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© 2023 Journal of Medical Ultrasound | Published by Wolters Kluwer - Medknow 1
Case Report
IntroductIon
The umbilical vein is the vessel that carries oxygenated
blood into the growing fetus from the placenta. The neonate’s
umbilical vein, within a week of birth is obliterated and is
replaced by a brous cord called ligamentum teres hepatis.
It extends from the umbilicus and joins with the falciform
ligament of the liver. Umbilical vein thrombosis can be caused
by cord compression, leading to stasis of the blood. Umbilical
vein varix is commonly diagnosed between weeks 22 and 33
of pregnancy, and the majority of patients have previously
normal prenatal sonograms. It is a developmental rather than a
congenital anomaly, and it may be caused possibly by elevated
venous pressure.[1] Blood type incompatibility between the
pregnant woman and fetus causes immune type of hydrops
fetalis and refers to the build‑up of abnormal uid collection
in two or more body areas of the fetus.
case report
A 28-year-old primi gravida female of gestational age 26
weeks presented to our rural hospital for an antenatal second-
trimester ultrasound scan. She is a known asthmatic and
uses a salbutamol inhaler intermittently. The patient had a
history of two episodes of mild asthma attacks on exertion
during the pregnancy. The patient was tested IgM positive for
cytomegalovirus on TORCH screening in the rst trimester
and did not undergo any treatment due to loss of follow-up.
Blood investigations revealed hemoglobin 10.3 g/dl, MCV
79.2 , WBC ‑ 8500 cells/cu.mm, and platelets ‑ 1,61,000
cells/cu.mm. APTT - 30 sec and prothrombin time of 12.3
sec, both were within normal limits. Ultrasound examination
showed increased nuchal fold thickness(11 mm), collection
of free uid in the abdomen [Figure 1], in the pleural cavity,
and in the pericardial space [Figure 2], giving the diagnosis of
hydrops fetalis. The ultrasound also showed a hypoechoic mass
of 29 × 25 mm in the extra‑abdominal portion of the umbilical
cord, which showed no color ow [Figure 3], indicating the
possibility of varix in the fetal umbilical vein. The power
Doppler of the umbilical artery showed absent diastolic ow
[Figure 4] and the umbilical vein showed absent waveform
on the pulse Doppler [Figure 5]. The fetal middle cerebral
artery Doppler showed a low pulsatility index (PI) of 1.2 and
S/D ratio of 2.8, suggesting fetal hypoxia [Figure 6]. Three
days following the scan, the patient was not perceiving the
fetal movements and was taken up for a repeat ultrasound,
which revealed the absence of cardiac activity suggesting
Antenatal Hydrops Fetalis with Umbilical Vein Varix and
Thrombosis – Ultrasound Imaging: A Rare Case
Manasa Suryadevara*, Roohi Gupta, Gaurav Vedprakash Mishra, Vadlamudi Nagendra, Pratik Jayprakash Bhansali
Department of Radiodiagnosis, Jawaharlal Nehru Medical College, DMIHER, Wardha, Maharashtra, India
Thrombosis of the umbilical vein/artery is a rare complication and is highly associated with fetal mortality. Varix of the fetal umbilical vein
is a very rare anomaly and refers to the focal dilatation of the umbilical vein of the fetus. It appears as a round or fusiform cystic structure in
the fetal abdomen. Here, in this case report, we hereby discuss a 28-year-old pregnant female with a gestational age of 26 weeks from last
menstrual period, who on antenatal ultrasound had ndings suggestive of umbilical vein varix with thrombosis and hydrops fetalis.
Keywords: Antenatal ultrasound, hydrops fetalis, umbilical vein thrombosis, varix
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Website:
www.jmuonline.org
DOI:
10.4103/jmu.jmu_11_23
Address for correspondence: Dr. Manasa Suryadevara,
Department of Radiodiagnosis, Jawaharlal Nehru Medical College, DMIHER,
Sawangi, Wardha ‑ 442 001, Maharashtra, India.
E‑mail: manasa.suryadevra@gmail.com
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How to cite this article: Suryadevara M, Gupta R, Mishra GV,
Nagendra V, Bhansali PJ. Antenatal hydrops fetalis with umbilical vein varix
and thrombosis – Ultrasound imaging: A rare case. J Med Ultrasound 0;0:0
Abstract
Received: 11-02-2023 Revised: 08‑04‑2023 Accepted: 05‑06‑2023 Available Online: 23-09-2023
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Suryadevara, et al.: Us imaging of umbilical vein varix and thrombosis
2Journal of Medical Ultrasound ¦ Volume XX ¦ Issue XX ¦ Month 2023
fetal demise. The patient received termination. The Gross
appearance of the neonate after the termination showed a
distended abdomen suggesting ascites and dilated umbilical
cord appearing bluish in color suggesting the umbilical vein
varix with internal thrombus [Figure 7]. Followup karyotyping
revealed no chromosomal abnormality.
dIscussIon
Asthma can lead to a decline in the amount of oxygen in the
mother's blood. The fetus gets the oxygen from the mother's
blood, and a drop in oxygen levels in the maternal blood could
result in a drop in oxygen levels in the fetal blood. This could
aect the growth and survival of the fetus as in our case.
Hydrops fetalis is a serious condition that refers to abnormal
amounts of fluid build-up in two or more extravascular
compartments of a fetus. It can be of two types, the immune
and non-immune, of which the latter is common and can be
caused by severe anemia, lung or heart problems.[2] Non-
immune hydrops fetalis (NIHF) aects around 1 in 1500 and
1 in 4000 newborns.[3]
The most common cause of congenital infection is
cytomegalovirus (CMV).[4] The likelihood of vertical
transmission from primary maternal CMV infection during
pregnancy ranges from 30% to 40%.[4] Ultrasound features
of congenital cytomegalovirus Infection include intrauterine
growth restriction, ascites, and fetal hydrops.[5,6]
A focal dilation of the umbilical vein is known as an umbilical
vein varix. It is an uncommon disease that raises fetal morbidity
and mortality rates.[7] Normal umbilical vein shows continuous
monophasic non‑pulsatile ow towards the fetus. Umbilical
vein thrombosis can be caused by cord compression leading
to stasis of the blood. Although uncommon, umbilical cord
vascular thrombosis can be fatal. prenatal autopsy incidence
ranges from 1/1000 to 1/1500 deliveries.[8,9] The rate is 1.6
times higher in males compared to females. Although umbilical
vein thrombosis seems more common than umbilical artery
thrombosis (71‑85% vs 11‑15%), the literature reports poor
neonatal outcomes more frequently with umbilical artery
thrombosis.[8] This thrombosis may be caused by abnormal
anatomy of the umbilical cord or mechanical injury to the
cord. Pregnancy raises a woman's risk of thrombosis by
four to ve times compared to the nonpregnant group due to
hypercoagulability of the blood.[10]
In fifty-two cases of umbilical cord thrombosis reported
by Heifetz,[8] thrombosis was found to be associated with
obstetrical complications (such as infection, phlebitis, and
preeclampsia), additional umbilical cord abnormalities (such as
vessel stretching, knots, etc.) or systemic fetal conditions (such
as fetomaternal hemorrhage, diabetes), which was considered
the likely cause of thrombosis. The compression of the cord
Figure 1: Gray‑scale axial ultrasound image of fetal abdomen showing
ascites (arrow)
Figure 2: Gray‑scale axial ultrasound image of fetal thorax showing
pericardial effusion (arrow)
Figure 3: Grey scale and Colour flow image of umbilical cord showing 2
umbilical arteries (red), umbilical vein (blue), and a hypoechoic septated
mass with no color flow (white arrow) suggesting umbilical vein varix
Figure 4: Umbilical artery Doppler showing absent diastolic flow
suggesting fetal hypoxia
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Suryadevara, et al.: Us imaging of umbilical vein varix and thrombosis
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Journal of Medical Ultrasound ¦ Volume XX ¦ Issue XX ¦ Month 2023
can lead to blood stasis and eventually, thrombosis in umbilical
vessels which occur in true umbilical cord knots formed in a
long cord. On the other hand, short cords are more susceptible
to vessel stretching during labor, which could also result in
vessel damage and eventually thrombosis.[11,12]
Another established risk factor for fetal thrombus formation
is maternal diabetes mellitus.[13] Children of diabetic mothers
have greater levels of 2‑antiplasmin and lower brinolysin
activity as well as a higher risk of thrombosis. Additionally,
they are more vulnerable to vasoconstriction and platelet
aggregation due to an imbalance between the factors that cause
vasodilatation and vasoconstriction.[14] Other fetal disorders
like hemolytic diseases, fetomaternal transfusion, and fetal
hydrops, which have anemia as a common factor, lead to stasis
of blood, thrombosis, and fetal heart failure and are believed
to play a role in the development of thrombi.[8,9]
Doppler sonography, in particular power Doppler, is thought
to be a crucial supplementary tool for the documenting of
thrombus,[15,16] but it's important to assess the standards that
are used to determine how frequently patients should be
monitored. Cord thrombosis should be regarded as a sign of
extreme severity and should demand more frequent monitoring
to improve results.[14]
Umbilical vessel thrombosis is a rare but serious pregnancy
complication. Correctly diagnosing and treating umbilical
cord thrombosis remains a hard challenge.[17] It is prudent to
rule out portal vein thrombosis when a neonate's umbilical
cord thrombosis is discovered. Infant end-organ damage
should be taken into account, and a thrombophilia screening is
essential. Decreased or disappeared fetal movement is the main
manifestation of umbilical cord thrombosis.[18] The main cause
of umbilical cord thrombosis is umbilical cord abnormalities
like the compression, twisting, or twining of the cord. Patients
should be made aware of the value of self-counting fetal
movement. Early identication of umbilical cord thrombi can
be aided by focusing on counting fetal movements, electronic
fetal monitoring, and specic signs like echogenic thrombus
showing absent color ow and spectral Doppler changes during
prenatal ultrasound. It is advised to do an emergency cesarean
section to lessen the chance of interrupting the umbilical cord's
blood supply, in case if the fetus is old enough to survive.[17]
conclusIon
Although uncommon, umbilical cord thrombosis can have
serious adverse eects on the pregnancy. High‑risk factors
for umbilical cord thrombosis include hypercoagulability,
abnormal umbilical cord, and abnormal blood glucose.
Decreased fetal movement and abnormal fetal monitoring
are the main clinical manifestations of the patients. Though
umbilical vein thrombosis is the main manifestation, adverse
fetal pregnancy outcomes are caused by both arterial and
venous thrombosis.[17] Those patients who showed two
umbilical arteries in the previous ultrasound but the second
ultrasound suggested a single umbilical artery should be highly
alerted of the possibility of an umbilical cord thrombosis.
When the fetus is more than 24 weeks of gestational age which
Figure 5: Umbilical vein Doppler showing absent waveform suggesting
thrombosis
Figure 6: Fetal middle cerebral artery Doppler showing low pulsatility
index of 1.2 and S/D ratio of 2.8 suggesting fetal hypoxia
Figure 7: Gross appearance of neonate, after the termination showing
abdominal distention suggesting ascites (arrow) and umbilical vein varix
and internal thrombus (star)
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Suryadevara, et al.: Us imaging of umbilical vein varix and thrombosis
4Journal of Medical Ultrasound ¦ Volume XX ¦ Issue XX ¦ Month 2023
could survive, actively terminating a pregnancy is an ecient
therapeutic method to lower perinatal mortality.
Declaration of patient consent
The informed consent form was lled out by the patient. In
the form, the patient has given her consent for the images and
other clinical information to be reported in the journal. The
patient understands that name and initials will not be published
and due eorts will be made to conceal identity.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conicts of interest.
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