Pediatric and Developmental Pathology 9, 14–19, 2006

DOI: 10.2350/05-05-0051.1
ª 2006 Society for Pediatric Pathology

ORIGINAL ARTICLES

Umbilical Cord Stricture and Overcoiling

Are Common Causes of Fetal Demise
HONG QI PENG,1 MICHELLE SMITH-LEVITIN,2 BURTON ROCHELSON,2 AND ELLEN KAHN1*
1
Department of Pathology, North Shore University Hospital, New York University School of Medicine,
300 Community Drive, Manhasset, NY 11030, USA
2
Department of Obstetrics and Gynecology, North Shore University Hospital, New York University School of Medicine,
Manhasset, NY 11030, USA

Received September 28, 2005; accepted May 31, 2005; published online April 4, 2006.

ABSTRACT entities commonly causing fetal demise. This observation

Although umbilical cord stricture and umbilical cord
overcoiling have been established as causes of intrauterine
fetal demise, relatively few studies addressed this issue,
most of them being case reports. We reviewed a total of 268
fetal autopsies during a 3-year period from 1998 to 2001.
One hundred thirty nine cases of fetal demise including
spontaneous abortion were identified. Nineteen percent (26
of 139) were associated with umbilical cord stricture,
overcoiling, or a combination of both. Stricture of the
umbilical cord was defined as a decrease in diameter in
relation of the remaining umbilical cord; overcoiling as 0.3
coil/cm or greater. Fetal demise most commonly occurred in
the second trimester, with a mean gestation age of 21 weeks.
The average maternal age was 33 years; 15% had a prior
fetal demise. We found that 77% (20 of 26) of these cases
had umbilical cord stricture only or with overcoiling, 23% (6
of 26) had umbilical cord overcoiling alone. Localized
deficiency of Wharton’s jelly and increased collagen were
found in all cases with umbilical cord stricture with or
without overcoiling. In patients with umbilical cord over-
coiling alone, 25% had Wharton’s jelly deficiency; half of
them had increased collagen deposition in the umbilical
cords. The placenta was reviewed for secondary thrombosis
of the vessels of the chorionic plate. Thrombosis of the
vessels of the chorionic plate was noted in 54% of the
patients. Our study suggests that umbilical cord stricture and
cord overcoiling may represent two distinct pathological
*Corresponding author, e-mail: ekahn@nshs.edu
reinforces the importance of a fetal autopsy with careful
examination of the placenta and umbilical cord with
documentation of the cord coil index.
Key words: coiling, fetal demise, stricture, thrombosis,
umbilical cord
INTRODUCTION
Fetal demise constitutes a major cause of perinatal
mortality. However, even by the latest listing of
causes using the ‘‘Extended Wigglesworth classi-
fication,’’ no etiology for death was found in 60%
of stillbirths [1]. We have noted that a significant
number of fetal demises were associated with either
umbilical cord stricture, umbilical cord overcoiling,
or a combination of both.
Although umbilical cord stricture and umbil-
ical cord overcoiling have been established as
causes of intrauterine fetal demise [2 4], relatively
few studies have addressed this issue, most of them
being case reports [5 7]. Most authors considered
these cord anomalies as an infrequent cause of fetal
demise. Only Machin and colleagues [4] demon-
strated that abnormal cord coiling with or without
cord stricture was significantly associated with
adverse perinatal outcomes. In the past, there has
Table 1. Frequency of umbilical cord stricture with or
without overcoiling
Group I: Group II: Group III:
stricture with stricture overcoiling
overcoiling, n only, n alone, n
14 (54%) 6 (23%) 6 (23%)
been a considerable debate as to whether these
lesions represent an artifact or constitute a real
cause of fetal loss. Presently, it is generally
accepted that umbilical cord stricture constitutes a
cause of fetal demise [3,8 11]
In this study, we report an incidence of 19%
of fetal demise associated with umbilical cord
stricture with or without overcoiling in a regional
tertiary hospital. The underlying pathogenesis of
these anomalies is discussed, and a new classi-
fication is proposed.
METHODS
A total of 268 fetal autopsies performed at North
Shore University Hospital, Manhasset, NY, from
1998 to 2000 were reviewed. Termination of
pregnancy or stillborns due to premature rupture
of membranes, termination of pregnancy for fetal
anomalies, and elective terminations were excluded
from the study. We excluded premature rupture of
membranes because we did not want to introduce a
known or possible risk factor for fetal demise. In
total, 139 cases of fetal demise including sponta-
neous abortion were identified. Of these, 26 were
associated with umbilical cord stricture, overcoil-
ing, or a combination of both. In all of these 26
cases, other causes for fetal demise were ruled out.
We defined stricture of umbilical cord as a
markedly decreased diameter in relation to the
remaining umbilical cord and overcoiled umbilical
cord as 0.3 coil/cm or more (cord coil index) by the
criteria of Machin and colleagues [4]. There are no
published data that define numerically a strictured
Table 2. Demographic data of fetal demise with cord stricture and overcoiling
Maternal Gestational
age, y age, wk Gravida
33 6 4.6* 21 6 6.7* 3.5 6 1.9*
*Values are mean 6 SD.
MVP, mitral valve prolapse; DM, diabetes mellitus; IBD, inflammatory bowel disease.
umbilical cord. In our material, strictures measured
between 1 and 3 mm in diameter, representing a
considerable difference with the diameter of the
Total no. umbilical cord proximal to the stricture. Our 26
of cases cases were divided into 3 groups: group I included
26 umbilical cord stricture with overcoiling, group II
had umbilical cord stricture only, and group III had
umbilical cord overcoiling alone. Gestational age-
matched 26 fetal demise cases without umbilical
cord anomalies were chosen as the control group.
Causes of fetal demise of the control group
included fetal thrombotic vasculopathy, maternal
infection, premature rupture of membranes with
inevitable abortion or nonviable fetus, termination
of pregnancy for premature rupture of membranes,
and elective termination of pregnancy.
Umbilical cord assessment was based on
gross and microscopic examinations. Two or three
sections from the umbilical cord at the stricture and
from the nonstrictured area were taken and
evaluated by hematoxylin-eosin, Alcian blue (pH
2.5), and trichrome stains. Wharton’s jelly of the
umbilical cord was scored as normal and de-
creased/absent by comparing with self and gesta-
tional age matched normal umbilical cord controls
using Alcian blue stain. Fibrosis of umbilical cord
was assessed as present and absent using the
trichrome stain.
The placenta was reviewed for secondary
thrombosis of the vessels of the chorionic plate.
Two pathologists read the slides independent of
each other. The placentas were specifically exam-
ined for the presence or absence of secondary
thrombosis. Any discrepancy was reviewed togeth-
er to obtain a consensus. The demographic data
and medical history were obtained by reviewing
the medical charts.
RESULTS
In our study population, 19% (26 of 139) were
found to have umbilical cord anomalies with
Past medical history, n
History of
Parity fetal demise, n Asthma MVP Migraine DM IBD
1.7 6 1.3* 4 3 2 2 1 2
UMBILICAL CORD STRICTURE AND OVERCOILING 15

Figure 1. Fetal demise at 19 weeks; the umbilical cord
showed proximal stricture and cord overcoiling with a cord
coil index of 1.02 coil/cm.
stricture, overcoiling, or a combination of both. As
displayed in Table 1, 20 of 26 of these cases had
umbilical cord stricture only or with overcoiling; 6
of 26 had only umbilical cord overcoiling. The
demographic factors and past medical history of
these patients were summarized in Table 2. Fetal
demise most commonly occurred in the second
trimester (mean gestational age, 21 6 6.7 weeks).
The average maternal age was 33 years, and the
mean gravida and parity were 3.5 and 1.7,
respectively. Fifteen percent of these patients had
a prior history of fetal demise. In 1 patient,
recurrent umbilical cord stricture was documented;
the other 3 reported missed abortions. More than
one third (38%) of these patients had a significant
past medical history including asthma (3), mitral
valve prolapse (2), migraine (2), diabetes (1), and
inflammatory bowel disease or colitis (2).
The most common site of the stricture was the
proximal portion of the umbilical cord (13 of 16),
followed by stricture in the middle portion of
Table 3. Frequency of Wharton’s jelly deficiency, fibrosis, and vascular thrombosis
Group no.
Group I: stricture with overcoiling 14
Group II: stricture only 5a
Group III: overcoiling alone 4a
a
Only cases available for special stain evaluation.
16 H.Q. PENG ET AL.
umbilical cord (3 of 16) (Fig. 1). The location of
the stricture could not be determined from the
autopsy report in 4 patients.
Sections from 23 cases were available for
Alcian blue and trichrome stains. The frequencies
of Wharton’s jelly deficiency, fibrosis of umbilical
cord, and thrombosis of the vessels of the chorionic
plate in 3 groups were summarized in Table 3. All
cases with umbilical cord stricture with or without
overcoiling showed a localized deficiency of
Wharton’s jelly and increased collagen (Fig. 2).
However, only 1 of 4r (25%) patients with
umbilical cord overcoiling alone had Wharton’s
jelly deficiency, 50% of them had increased
collagen deposition in their umbilical cords.
Thrombosis of the vessels of the chorionic plate
was found in 71% of group I patients, 40% of
group II patients, and 50% of group III patients,
respectively.
DISCUSSION
Fetal demise was observed in 52% (139 of 268) of
our perinatal autopsies. Fetal demise with umbilical
cord stricture or overcoiling was noted in almost
20% of all of our fetal demise cases. Umbilical
cord stricture with or without cord overcoiling was
noted in 20 of these 26 cases, representing an
incidence of 14%. Machin and colleagues [4]
reported a similar incidence of 13% of fetal demise
associated with cord stricture with overcoiling.
Strictures were noted predominantly near the
cutaneous insertion of the umbilical cord. How-
ever, less frequently, midsegmental strictures were
observed as reported by others [3].
Most authors considered cord stricture and
cord overcoiling to be similar or related entities. In
many cases reported in the literature, cord stricture
occurs in the context of cord overcoiling. However,
instances in which cord stricture has resulted in
fetal death independent of cord overcoiling have
been reported [12 15]. Bakotic and colleagues
Wharton’s jelly, n Fibrosis, n Thrombosis, n
Normal Decrease Absence Present Absent Present Absent
0 7 7 14 0 10 4
1 2 2 5 0 2 3
3 0 1 2 2 2 2
Figure 2. Sections from a strictured umbilical cord and a gestation age matched normal umbilical cord control (A and B,
hematoxylin-eosin stain; C and D, trichrome [pH 2.5]; E and F, Alcian blue; original magnification, 34).

[15] described a recurrent umbilical cord over-
coiling and stricture leading to fetal death in 3
subsequent pregnancies. In the 3rd pregnancy of
this series, the primary stricture site lacked the
marked coiling observed in the previous 2
gestations. The affected region of the cord showed
marked attenuation with fibrosis in association
with mild coiling. The authors suggested that cord
UMBILICAL CORD STRICTURE AND OVERCOILING 17
stricture, in lieu of a significant coiling, may have
played a more significant role in the eventual
demise of the fetus [15]. We found a total of 20
cases with cord stricture; although 14 of 20 had
coexisting cord overcoiling, 6 of 20 had cord
stricture only. These findings suggest that cord
stricture is a distinctive entity, which may predis-
pose to cord overcoiling, thus explaining the
frequent coexistence of both. On the other hand,
Sun and colleagues [3] suggested that umbilical
cord stricture is a separate entity and cord over-
coiling is a common and nonspecific finding in
both normal and stillborn infant. Cord overcoiling
may be associated with adverse fetal and placental
changes prior to fetal demise. Cardiac arrhythmias,
heart failure, nonimmune hydrops, intrauterine
growth retardation, placental insufficiency, and
oligohydramnios may all occur secondary to cord
coiling [16,17]. The reported normal cord coil
index is 1 coil/5 cm, or 0.2 6 0.1 (1 SD) coil/cm.
Machin and colleagues documented that abnormal
umbilical cord coiling (overcoiling or undercoil-
ing) was associated with adverse perinatal out-
comes in a large series study; 21% of cords were
overcoiled and 13% were undercoiled among 1329
cases. The principal adverse perinatal outcomes
associated with overcoiled cords were fetal demise
(37%), fetal intolerance to labor (14%), intrauterine
growth retardation (10%), and chorioamnionitis
(10%). For undercoiled cords, the frequencies of
these adverse outcomes were 29%, 21%, 15%, and
29%, respectively. These authors observed that
cord stricture was present in 13% of overcoiled
cords but did not correlate with undercoiled cords.
Among their 234 fetal demise cases, 44% were
found to have cord overcoiling and 29% of those
fetal demises with cord overcoiling had cord
stricture (incidence of 13%) [4]. We noted in our
study, 23% of fetal demise cases had cord
overcoiling alone; this number may represent an
underestimation because the cord coil index was
not always reported.
The primary pathological feature seen in
umbilical cord stricture with or without overcoiling
is a localized deficiency of Wharton’s jelly and
replacement of the stroma by dense hyalinized
collagenous tissue. These changes have also been
noted by other authors [4,5,15]. The underlying
mechanism of Wharton’s jelly deficiency is still not
clear. For some, the absence of Wharton’s jelly
represents a congenital deficiency, whereas for
18 H.Q. PENG ET AL.
others, the decrease in Wharton’s jelly is inter-
preted as a secondary phenomenon [3,8,11]. An
excessively length of the umbilical cord with
excessive fetal movements is implicated by others
[10]. Definite proof favoring congenital deficiency
over excessive length of cord is beyond the scope
of this paper. However, absence of Wharton’s jelly
was only observed in 1 case with umbilical cord
overcoiling alone, further supporting that cord
stricture and cord overcoiling are 2 distinct entities.
The thrombosis of the chorionic plate vessels
was frequently seen in umbilical cord stricture and
overcoiling. It was more common when both cord
stricture and overcoiling coexisted. This implies
that umbilical cord stricture or overcoiling could
decrease the blood flow with subsequent mural
thrombosis. With advancing gestation, a critical
point is reached where a decrease in umbilical
blood flow results in hypoxemia in the fetus.
Hypoxia is known to cause abnormal patterns of
fetal movements and variation in the fetal heart
rate. Fetal death may occur if the fetal movements
result in twisting of the stenosed section of cord,
which is unprotected by the Wharton’s jelly.
In summary, we have demonstrated that
umbilical cord stricture and cord overcoiling may
represent 2 distinct pathological entities that
constitute a common cause of fetal demise.
Umbilical cord overcoiling with stricture should
be classified as cord stricture. The etiology of cord
stricture and cord overcoiling should be addressed
by future prospective studies. We agree with
Machin and colleagues that a cord coil index
should be documented routinely in perinatal
autopsy. Our study reinforces the importance of a
fetal autopsy with careful examination of the
placenta and umbilical cord.
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