Rom J Leg Med [22] 177-180 [2014]
DOI: 10.4323/rjlm.2014.177
© 2014 Romanian Society of Legal Medicine A case of umbilical cord hemangioma
Jie Yan, Yueran Zhu, Huidan Liao, Lina Su, Yadong Guo, Jifeng Cai*
_________________________________________________________________________________________
Abstract: We report a rare case of an umbilical cord hemangioma. The umbilical cord consisted of Capillaries and
cavernous blood vessels and the hemangioma stressed umbilical arteries and vein. At 40 weeks of gestation, it resulted in
intrauterine death of the fetus. Microscopically, there were lots of vessels with various thicknesses in the umbilical cord. Autopsy
shows evidences of smothering. These findings indicate there may be a causal connection between the umbilical cord hemangioma
and fetal death because of the impaired umbilical circulation.
Key Words: umbilical cord hemangioma, umbilical blood vessel, fetal death.
A
s an important part of fetal appurtenance,
umbilical cord is the channel for gas
exchange, nutrient supplies and metabolites excretion
between the mother and the fetus. The pathological
change of the cords will have Serious impacts on not
only the fetus, but also the mother. Among all kinds of
the pathological changes of the umbilical cords, umbilical
blood vessel malformation is the most common structure
abnormality. Usually, there are two arteries and one vein
in a umbilical cord. However, in some cases, there are
some unexpected vascular patterns found in umbilical
cords. Among them, fetal umbilical cord hemangioma is
a rare kind of umbilical vessel abnormalities. It originates
from the remnants of embryo hemangioblast and turn
to vessel-like structure. These thin-walled capillary
proliferation originate from umbilical arteries, umbilical
vein or vitelline capillaries [1]. We report a case of
umbilical cord hemangioma that led to fetal asphyxia.
Case report
A gravida with cessation of menstruation for
40+6 weeks was sent to hospital preparing for delivery
on March 5th, 2013. The fetal heart rate was 140 bpm.
Both of the maternal pelvis and fetal presentation were
normal. There was no uterine contraction activity. On
the vaginal examination, the gravida’s cervix canal
was found to be midposition and 70% of it was effaced
with cervix still being closed. B ultrasonic showed fetal
presentation of LOA, biparietal diameter of 96 mm,
femur length of 73 mm and cardiac rate of 142 bpm. The
anterior placenta was 30 mm thick and was found to
be Maturity Level II. Amniotic fluid index was 80 mm.
Electrocardiogram showed sinus rhythm and normal
electrocardiogra. Hospital diagnosis showed “G2P0,
LOA, intrauterine pregnancy 40+6 weeks”. At 14 o’ clock
on next day, Oxytocin challenge was tested to be negative.
At 23 o’ clock, a nurse reported she couldn’t feel the fetal
heartbeat. Doctors rushed off to the gravida only to find
the fetus had been dead intrauterine.
Autopsy findings
Autopsy was operated on next day. The stillborn
weighted 4.03 kg and measured 55 cm in length. He had
grown normal and was at medium nutrition level. Head
circumference measured 32 cm with 1cm long hair and
* Dept. of Forensic Science, School of Basic Medical Sciences, Central South University, Changsha 410013, Hunan, China
* Corresponding author: Tel. +86 731 82355414; Fax +86 731 82355414, Email: [email protected]
177
Yan J. et alA case of umbilical cord hemangioma
chest circumference measured 33 cm. Rigor mortis could
be found at large joints of limbs. Livor mortis was reddish
and located on the back of the body where had not
been pressed. When pressed with a finger, it would fade
slightly. Palpebral conjunctiva and bulbar conjunctiva
hyperaemia corneal were mild cloudy. The isocorias were
dilated with diameters of 5 mm. It was cyanotic on lips,
nails and toes. No obvious damages were found on face,
neck, chest or abdomen. The spine was normal.
Visceral post-mortem examination: The
subcutaneous hemorrhagic region measuring 8 cm ×
7 cm was found on the top of the head. The anterior
fontanel measured 3 cm × 3 cm and the posterior
fontanel measured 0.8 cm × 0.8 cm. No skull fracture
was seen. No hemorrhage was found in epidural space,
subdural space or subarachnoid space. The brain weighs
520 g with vascular congestion. There was no damage,
bleeding or space-occupying lesions in the section of
the brain. Hemorrhagic spots could be found on the
surface of the lung. Hydrostatic test of lung was negative.
The pericardial was intact and 2.0 ml pale yellow liquid
was found in pericardial cavity.
The heart weighs 30 g with scattered hemorrhagic
spots on the surface, especially at right edge of the heart
and the atrioventricular junction. There was a coloboma
measured 0.4 cm × 0.3 cm on the interatrial septum. Each
of the cardiac valves was normal. The liver, gallbladder,
kidneys, esophagus and stomach were normal, too.
Placenta measured 20 cm × 18 cm × 2 cm with part of
umbilical cord measured 44 cm attached. Both the fetal
surface and the maternal surface were smooth. The other
part of the umbilical cord, 20 cm long, was attached to
the fetal navel. A dark red-black hemorrhagic region
surveying 8 cm could be found, which was 10 cm far from
the navel (Fig. 1A).
Microscopy: In subarachnoid space, vessels was
extended with hyperemia and leakage hemorrhage was
found, and pericapillary space became broader. Sheet
hemorrhage could be found in the epicardium with
focal myocardial gap widened and interstitial vascular
hyperemia. Angiotelectasis and hyperaemia also could be
found in lungs, hepatic sinusoid, spleen, renal interstitial,
thymus and thyroid interstitium. In Wharton’s jelly,
there were lots of hemorrhagic vessels with various
thickness (Fig. 1B).The vessels, highly dilated, were
connected in dendritic with endothelial cells in the
lining of the blood vessels and few connective tissues in
the interstitium. Venous congestion and arterial sheet
hemorrhage were found in umbilical cord (Fig. 1C).
The umbilical artery was squashed nearly occlusive (Fig.
1D). Angiotelectasis and hyperaemia also be found in
placental interstitium. Immunohistochemical staining
of coagulation factor (F)VIII and CD34 was performed,
FVIII-positive cells were observed in the area of dense
vessels and CD34 labelling cells were found to be negative
(Figure 1. E-F).
178
Discussion
Both of benign and malignant tumours are
extremely rare lesions of the umbilical cord in clinical
cases. Among them, angiomas, angiofibromas and
angiocavernoma are benign tumours [2]. Different from
the maliganant tumours, the benign ones have a special
impact on fetus by vascular compression. Umbilical
hemangioma are mostly located in distal third of the cord
and mainly affect umbilical vessels and circulation as in
our case [3]. They are often not of uniform size, vary in
colour and surrounded by Wharton’s jelly. Coagulation
factor (F)VIII and CD34 are often used as markers of
vascular endothelial cells.
In the present case, immunohistochemical results
shows positive of coagulation factor (F)VIII, indicating it
is the vascular tissue.
Both clinical course and the result of autopsy
support a sudden fetal death due to asphyxia. This is easily
explained hemodynamically. The vessels have thinner
and severely deformed blood-vessel walls. As time goes
on, umbilical facial blood flow increases gradually. When
the fetus is coming out from the uterus, due to the strong
intermittent pressure in utero, capillaries of the umbilical
hemangioma expand and abnormal vessels may burst
easily without protection of amniotic fluid, finally leading
to intrauterine asphyxia even fetal death [4]. There are
also other reports point out umbilical cord torsion is
another potential mechanism. What’s more, coupled
with the proliferation of the hemangioma, the umbilical
vessels could be more and more narrow and limited [5].
The poor blood circulation caused by umbilical
hemangioma could also lead to other problems. There
are references pointing out that umbilical thrombus
could cause non-immune hydrops, polyhydramnios,
fetal disseminated intravascular coagulation and fetal
hydrops [6-9]. It is possible that these changes might
lead to further reduction in umbilical circulation which
has already been impaired by tumor compression.
Many researchers speculate this additional reduction in
umbilical blood flow would result in fetal demise. Some
researches illustrate the umbilical hemangioma may be
caused by trisomy 18 [10-11].
The morbidity and mortality rate of umbilical
cord hemangiomas has been reported to be 35% [12], but
till now we are still lack of sufficient evidence to specify
how umbilical cord hemangiomas leads to series of severe
consequences. In order to decrease the risk of umbilical
cord hemangiomas, comprehensive and detailed
evaluation of clinical events and stillborn fetus should be
operated the sooner be better. Routine ultrasound, as the
most popular detecting artifice for prenatal diagnose and
regular reexamination, should be emphasized [13]. It is
reported that amniotic fluid oc-fetoprotein concentration
may also be a valuable reference on umbilical cord
hemangiomas [5].
Romanian Journal of Legal Medicine A
B
C
D
E
F
Vol. XXII, No 3(2014)
Figure 1. A. A dark red-black hemorrhagic region on the cord, which was 10 cm far from the navel. B. Histologic section of the
umbilical cord hemangioma showing vessel-like structure in wharton's jelly. C. Sheet hemorrhage were found in umbilical cord.
D. Histologic section of squashed umbilical artery. E. Immunohistochemical staining of coagulation factor (F)VIII shows F8positive cells at the lining of the dense vessels. F. Immunohistochemical staining of CD34 shows CD34-negative cells on the inner
wall of the tube.
Acknowledgment. This study was supported
by the National Natural Science Foundation of China
(NSFC, No. 81302615), Science Foundation for The
Youth Scholars of Central South University (NO.120973)
and Central South university student innovation test
plan (2282013bks073). Jie Yan is a postdoctoral fellow of
School of Basic Medical Sciences. Thanks to all members
of forensic interest community of Central South
University for the work.
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