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J Clin Ultrasound 24:333-338, September 1996
0 1996 by John Wiley & Sons, Inc.
CCC 0091-2751/96/070333-06
Prenatal Diagnosis with Ultrasound of Anomalous
Course of the Umbilical Vein and Its Relationship
to Fetal Outcome
Arun S. Kinare, MD,* Shrikant T. Ambardekar, MD,* Debasis Bhattacharya, MD,t
and Satish A. Pande, MD*
Abstract: Diagnosis of three types of anomalous course of the umbilical vein (UV) was
made in 10 cases using antenatal ultrasound (US). Pulsed wave (PW) Doppler ultrasound was used to evaluate the UV in all cases. In one case, the UV was directly joining
the superior vena cava (SVC) without forming subcutaneous collaterals, an abnormal
course of UV not yet reported previously. Of these ten fetuses, six had various associated anomalies, including three fetuses with central nervous system (CNS) abnormalities. 0 1996 John Wiley & Sons, Inc.
Indexing Words: Prenatal ultrasonography . Umbilical vein . Fetal anomalies . Doppler ultrasonography
An anomalous course of the umbilical vein (UV)
is an uncommon finding. There are few reports
describing the anomalous course of UV although
the first article dates back to 1826.l Jeanty reported the first case of persistent right umbilical
vein (PRUV) diagnosed antenatally by US in
198g2and a series of 6 cases of PRUV in 1990.3To
our knowledge, only, two case reports of antenatal US detection of the UV directly entering into
the right atrium have been published since
then.*y5The published articles indicate that there
is a high incidence of associated anomalies with
this condition. We describe our experience with
ten cases of anomalous course of the UV and the
associated findings.
MATERIALS AND METHODS
The study includes ten cases of anomalous course
of the UV diagnosed in antenatal ultrasound examinations between September 1991 and November 1994. The population studied was unselected
and included a high risk group as well as routine
referrals. During this period, a total of 5754 paFrom the Departments of Ultrasound and ?Obstetric and Gynaecology, K.E.M. Hospital, Rasta Peth, Pune, India. For reprint requests contact Arun S. Kinare, M.D. Department of
Ultrasound, K.E.M. Hospital, Rasta Peth, Pune 411 011, India.
VOL. 24,
NO. 7, SEPTEMBER 1996
tients underwent ultrasound examination at all
stages of gestation.
The examinations were carried out using
Aloka SSD 280 and SSD 650 scanners with a 3.5
MHz mechanical sector transducer and a 3.5 MHz
curvilinear transducer. Pulsed wave Doppler examinations were carried out with an Aloka SSD
650 scanner using less than 50" angle of insonation and 100 Hz filter. The sample volume
was adjusted according to the vessel size.
PRUV was diagnosed by the following published riter ria.^
1. The UV is connected with the right portal vein
instead of the left portal vein.
2. The position of the right UV is lateral rather
than medial to the gallbladder (Figure 1).
3. The portal vein curves toward the stomach
(Figure 2).
The normal anatomy of left umbilical vein is
shown in Figure 3 for comparison.
The other courses of the UV were confirmed by
tracing the UV with the help of pulsed Doppler
ultrasound. A detailed fetal US examination was
performed. The neonates were examined clinically, and by US after delivery.
RESULTS
Of the ten cases of anomalous course of the UV,
eight fetuses showed PRUV. In one fetus, the UV
333
KINARE ET AL.
FIGURE 1. Transverse view of the abdomen in a fetus with persistent right umbilical vein shows the umbilical
vein (UV) lateral t o the gall bladder (GB).ST, stomach.
FIGURE 2. Transverse view of fetal abdomen in a fetus with a persistent right umbilical vein shows the
umbilical vein (UV) with its concavity toward the stomach (ST). GB, gallbladder.
was seen directly entering in the right atrium
(RA) (Figure 4). In another fetus, the UV was
seen continuing directly into the thorax and joining the SVC. (Figure 5, Table 1).In the fetuses
with PRUV, four fetuses did not have an anomaly
detected antenatally or after birth. One fetus had
severe IUGR and a Dandy- Walker malformation
which was confirmed after birth. This fetus delivered prematurely and died in the neonatal period.
In another patient referred to our institute for
severe hypertension, the fetus had a meningomy334
elocele, hydromyelia, mildly dilated lateral ventricle, and cerebellar herniation (Chiari type I1
malformation). The pregnancy was terminated
for uncontrolled maternal hypertension. The findings were confirmed postnatally.
Two fetuses were shown to have small septal
defects with echocardiography after birth. One
had an atrial septal defect (ASD); the other had a
ventricular septal defect (VSD). The fetus with
the VSD had glandular hypospadius which was
not detected with antenatal US.
JOURNAL OF CLINICAL ULTRASOUND
ANOMALOUS COURSE OF UMBILICAL VEIN
FIGURE 3. Transverse view of the abdomen showing the relationships of the normal left umbilical vein (UV)
with the gallbladder (GB) and stomach (ST) for comparison.
FIGURE 4. Umbilical vein directly entering the right atrium (RA). Doppler ultrasound shows flow similar to the
flow in the RA (right panel)
On antenatal US, the fetus with the umbilical
vein directly draining in the RA had right hydronephrosis and abnormal spinal curvature, which
was thought to be due to a hemivertebra. After
birth, in addition to these findings, the fetus was
found to have syndactyly and hypertelorism.
The fetus with the UV continuing directly in
the thorax and joining the SVC had exencephaly
meningocele, dextrocardia, and a two-vessel cord.
In view of these anomalies, the parents opted for
termination but refused autopsy. Hence the umbilical vessels were cannulated immediately after
delivery and contrast was injected, which confirmed the US findings (Figure 6).
DISCUSSION
Embryologically the sinus venosus is joined by
the paired umbilical veins between 2nd and 4th
VOL. 24, NO. 7, SEPTEMBER 1996
weeks, menstrual age. The liver bud grows and engulfs the umbilical veins. The umbilical veins develop connections with the liver sinusoids, and during this process their connection with the sinus
venosus is lost. The right umbilical vein regresses
between the 4th and 5th weeks. The left umbilical
vein persists and drains into the right atrium
through the portal venous system and the ductus
venosus, which develops at about the same time.
If the anastomoses between the hepatic sinusoids and umbilical veins do not develop, blood is
then diverted through other channels leading to
an anomalous course of the UV. The PRUV appears to be the most common path. The UV directly entering the right atrium is one of the
other anomalous courses. The other pathways described are the UV joining the iliac veins, inferior
vena cava above or below the liver, or SVC
through subcutaneous collaterals.
335
KINARE ET AL.
FIGURE 5. Umbilical vein (UV) continuing into the thorax and joining the superior vena cava. No evidence of
subcutaneous collaterals is obvious.
We are not aware of any report describing the
UV directlyjoining the SVC without forming subcutaneous collaterals. The umbilical vein in this
case probably joins the SVC through the azygos
vein. In the present series, of the eight fetuses
with PRUV four had associated anomalies.
Jeanty3 has suggested that PRUV probably represents a more benign form of this anomaly, and
our findings support this possibility. All fetuses
with the other two anomalous courses of the umbilical vein had associated anomalies. Jouk et a14
described one case of a UV directly entering in
the right atrium without any associated anomaly.
The fetus with the UV joining the SVC (our series) had severe anomalies; this probably represents severely disorganized embryonic development.
Interestingly and strikingly, the long list of associated anomalies with an anomalous course of
the UV shows that association with CNS anomalies is rare. The present series had a high incidence of CNS anomalies, including a case of
Dandy- Walker malformation, Chiari type I1
malformation, and one with exencephaly and a
meningocele. Except for the series of Hill et a1,6
which includes a case of anencephaly, our review
TABLE 1
Cases Showing Anomalous UV Associated Anomalies Detected in Ultrasound and Their Outcome
No.
1
Menstrual
Age
wks
27
Indication
PIH, anemia, size less than
dates
BOH
Routine
2
3
39
4
5
30
20
Routine
Large for dates
6
7
22
39
Routine
Small for dates
8
24
18
36
Routine
Routine
Severe PIH, suspected
anomaly on level I scan
done elsewhere
9
10
ASD
BOH
IUGR
PIH
PRUV
RA
svc
uv
VSD
336
28
Anomalies on US
PRUV, Dandy-Walker malformation
PRUV, no anomaly
UV draining in RA, right
hydronephrosis, hemivertebra
PRUV, no anomaly
UV continuing via azygos vein joining
SVC, exencephaly, meningocele,
dextrocardia, two-vessel cord
PRUV, no anomaly
PRUV, scanty amniotic fluid, no renal
or any other anomaly
PRUV, no anomaly
PRUV, no anomaly
PRUV, meningomyelocele,
hydromyelia, mildly dilated lateral
ventricles, cerebellar herniation
(Chiari t w e II malformation)
Anomalies Detected
After Birth & Outcome
Dandy-Walker malformation, severe
IUGR, neonatal death
No anomaly
Right hydronephrosis, hypertelorism,
syndactyly, hemivertebra
No anomaly
Confirmed by radiological methods
after termination of pregnancy
No anomaly
Glandular hypospadias, small VSD,
detectable only by Doppler US
Small ASD
No anomaly
Induction of labor, fresh stillbirth,
meningocele present, autopsy not
done
atrial septal defect
bad obstetric history
intrauterine growth retardation
pregnancy induced hypertension
persistent right umbilical vein
right atrium
superior vena cava
umbilical vein
ventricular septal defect.
JOURNAL OF CLINICAL ULTRASOUND
ANOMALOUS COURSE OF UMBILICAL VEIN
FIGURE 6. Contrast study of the same fetus as shown in Figure 4 after
termination of pregnancy. Note the course of the umbilical vein, dextrocardia, and the cranial anomaly.
of literature failed to show an association of CNS
anomalies with an anomalous course of the UV.
One of the ten fetuses had a two-vessel cord;
each of the other fetuses had a three-vessel cord.
A two-vessel cord is known to be associated with
various anomalies.' A four-vessel cord is a very
rare occurrence. In one of the reported cases, the
fourth vessel was thought to be due to persistence
of the caudal portion of the right umbilical vein.
This fetus had multiple a n ~ m a l i e s No
. ~ case in
the present series showed a four-vessel cord with
an anomalous course of the UV. However, it
would be interesting to look for this association.
We believe that, with proper technique, PRUV
and the other anomalous courses of the UV can be
diagnosed in B-mode images. Pulsed Doppler ultrasound is useful in confirming and differentiating the UV from the gall bladder in a few difficult
cases. As reported earlier: pulsed Doppler ultrasound studies of the UV directly entering the
right atrium show a waveform similar to that
seen in the right atrium (Figure 4). A right atrial
waveform was also noted in the UV joining the
SVC near their junction. Because facilities for
color Doppler ultrasound examinations were not
available in our institution, we were restricted to
image-directed Doppler ultrasound evaluations.
In the fetuses with PRUV, we searched for the
course of the UV after birth. With a high frequency transducer, the Course of the UV could be
traced and it was seen lateral to the gallbladder
(Figure 7). We agree with Jeanty3 that unless the
anomaly is already known, it can be difficult to
diagnose after birth.
Because the abdominal circumference is measured in all second and third trimester obstetric
scans, an anomalous course of UV should be
FIGURE 7. The umbilical vein (UV) is seen lateral to the gallbladder ( G B )in a neonate (on the second day after
birth) with an antenatally diagnosed persistent right umbilical vein.
VOL. 24, NO. 7, SEPTEMBER 1996
337
KINARE ET AL.
looked for routinely. Such a finding requires a
search for other anomalies including CNS anomalies. The appropriate time for this search would
be between 16 and 20 weeks, menstrual age.
We agree with Hill et a16 that fetal management depends on the type and severity of the associated malformation. The course of the UV by
itself does not warrant a change in antenatal
management.
3.
4.
5.
REFERENCES
1. Mende D: Insertionis venae umbilicalis in partem
atrii cordis dextri anteriorem unius vero arteriae
umbilicalis, ex aorta abdominali prorumpentis.in
foetu masculo maturo ac neonat singularem casum.
Noua Acta Acad Caesar Leop Carol 13:871-874,
1826.
2. Jeanty P: Fetal and funicular vascular anomalies:
338
6.
7.
identification with prenatal US. Radiology 173:
367-370, 1989.
Jeanty P: Persistent right umbilical vein: an ominous prenatal finding? Radiology 177:735-738,
1990.
Jouk PS, Champetier J: Abnormal direct entry of
the umbilical vein in to the right atrium: antenatal
detection, embryologic aspects. Surg Radio1 Anat
13:59-62, 1991.
Greiss HB, McGahan JP: Umbilical vein entering
the right atrium: significance of in utero diagnosis.
J Ultrasound Med 11:lll-113, 1992.
Hill LM, Mills A, Peterson C, Boyles D: Persistent
right umbilical vein: sonographic detection and subsequent neonatal outcome. Obstet Gynecol 84:923925, 1994.
Painter D, Russell P. Four-vessel umbilical cord associated with multiple anomalies. Obstet Gynecol50:
505-507, 1977.
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