2001-2011. , 2016, Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society.

[19]  B. Mišković,et al.  Do we sometimes see too much? Prenatal diagnosis of a true umbilical cord knot. , 2015, European journal of obstetrics, gynecology, and reproductive biology.

[20]  H. Su,et al.  Analysis of intrauterine fetal demise--a hospital-based study in Taiwan over a decade. , 2013, Taiwanese journal of obstetrics & gynecology.

[21]  J. Kingdom,et al.  Complex umbilical cord entanglement , 2012, The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians.

[22]  K. Benirschke,et al.  Placental Histologic Criteria for Diagnosis of Cord Accident: Sensitivity and Specificity , 2012, Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society.

[23]  J. Hoh,et al.  Fetal heart rate parameters and perinatal outcomes in fetuses with nuchal cords , 2012, The journal of obstetrics and gynaecology research.

[24]  Z Alfirevic,et al.  Practice guidelines for performance of the routine mid‐trimester fetal ultrasound scan , 2011, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[25]  P. Dursun,et al.  Perinatal features and umbilical cord blood gases in newborns complicated with nuchal cord. , 2008, The Turkish journal of pediatrics.

[26]  C. Le Ray,et al.  Management of Nuchal Cord With Multiple Loops , 2008, Obstetrics and gynecology.

[27]  T. Boyd,et al.  Placental histologic criteria for umbilical blood flow restriction in unexplained stillbirth. , 2008, Human pathology.

[28]  D. Valsky,et al.  Overburdened and undernourished. , 2007, American journal of obstetrics and gynecology.

[29]  W. Sepulveda,et al.  Three‐Dimensional Power Doppler Sonography in the Prenatal Diagnosis of a True Knot of the Umbilical Cord , 2007, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[30]  S. Duffy,et al.  A difficult delivery associated with a nuchal cord found nine times around the fetal neck , 2007, Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology.

[31]  L. Stempel Beyond the pretty pictures: giving obstetricians just enough (umbilical) cord to hang themselves. , 2006, American journal of obstetrics and gynecology.

[32]  R. Usher,et al.  Obstetrical factors related to nuchal cord , 2006, Acta obstetricia et gynecologica Scandinavica.

[33]  L. Bell,et al.  A case of seven nuchal loops and a review of the literature. , 2005, Missouri medicine.

[34]  D. Sherer,et al.  Recurrent antepartum compression of a single artery double nuchal cord necessitating emergency cesarean delivery. , 2005, American journal of perinatology.

[35]  R. Zimmermann,et al.  Nuchal Cords in Term and Postterm Deliveries—Do We Need to Know? , 2005, Obstetrics and gynecology.

[36]  S. Ramin,et al.  Effects of nuchal cord on birthweight and immediate neonatal outcomes. , 2005, American journal of perinatology.

[37]  O. Ozyuncu,et al.  Nuchal cord type B associated with an excessively long umbilical cord as a cause of stillbirth: a case report. , 2004, Clinical and experimental obstetrics & gynecology.

[38]  C. L. Ramón y Cajal,et al.  Prenatal diagnosis of true knot of the umbilical cord. , 2004, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[39]  J. Clapp,et al.  The natural history of antenatal nuchal cords. , 2003, American journal of obstetrics and gynecology.

[40]  F. Ghezzi,et al.  In-utero characterization of the blood flow in the Hyrtl anastomosis. , 2001, Placenta.

[41]  Y. Qin,et al.  Color ultrasonography: a useful technique in the identification of nuchal cord during labor , 2000, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[42]  F. Ghezzi,et al.  Prenatal assessment of the Hyrtl anastomosis and evaluation of its function: case report. , 1999, Human reproduction.

[43]  D. Sherer,et al.  Prenatal ultrasonographic diagnosis of nuchal cord(s): disregard, inform, monitor or intervene? , 1999, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[44]  A. Vintzileos,et al.  Ultrasound diagnosis of nuchal cord: the gray-scale divot sign. , 1999, Obstetrics and gynecology.

[45]  B. Mueller,et al.  Risk factors and outcomes associated with nuchal cord. A population-based study. , 1999, The Journal of reproductive medicine.

[46]  F. Morgan-Ortiz,et al.  [Evaluation of ultrasound as a diagnostic test in nuchal encirclement by the umbilical cord during labor]. , 1997, Ginecologia y obstetricia de Mexico.

[47]  W. Rayburn,et al.  Nuchal Cord Entanglements and Gestational Age , 1997, American journal of perinatology.

[48]  E. Jauniaux,et al.  Perinatal Features of Pregnancies Complicated by Nuchal Cord , 1995, American journal of perinatology.

[49]  K. Benirschke,et al.  Anatomy and Pathology of the Umbilical Cord and Major Fetal Vessels , 1995 .

[50]  F. Rodesch,et al.  Nuchal cord in normal third‐trimester pregnancy: a color Doppler imaging study , 1992, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[51]  W. F. Miser Outcome of infants born with nuchal cords. , 1992, The Journal of family practice.

[52]  W. Spellacy,et al.  The umbilical cord complications of true knots, nuchal coils, and cords around the body. Report from the collaborative study of cerebral palsy. , 1966, American journal of obstetrics and gynecology.

[53]  J. Selwyn Crawford Cord Around the Neck: Further Analysis of Incidence 1 , 1964, Acta paediatrica.

[54]  A. Dippel MALIGNED UMBILICAL CORD ENTANGLEMENTS. , 1964, American journal of obstetrics and gynecology.

[55]  A. Dippel Maligned umbilical cord entanglements , 1964 .

[56]  J. Selwyn Crawford Cord Round the Neck , 1962 .

[57]  J. Selwyn Crawford Cord round the neck. Incidence and sequelae. , 1962, Acta paediatrica.

[58]  N. J. Eastman,et al.  COILING OF THE UMBILICAL CORD AROUND THE FOETAL NECK , 1957, The Journal of obstetrics and gynaecology of the British Empire.