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Archives of Perinatal Medicine 20(1), 22-27, 2014

ORIGINAL PAPER

Umbilical cord abnormalities in fetal


and neonatal pathology in Bogota
MERCEDES OLAYA1, J. E. BERNAL2
Abstract

Introduction: Analysis was carried out on cases of fetal and perinatal mortality from 2007-2011, on fetuses and

newborns in the Pathology Department at San Ignacio University Hospital (HUSI), in Bogota. Typical characteristics of the umbilical cord, their abnormalities and clinical outcomes were studied. Methods: A prospective
study was performed for neonatal and fetal autopsies; placentas were also studied, and HUSI clinical cases were
reviewed. Results: A total of 914 cases including fetuses, embryos, placentas and newborns made up the study.
323 cases (35.33%) had umbilical cord abnormalities: length abnormalities (7.87%): short (3.39%), long (4.48%);
abnormal insertion (23.63%): velamentous (4.04%), furcate (0.43%), marginal- paramarginal (19.14%); coiled cords
(6.01%): hypocoiled (0.65%), hypercoiled (5.36%); number of vessels (single umbilical artery) (0.43%); entanglements in fetal parts (0.54%); true knots (0.21%). In 4.81% more than one alteration was found: hypercoiled, the
most frequent, appeared in six cases with marginal insertion, in five cases with long cords, in four cases with short
cords. Cause of death attributable to umbilical cord (umbilical cord collision) occurred in 3.82%; there were
thrombosis of the fetal circulation in 6,6%. Umbilical cord abnormalities were significantly associated with chromosomal abnormalities (p < 0.003, OR 2.53), multiple gestations (OR 5.78, 59.3%) and maternal hypertension
(mainly preeclampsia) (p < 0.018, OR 1.85). Conclussions: Every anatomical feature of the umbilical cord has
a meaning. The abnormalities of the umbilical cord associated with undesirable outcomes are varied and should
be recognized and described. Among the factors recognized as predisposing are chromosomal abnormalities,
multiple gestations and as a like a new item, we found preeclampsia.
Key words: stillbirth, umbilical cord, cord accident, fetal thrombotic vasculopathy, intrauterine growth restriction
(IUGR), preeclampsia.

Introduction
Infant mortality is considered to be one of the pillars
for planning and programming health related activities.
It is considered to be a universal indicator for both living
conditions and the level of quality and access to health
services in any country. Neonatal mortality greatly influences infant mortality. Moreover, many handicaps of
neurological origin are attributable to complications
during birth. Umbilical cord abnormalities are observed
in around 35% of spontaneous abortions [1]; in stillbirth
autopsies, fetal circulation compromise is suspected in
at least 20% [2]. Intrauterine deaths originating from umbilical cord abnormalities are estimated at around 22.5%
[3]. Umbilical cord collisions are considered to be the
main cause of intrauterine anoxia in 28.8% [4]. This importance given to the umbilical cord is understandable
since it is the vital extension of the fetal circulatory system, which regulates blood flow, in both directions, thus
allowing for mother child immunological communication, oxygenation, fetal nutrition and in utero cardiac
1

function. Abnormal umbilical cord length (both excessive


and deficient) is considered to be the main factor that
predisposes peripartum complications related to the umbilical cord [5]. It is estimated that excessively long umbilical cords occur in 4% to 6% of cases while abnormally
short cords occur in approximately 1% to 2% [6]. Factors
that determine abnormalities in the morphology and
functioning of the umbilical cord are unknown. This work
analyzed fetal and neonatal mortality between 2007-2011
on fetuses and newborns in the Pathology Department
at San Ignacio University Hospital (HUSI), in Bogota.
Typical characteristics of the umbilical cord, their abnormalities and clinical outcomes were studied.
Methods
A prospective study on a series of perinatal autopsy
cases was performed to describe anatomic characteristics of the umbilical cord and its relation to maternal,
fetal and newborn clinical factors. Autopsies had parental

Department of Pathology, Medical School, Pontificia Universidad Javeriana San Ignacio University Hospital, Bogot, Colombia
2
Institute of Human Genetics, the Medical School, Pontificia Universidad Javeriana, Bogota, Colombia

Umbilical cord abnormalities

consent and all corresponded to natural deaths. Results


from 413 autopsies were analyzed and related to the individuals diseases, placenta, in utero life and delivery.
The autopsies also included a complete study of placenta
characteristics, including those of the umbilical cord:
length (which was compared to tables available in relevant literature; for short cords it was necessary to have
the complete umbilical cord; for long cords, fragments of
separated cords could be joined), insertion (velamentous, furcate, marginal and paramarginal inserted within 1 cm from the placental edge were considered abnormal), coiled cords (normal coiling index between 0.07
and 0.3 coils per cm), number of cord vessels (two or
three or other), entanglements (cord tangles in fetal
body parts) and true umbilical cord knots. Microscopic
abnormalities were also observed in all organs and the
placenta. Maternal diseases were searched for in medical records along with information on pregnancy, birth
and neonatal adaptation complications. Primary and secondary diagnoses were extracted from fetal or newborn
autopsy protocols, as were placenta alteration diagnoses,
including those for the umbilical cord.

23

Fig. 1. Comparison between abnormal umbilical cord


coiling: hypercoiled (top) and hypocoiled (bottom)

Results
Between 2007 and 2011 there were 914 perinatal
cases including fetuses, embryos, placentas and newborns at HUSI. There were 487 (53.27%) placentas,
which arrived individually (because the children were alive or because the parents did not accept post mortem
examination); 413 (45.18%) cases of fetuses and newborns and 14 cases (1.53%) of embryos. Of all of these,
323 cases (35.33%) presented umbilical cord abnormalities, divided among 72 cases (7.87%) of length abnormalities: 41 long cord cases (4.48%), 31 short cord
cases (3.39%); 216 cases of abnormal insertion (23.63%):
37 cases of velamentous insertion (4.04%); 4 cases of
furcate insertion (0.43%), 175 cases of marginal and paramarginal insertions (19.14%); abnormal cord coiling in
55 cases (6.01%): 6 hypocoiled cases (0.65%) and 49
cases of hypercoiled cords (5.36%) (Fig 1); number of
vessels: 4 bivascular cords (0.43%) (Fig 2); entanglements in the fetal body: 5 cases (0.54%) and true knots:
2 cases (0.21%).Presented above abnormalities are summarized in Fig 3. In 44 cases (4.81%) there was more
than one alteration. The most frequent association was
hypercoiling: marginal insertion in 6 cases, long cords in
5 cases and short cords in 4 cases.
The cause of death was attributable to the umbilical
cord (umbilical cord collision) in 35 cases (3.82%). Umbilical cord abnormality was significantly associated with

Fig. 2. Bivascular umbilical cord (umbilical cord artery (SUA))


Entaglements, 0.54
Shortlength, 3.39
Bivasculars, 0.43
Trucknots, 0.21
Long length, 4.48 Velamentous
Hypercoiling, 5.36
insertion,
Hypocoiling, 0.65
4.04

Furcate
insertion, 0.43
Marginal and paramarginal insertion, 19.14

Fig. 3. Umbilical cord abnormality distribution


HUSI 2007-2011 Bogota, Colombia

chromosomal disorders (25 cases (p < 0.003, OR 2.53))


and with maternal hypertensive disease (primarily preeclampsia) (p < 0.018, OR 1.85). There was no statistical significance for association with placental hypoxic-ischemic disorders (p < 0.7), chronic villitis (p < 0.20) or
ascending infections. Multiple pregnancies had a great

24

M. Olaya, J.E. Bernal

risk of having umbilical cord abnormalities (OR 5.78,


59.3%).
Discussion
Reproduction is one of the most important evolutionary elements and the placenta and umbilical cord are
protagonists for mammal reproduction; it can be inferred
that this has been successful given that there are currently only two families of mammals that lay eggs (Theria) and 18 families of marsupials (Metatheria). In contrast, there are 115 families of placental mammals
(Eutheria) with 4,486 species [7]. In addition to having
resisted millions of years of evolution, uterine development with placenta has involved a greater number of
species, which suggests evolutionary advantages for survival and demonstrates reproductive efficiency for a large group of animals denominated as superior. Because
life in the uterus literally hangs on the umbilical cord,
its structure has had to adapt itself to the needs of each
type of creature given that adverse intrauterine microenvironments can lead to fetal death, peripartum complications, neonatal death, neurological sequelae; it also
plays a recognized role in the development of certain
adulthood diseases [8-16]. The umbilical cord is an evolutionary adaptation at risk for its apparently exposed
vessels that literally transport life. Umbilical cord abnormalities can be readily evident on macroscopic examination as an accident such as in the case of true knots;
however, less obvious abnormalities such as vascular occlusion even intermittent can lead to stillbirth or neurological damage. Compression and vasospasms are
important factors in the Unsatisfactory Fetal State,
a dangerous medical condition for the fetus. Umbilical
cord collision rate is variable in the literature. We found
a 3.82% rate in 413 cases, which is comparable to Ovalle
[17] which reports a 4.3% rate from 279 autopsies. However, Horn [3] reported a 22.5% rate of umbilical cord
collisions (deaths attributable to the umbilical cord) in
310 autopsies. There were seven cases classified as Unsatisfactory Fetal State which were related to umbilical
cord abnormalities (one paramarginal, one velamentous
and five marginal, two of which concomitantly showed
hypercoiling). Umbilical cord abnormalities are now
beginning to be studied during the fetal stage, rather
than from delivery, with new imaging technology which
correlates them to fetal development and well-being [18].
Umbilical cord characteristics:
Length: Average umbilical cord length for term fetuses
is 55 cm [19, 20]. Tables exist that consolidate cord

length for each fetal age given the fact that it grows asynchronously throughout pregnancy; this is faster prior to
week 28. Long cords: Excessively long cords in our
study represented 4.48%, which corresponds to figures
reported in relevant literature. Long cords have been
associated with entanglement (in any part of the body),
narrowness, increased coiling, reverse coiling, true
knots, single artery and thrombosis [5, 21, 22]. The
latter is related to neonatal and fetal mortality and poor
neurological outcomes [23, 24]. Excessive cord length
requires greater perfusion pressure those resulting in
greater resistance against flow. This in turn demands
greater myocardial effort, which may induce fetal cardiac
abnormalities such as ventricular hypertrophy and
cardiomegaly [21, 25] with signs of intrauterine hypoxia,
IUGR, degenerative cerebral changes, altered imaging
studies, neurological damage and poor fetal prognosis
[21]. These neurological abnormalities have been explained through germinal matrix or intraventricular hemorrhage, gliosis or necrosis of the white matter and
neuronal necrosis [26], neurological complications which
come together in fetal thrombotic vasculopathy [27, 28].
In our study 61 cases were found with fetal circulation
obstruction and thrombi were observed in cord vessels
or chorionic vessels; these abnormalities were associated
with long cords (4 cases), short cords (4 cases), hypercoiled cords (9 cases) hypocoiled cords (1 case), bivascular cord (1 case) and abnormal cord insertion into the
placenta (42 cases).
In autopsies with no apparent cause of death, frequently the only finding may have been abnormal umbilical cord length [21]. Additionally, there are obstetric
complications such as umbilical cord prolapse and true
cord knots. In our study the long cords were not associated with true knots or with observed entanglements.
Short cords: Cords shorter than 32 cm at the end of
pregnancy were considered abnormally short, which implies increased risk of complications during birth and
fetal morphological abnormalities [15]; these have an
approximate incidence rate of 1% to 2% [6]; our study
showed a rate of 3.39%. They have a high association
rate with fetal abnormalities, such as abdominal wall defects and defects in the extremities and spine [20] (One
of our short cords corresponded to limb-body wall
complex). They are also associated with unsatisfactory
fetal state, central nervous system complications and low
APGAR and IQ scores [29]. Obstetric complications
associated with short umbilical cords include: cord rupture, abruptio placentae, uterine inversion, cord hematomas and fetal descent disorders during labor.

Umbilical cord abnormalities

True umbilical cord knots: These correspond to knots


made on the umbilical cord with an average incidence of
1% [30]; in our study we observed just 0.21%. The accompanying venous stasis leads to thrombosis and is
associated with unsatisfactory fetal state, fetal hypoxia,
perinatal mortality and neurological damage [21].
Umbilical cord insertion in the placenta: Umbilical cord
insertion in the chorionic plate should be central or paracentral, although on occasion it is abnormal, for example, when there is furcate insertion, which refers to early
loss of Wharton jelly, leaving umbilical vessels exposed;
marginal insertion (and its para-marginal variant defined
as 1 cm or less of the placental edge), when the cord
arrives up to the placental edge; and, velamentous insertion which is when the cord reaches the membranes.
Abnormal insertions are related to IUGR, preterm delivery, abnormal intrapartum fetal heart rate, low APGAR
(at one minute and at 5 minutes), neonatal mortality and
abruptio placentae [31]. Marginal insertion has an
incidence in term placentas of 7% whereas velamentous
has 1% [21]; we observed 19.14% and 4.04%, respectively. In twin pregnancies more umbilical cord abnormalities are described such as velamentous insertions (9
times more frequent) and marginal insertions (twice as
frequent); we observed 6 and 20 cases respectively.
Hypocoiling and single umbilical artery in twins are also
described as more frequent [32]; we did not observe any
of these in HUSI cases. Velamentous insertion is related
to IUGR [14] and can be complicated by vasa previa
which occurs during vaginal birth when the vessels precede the fetus.
Umbilical cords entanglements: Umbilical cord entanglements are most often present around the neck (15%
to 20%), but they may also occur around the extremities
or other parts of the body and may be tight or not. Tight
entanglements are those that have the severest consequences for the fetus. Additionally, entanglements can
be single or multiple. The result is related to low
APGAR and stillbirth. Seventy-five percent of entanglements around the neck, that last for four weeks or more
have been associated with asymmetric IUGR [33].
Minimal histological criteria have been defined for the
placenta in order to correlate umbilical cord accidents
with villous repercussion and its relationship to fetal
mortality [34]. These criteria include: vascular ectasia
and thrombosis, which can be found on the umbilical
cord, on the chorionic plate and/or the stem villus; and
regional distribution of avascular villi or villi with stromal
karyorrhexis [35]. Among the cases we observed, one
corresponded to a deformed fetus (thanatophoric dys-

25

plasia) from a drug-dependent mother. The other died


from placental insufficiency (massive deposits of intervillous fibrin).
Umbilical cord coiling: Umbilical cord coiling serves
a physiological purpose as it protects against forces that
could compress or distend the cord. Its origin is unknown and it usually occurs with both arteries over the
vein to the left; if it goes in the other direction it is considered contrary which is associated, among other
things, with placenta previa [36]. Umbilical hypercoiling
is associated with fetal mortality [37], preterm birth, low
umbilical artery pH, chromosomal or structural abnormalities, fetal placental vessel thrombosis, hypoxia/chronic fetal ischemia and low fetal weight [38]. Hypocoiled
umbilical cords are associated with meconium staining,
induced delivery, low APGAR, abnormalities to fetal pH
level and chromosomal abnormalities [37, 39]. In cases
of fetal death, abnormal coiling is twice as frequent as in
cases with no fetal death [40]. Additionally, Otsubo et al.
[40] described a high percentage of abnormal cord insertions (velamentous and marginal) with hypocoiled
cords. Both, increased and decreased coiling, increse the
possibility of complications and adverse perinatal outcome [39, 41]. At HUSI we observed hypercoiling associated with preeclampsia (7 cases) and chromosomal disorders (9 cases). Hypocoiling was associated with chromosomal abnormalities in 1 case, cord accident in 1 case
and VTF in 2 cases. Fetal thrombotic vasculopathy occurred in 61 cases: short umbilical cord, 4; bivascular
cord, 1; hypercoiled cord, 9; hypocoiled cord, 1 and abnormal insertions, 42.
According to the literature, umbilical cord abnormalities were significantly associated with chromosomal disorders (25 cases (p < 0.003, OR 2.53)) and with twin
pregnancies (OR 5.78, 59.3%). In this study maternal hypertensive disease (mainly preeclampsia), which
has not previously been described elsewhere, was also
strongly associated with umbilical cord abnormalities
(p < 0.018, OR 1.85).
At present, factors that determine umbilical cord features, such as insertion or length, are unknown. The relationship to preeclampsia that we are describing might
be associated with immunologic aspects that have been
reported in primipaternity [42, 43]; our group also reports primipaternity associated with abnormal long cord
[44].
Conclusions
The different variables that can affect the health of
the fetus and the newborn have acquired particular im-

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M. Olaya, J.E. Bernal

portance over the last few years given the interest in identifying them and, eventually, modifying them. Furthermore, the recognition of severe neurological sequelae originating from an inappropriate neonatal period has also contributed to an increase in lawsuits for medical malpractice
stemming from unfortunate peripartum outcomes.
Each anatomical umbilical cord characteristic has
had evolutionary significance and plays an important role
in successfully contributing to reproduction for the
preservation of the species.
Risk factors associated with umbilical cord abnormalities, such as twin pregnancies and chromosomal disorders, are well known. Additionally, in this study we
found significant association of these abnormalities with
pregnancy hypertensive disorders. Therefore, in fetal
and neonatal autopsies, as well as in newborn examination, strict attention must be paid to umbilical cord features, given the fact that they are highly important in
newborn diagnoses, counseling on reproductive aspects
and can provide answers to unexpected adverse outcomes such as neurological abnormalities and neonatal
mortality.
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Mercedes Olaya
Pontificia Universidad Javeriana
Facultad de Medicina, Departmento de Patologa
Carrera 7a 40-62 Bogota, Colombia
e-mails: olaya.m@javeriana.edu.co

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