CASE REVIEW

Umbilical Cord Complications

A Review
Joanna Chan, MD, and Rebecca N. Baergen, MD
DISCUSSION
Abnormalities of the umbilical cord such as knots, velamentous insertion, entanglement, cord prolapse, and abnormal cord length or coiling can lead to decreased blood ow in umbilical vessels and can result in poor perinatal outcome and fetal demise.15 Flow through the umbilical cord can be diminished either by disruption or compression of the umbilical vessels. Damage to the umbilical cord can also occur due to longstanding meconium discharge associated with vascular myonecrosis. Thus, there are multiple etiologies for both umbilical vessel disruption and compression. Umbilical cord compression can lead to physical obstruction of blood ow between the fetus and placenta.17 Compression may occur by direct compression on the cord by the fetus, such as in cases of cord entanglement, or by physical abnormalities of the cord itself, such as knots, abnormal coiling, or abnormal cord length. Generally these conditions are chronic, and thus are associated with fetal growth restriction.7 However, even in the setting of chronic obstruction there may be an acute exacerbation or progression at the time of delivery where the combination of infant movement, fetal descent into the pelvis, and lack of amniotic uid decreases the amount of cushioning available to the cord. Complete obstruction will eventually lead to fetal death, whereas incomplete obstruction can lead to neurologic injury.1,5,8,9 As a general rule, with compression, the umbilical vein will be compressed rst, and to a greater degree than the umbilical arteries, leading to decreased venous return from the placenta and vascular congestion. In severe cases, the compression can lead to hypovolemia and anemia in the fetus. Decreased venous return in this setting has been conrmed by Doppler studies.10 Chronic compression or obstruction also leads to venous stasis, as well as vascular thrombosis in both fetal placental vessels in the placenta and fetus itself.1113 Thrombi most often occur in the chorionic plate vessels and these may often be recognizable on gross examination, particularly when they are more remote. Older thrombi appear as white streaks in the vessels due to the calcication, whereas recent thrombi appear as dark clots within the vessel lumen. Microscopically, thrombi often present as mural thrombi with brin and platelets attached to the vascular wall but also may be occlusive (Fig. 5).

Abstract: Abnormalities of the umbilical cord are associated with poor perinatal outcome and fetal demise. These abnormalities include excessively long or short cord, over and under-coiled cord, true knots, nuchal and body entanglements, velamentous insertion, meconium staining, and cord rupture. These abnormalities in umbilical cord lead to decreased perfusion of the fetus, which is believed to be the mechanism for the poor outcome. Cord abnormalities may help explain some histologic ndings in the placentas of infants with poor perinatal outcome or fetal demise. Key Words: placenta, umbilical cord, cord complications (Pathology Case Reviews 2010;15: 40 44)

CASE REPORT
A 33-year-old gravida 1 para 0 woman presented in active labor at 39 weeks gestation. Her perinatal course had been previously uneventful. Fetal heart rate monitoring showed a non reassuring pattern and delivery was then facilitated with vacuum assistance. The infant was delivered without a heart beat and could not be resuscitated. At delivery there were no congenital anomalies noted but the umbilical cord was noted to be hemorrhagic and disrupted. The placenta was submitted for pathologic examination. On gross examination the placenta measured 18 14 3 cm in greatest dimensions and had a trimmed weight of 350 g. The fetal surface and fetal membranes were discolored green consistent with meconium staining. The umbilical cord was received in 2 fragments and these measured a total of 46 cm in length. The cord diameter measured from 1 to 4 cm. There was a large, recent hematoma within the cord which measured 20 cm in length (Fig. 1). The cord was ruptured in the area of the hematoma. On sectioning of the cord, there was extensive hemorrhage throughout Whartons jelly (Fig. 2) and the umbilical vein showed marked dilatation. The placenta showed unremarkable villous tissue with no infarcts or gross lesions. On microscopic examination of the umbilical cord there was extensive hemorrhage throughout Whartons jelly in the area of the gross hematoma (Fig. 3). Focally, there was an acute inammatory inltrate in the umbilical vessels. In one area there was aneurysmal dilatation of the umbilical vein with marked thinning of the smooth muscle which was conrmed on trichrome stain (Fig. 4). Rupture of the umbilical vein was seen in one section (Fig. 4).

Umbilical Cord Length

The average umbilical cord at the end of a term pregnancy ranges from 55 to 61 cm in length, with a cord less than 35 cm being described as an abnormally short cord, and a cord greater than 70 cm being an excessively long cord.7,14,15 Cord length correlates with fetal activity in utero, short cords being associated with restricted fetal movement and long cords with fetal hyperactivity. Thus, short cords are seen in conditions in which there is little fetal movement such as skeletal dysplasias and neurologic conditions and when there is restriction of fetal movement such as uterine malformations and multiple gestation.3,7 Animal studies have also conrmed this association as those fetuses who are exposed to drugs or conditions that decrease or restrict fetal movement have shorter cords compared with those who have not.3 There also may be a genetic component in that long cords are more common in pregnancies of women who have previously had pregnancies with long cords.3 Despite these

Diagnosis
Aneurysmal dilatation of umbilical vein with rupture and associated umbilical cord hematoma resulting in intrapartum fetal death.

From the Department of Pathology and Laboratory Medicine, Weill-Cornell Medical College, New York, NY. Reprints: Rebecca N. Baergen, MD, 520 East 70th St, Starr Pavilion, 1002, New York, NY 10065. E-mail: rbaergen@med.cornell.edu. Copyright 2010 by Lippincott Williams & Wilkins ISSN: 1082-9784/10/1502-0040 DOI: 10.1097/PCR.0b013e3181dcdf0f

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Umbilical Cord Complications

FIGURE 1. Gross photograph of umbilical cord with fusiform enlargement and massive engorgement of blood. Note the rupture in the central portion of the hematoma. FIGURE 3. Low power photomicrograph of the umbilical cord. Two vessels are identified and extensive recent hemorrhage is present within Whartons jelly.

during birth may lead to the IQ decit. The complications associated with short cords can also be seen in functionally short cords, which are caused by cord entanglement. Excessively long cords have an increased incidence of other cord problems such as true knots, excessive coiling, cord entanglement, and single umbilical artery.3 These complications, along with the presence of a long cord alone, can lead to decreased ow through the umbilical vessels. It has been hypothesized that simply by having a longer cord, increased perfusion pressure is necessary. Placentas with an excessively long cord and no other cord complications have signs of intrauterine hypoxia, such as chorangiosis and increased nucleated red blood cells.3,17 Fetuses with excessively long cords have an increased incidence of fetal distress, intrauterine growth restriction, fetal demise and long-term neurologic impairment.3 FIGURE 2. Cross-section of umbilical cord with all 3 umbilical vessels visible. The umbilical vein is markedly dilated but in this cross-section appears intact. Whartons jelly shows marked hemorrhage. associations, the complete picture has yet to be elucidated. However, it is clear that both excessively long and excessively short cords are associated with adverse outcome. Abnormally short cords have an increased incidence of cord rupture, premature separation, and abruption.7 If the placenta is implanted in the fundus, which is the most common site, a cord that is less than 35 cm will not allow for a vaginal delivery in the vertex position. Failure to descend is a common complication of short cords but cord rupture, placental abruption or even uterine inversion can occur. In addition, if a placenta accreta is present, there is abnormal adherence of the placenta to the uterine wall and these complications, particularly cord rupture or uterine inversion, are more likely. Fatal bleeding from mother or infant can occur, the sequelae of which includes severe neurologic damage or fetal death.6 Short cords have also been associated with fetal distress, low Apgar scores, and depressed intelligence quotient (IQ).16 In the instance of lowered IQ, it difcult to determine what the causative relationship is, as infants with a depressed IQ are known to have decreased fetal movement, which may have led to the short cord; however, the neurologic damage that can be caused by short cord complications 2010 Lippincott Williams & Wilkins

Umbilical Cord Coiling

Umbilical cord coiling refers to spiraling or twisting of the umbilical cord. A coiling index has been developed and is calculated by taking the number of twists per length of cord. The normal cord coil index is approximately one coil/5 cm, corresponding to a coiling index of 0.2.2 Similarly to cord length, it is thought that coiling is associated with in utero movement of the fetus, with decreased coiling being associated with decreased fetal movement and increased coiling being associated with fetal rotation and activity.3,6,8,14 A decreased coiling index is associated with fetal distress, fetal anomalies, chromosomal errors, and increased fetal and perinatal mortality.18 22 Again, in a situation analogous to short umbilical cord length, the causative relationships are complicated. It is hypothesized that undercoiled cords are more likely to be compressed by the fetus, leading to malperfusion. Currently, it is unclear whether the defects intrinsic to the fetus lead to decreased movement and decreased coiling, or if the undercoiled cord caused malperfusion leading to damage of the fetus. Umbilical cords with an increased coiling index are also associated with adverse outcome such as fetal asphyxia, small for gestational age infants and fetal demise.8,22 Hypercoiled cords often have focal areas of diminished diameter or strictures, which may show congestion of the cord and vessels on one side of the stricture point, thrombi in umbilical vessels, or distention of the umbilical vein.7 These stricture points are commonly found at the insertion of www.pathologycasereviews.com | 41

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FIGURE 4. A, Section of umbilical vein showing marked thinning, aneurysmal dilatation and loss of smooth muscle. Note that the wall consists almost entirely of fibrin. B, Trichome stain of umbilical vein showing loss of smooth muscle and a markedly thinned vascular wall, particularly on the right of the figure. common nding of acute compression is marked distension of the umbilical vein and chorionic plate vessels. Villous capillary congestion may also occur although these ndings are not specic for clinically signicant cord compression. When a knot that has been present for an extended amount of time is untied, the cord will remain curled, whereas a recently formed knot will lie at. If there has been a signicant chronic obstruction of blood ow, thrombi can develop in the fetal circulation. Thus, as in other cord complications, the pathologist should search for and document thrombi in the chorionic plate and stem vessels. This is particularly important in the setting of adverse outcome.

Umbilical Cord Entanglement

Cord entanglement is usually divided into nuchal cords and entanglement with other body parts, with nuchal cord being the most common entanglement. As previously mentioned, cord entanglements are more common with excessively long umbilical cords. Most cord entanglements have no adverse outcomes, as many of them are loose entanglements with no compression of the umbilical vessels. Similarly to true knots, entanglements can tighten with fetal movement or descent. When looking at outcomes of tight nuchal cords only, there is an association with low Apgar scores and increased frequency of stillbirth.23 If the nuchal cord is tight, there can be compression of the umbilical vein, which is more distensable than the arteries, leading to obstruction of venous return to the extent that oxygenated blood is not returned to the fetus. In these situations there may be a signicant anemia and hypovolemic shock has been reported.25,26

FIGURE 5. Stem vessel with fibrin thrombus within the wall of the vessel. Note how the thrombus protrudes into the lumen of the vessel. These mural thrombi are often referred to an intimal fibrin cushions due to this effect. the cord into the fetus and are also an important cause of fetal demise.

Umbilical Cord Knots

False knots are simply focal varicosities or redundancies of umbilical vessels and have no known clinical consequences. On the other hand, true knots are associated with adverse outcome, ostensibly due to compression of umbilical vessels. The frequency of true knots ranges from 0.3% to 2.1%.23 This frequency is higher in long or excessively coiled cords. True knots are associated with fetal distress and antepartum fetal death.24 They are thought to develop weeks or months before term when the fetus has the space in the amniotic cavity to become entangled. In fact, fetal demise due to a tight true knot does occur in the second or even rst trimester. However, many knots begin relatively loose but then tighten with fetal descent during the latter part of pregnancy and particularly during labor and delivery. This can lead to signicant tightening, obstruction of blood ow and even excessive traction on the cord. Tight knots cause compression of the Whartons jelly, leading to placental and umbilical vein congestion, as well as thrombosis. A 42 | www.pathologycasereviews.com

Velamentous Vessels and Velamentous Insertion

Velamentous insertion of cord occurs in approximately 1% of placentas.4,7 They are more common in twins and in high multiples. Due to loss of Whartons jelly, which protects the umbilical vessels, velamentous vessels are susceptible to thrombosis, compression, disruption, and trauma. These vessels are particularly susceptible after membrane rupture, when the cushioning protection of the amniotic uid is also lost. Velamentous vessels are not limited only to velamentous cord insertion, they can also be seen in multilobed placentas and in marginal insertions. The same susceptibility applies to these velamentous vessels. The longer the distance the velamentous vessels travel throughout the membranes, the higher the chance there is of vessel damage or rupture. In vasa previa, velamentous vessels cross the cervical os, and may be disrupted in a vaginal delivery.27,28 Velamentous vessel rupture has a high mortality rate which is estimated to be from 58% to 73%. In less severe cases, other consequences include fetal distress, hypoxia, and neurologic 2010 Lippincott Williams & Wilkins

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Umbilical Cord Complications

to underperfusion of the placenta and fetus, either by cord vessel disruption or compression. Evidence of compression is evidenced histologically by signs of venous stasis, such as intimal brous cushions and thrombi in fetal and placental vessels. Both cord disruption and compression can lead to decreased perfusion of the placenta, evidence of which includes avascular villi, villous infarcts and Tenney-Parker changes (multifocal villous agglutination, increased syncytial knots). Understanding cord complications may help clinicians and pathologists correlate histologic ndings in the placenta to cord abnormalities and clinical outcomes. REFERENCES
1. Redline RW, ORiordan A. Placental lesions associated with cerebral palsy and neurologic impairment following term birth. Arch Pathol Lab Med. 2000;124:17851791. 2. Machin GA, Ackerman J, Gilbert-Barness E. Abnormal umbilical cord coiling is associated with adverse perinatal outcomes. Pediatr Dev Pathol. 2000;3:452 471. 3. Baergen RN, Malicki D, Behling C, et al. Morbidity, mortality, and placental pathology in excessively long umbilical cords: retrospective study. Pediatr Dev Pathol. 2001;4:144 153. 4. Bernischke K, Kaufmann P, Baergen RN. Pathology of The Human Placenta. 5th ed. New York, NY: Springer; 2006. 5. Grafe MR. The correlation of prenatal brain damage with placental pathology. J Neuropathol Exp Neurol. 1994;53:407 415. 6. Miller ME, Higginbottom M, Smith DW. Short umbilical cord: its origin and relevance. Pediatrics. 1981;67:618 621. 7. Baergen RN. Manual of Bernischke and Kaufmanns Pathology of The Human Placenta. 5th ed. New York, NY: Springer; 2005. 8. Peng HQ, Levitin-Smith M, Rochelson B, et al. Umbilical cord stricture and overcoiling are common causes of fetal demise. Pediatr Dev Pathol. 2006; 9:14 19. 9. Murpy DJ, MacKenzie IZ. The mortality and morbidity associated with umbilical cord prolapse. Br J Obstet Gynaecol. 1995;102:826 830. 10. Gembruch U, Baschat AA. True knot of the umbilical cord: transient constrictive effect to umbilical venous blood ow demonstrated by Doppler sonography. Ultrasound Obstet Gynecol. 1996;8:5356. 11. Redline RW. Clinical and pathological umbiloical cord abnormalities in fetal thrombotic vasculopathy. Hum Pathol. 2004;35:1494 1498. 12. Kraus FT, Acheen VI. Fetal thrombotic vasculopathy in the placenta: cerebral thrombi and infarcts, coagulopathies and cerebral palsy. Hum Pathol. 1999; 30:759 769. 13. Heifetz SA. Thrombosis of the umbilical cord: analysis of 52 cases and literature review. Pediatr Pathol. 1988;8:3754. 14. Naeye RL. Umbilical cord length: clinical signicance. J Paediatr. 1985;107: 278 281. 15. Gardiner JP. The umbilical cord: normal length; length in cord complications: etiology and frequency of coiling. Surg Gynecol Obstet. 1922;34:252256. 16. Bain C, Eliot BW. Fetal distress in the rst stage of labor associated with early fetal heart rate decelerations and a short umbilical cord. Aust N Z J Obstet Gynaecol. 1957;16:5156. 17. Rayburn WF, Beynen A, Brinkman DV. Umbilical cord length and intrapartum complications. Obstet Gynecol. 1981;57:450 452. 18. Kalish RB, Hunter T, Sharma G, et al. Clinical signicance of the umbilical cord twist. Am J Obstet Gynecol. 2003;189:736 793. 19. Strong TH, Elliott JP, Radin TR. Non-coiled umbilical blood vessels: a new marker for the fetus at risk. Obstet Gynecol. 1993;81:409 411. 20. de Laat MW, van Alderen ED, Franx A, et al. The umbilical coiling index in complicated pregnancy. Eur J Obstet Gynecol Reprod Biol. 2007;130:66 72. 21. Bernischke K. Obstetrically important lesions of the umbilical cord. J Reprod Med. 1994;39:262272. 22. de Laat MW, Franx A, Bots M, et al. Umbilical coiling index in normal and complicated pregnancies. Obstet Gynecol. 2006;107:1049 1055. 23. Spellacy WN, Gravem H, Fisch RO. The umbilical cord complications of true knots, nuchal coils, and cord around the body. Am J Obstet Gynecol. 1966;94:1136 1142. 24. Hershkovitz R, Silberstein T, Sheiner E, et al. Risk factors associated with true knots of the umbilical cord. Eur J Obstet Gynecol Reprod Biol. 2001; 98:36 39.

FIGURE 6. High power view of umbilical artery in a case of meconium staining. The vascular lumen is at the right and the muscle cells of the wall are rounded up, with eosinophilic cytoplasm and rounded pyknotic nuclei consistent with myonecrosis. Some meconium filled macrophages can be seen on the left of the figure and the umbilical cord was grossly meconium stained.

injury. Thrombosis of the velamentous vessels has been also associated with neonatal purpura and fetal death.

Meconium and Umbilical Cord Myonecrosis

Meconium is the intestinal content of the fetus. Meconium discharge into the amniotic uid is a common event, particularly in term and post term infants. In most cases, it is not associated with signicant poor outcome. However, meconium is a noxious material and damages the amniotic sac lining and may damage the fetal lung if aspirated. In addition, prolonged meconium exposure can lead to damage of the umbilical cord muscle.29 Furthermore, in vitro studies have shown that meconium causes vasoconstriction of umbilical vessels.29 Myonecrosis of umbilical vessels usually affects the umbilical arteries presumably because they are closer to the surface (Fig. 6). Meconium can also lead to ulceration and even rupture of the umbilical cord. The combination of arterial necrosis and cord ulceration has also been described in association with meconium discharge in infants with hypoxic ischemic encephalopathy.30

Umbilical Cord Rupture

Cord rupture is associated with functionally short and short cords, velamentous vessels, necrotizing funisitis and meconium induced myonecrosis. During a vaginal delivery, functionally short and short cords can avulse from the placenta. Spontaneous cord rupture is extremely rare.31,32 The most common site of cord rupture is at the site of insertion. Cord rupture is always associated with a signicant hematoma as was seen in this case and is associated with signicant fetal morbidity and mortality.

Summary
Cord abnormalities are associated with adverse perinatal outcome, such as neurologic decits and fetal demise. While in some cases it is difcult to determine whether a pre-existing central nervous system defect led to the cord abnormality, as discussed in reference to abnormally short cord and undercoiled cords, in many cases it is presumed that the cord abnormality led 2010 Lippincott Williams & Wilkins

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25. Shepard AJ, Richardson CJ, Brown JP. Nuchal cords as a cause of neonatal anemia. Am J Dis Child. 1985;139:7173. 26. Vanhaesebrouck P, Vanneste K, de Praeter C, et al. Tight nuchal cord and neonatal hypovloemic shock. Arch Dis Child. 1987;62:1276 1277. 27. Torrey WE Jr. Vasa previa. Am J Obstet Gynecol. 1952;63:146 152. 28. Cordero DR, Helfgott AW, Landy HJ, et al. A non-hemorrhagic manifestation of vasa previa: a clincopathologic case report. Obstet Gynecol. 1993;82:698 700. 29. Altshuler G, Hyde S. Meconium induced vasoconstriction: a potential cause

of cerebral and other fetal hypoperfusion and of poor pregnancy outcome. J Child Neurol. 1989;4:137142. 30. Khurana A, Huettner PC, Coles FS. Umbilical cord ulceration as a cause of hypoxic-ischemic encephalopathy: report of a case and review of the literature. J Perinatol. 1995;15:423 425. 31. Bahary CM, Gabbai M, Eckerling B. Rupture of the umbilical cord: report of a case. Obstet Gynecol. 1965;26:130 132. 32. Sidall RS. Spontaneous rupture of the umbilical cord. Am J Obstet Gynecol. 1925;10:836 840.

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