A New Classification of Congenital Abnormalities of UPVS - Sonographic Appearances, Screening Strategy and Clinical Significance - Insights Into Imaging - Full Text

1/11/23, 1:19 PM A new classification of congenital abnormalities of UPVS: sonographic appearances, screening strategy and clinical significance | Insights
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Table 1 Ultrasonographic manifestations,

diagnostic grading, prognosis and perinatal
management of new classification of congenital
abnormalities of UPVS
From: A new classification of congenital abnormalities of UPVS: sonographic appearances, screening strategy and
clinical significance
https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-01068-5/tables/1 1/14
1/11/23, 1:19 PM A new classification of congenital abnormalities of UPVS: sonographic appearances, screening strategy and clinical significance | Insights into Imaging | Full Text
New Subtypes Prenatal Prenatal Clinical findings Perinatal management

classification ultrasonographic diagnostic and prognosis
manifestations grading
Type I: Preduodenal Prenatal ultrasound Difficult A majority of patients No special management is
Anatomy and portal vein may demonstrate with PDPV are needed in the case with no
morphological (PDPV) duodenal obstruction asymptomatic, but clinical symptoms, and
abnormalities as “double bubble” various clinical symptomatic work-up and
of the portal sign presentations and treatment are required in the
vein coexisting conditions case with clinical symptoms,
can be present, the such as plain abdomen
most common being radiography examination and
duodenal duodenoduodenostomy
obstruction


Portal vein and There is no report Difficult Most patients may No special management is
bile duct inverted related to prenatal have no obvious needed in the case with no
variation ultrasound diagnosis clinical symptoms, or clinical symptoms
of the abnormality mild abdominal
distension or
jaundice
Duplication of the There is no report Difficult The condition may No special management is
portal vein (DPV) related to prenatal be a cause of needed in the case with no
ultrasound diagnosis abdominal pain and clinical symptoms, and
of the abnormality can give rise to symptomatic work-up and
portal hypertension, treatment are necessary in
with the the case with clinical
development of symptoms, such as
esophagogastric postnatal ultrasonography
varices, and may and enhanced computed
provide a source of tomography
fatal hemorrhage
during childhood
Cavernous There is no report Difficult Clinical symptom of Postnatal utrasonography or
transformation of related to prenatal CTPV can include enhanced computed
portal vein ultrasound diagnosis portal hypertension, tomography can be
(CTPV) of the abnormality splenomegaly, performed for follow-up; the
ascites, Rex bypass shunt is
gastrointestinal considered the gold
varices, obstructive standard strategy
jaundice, mesenteric
venous congestion
and ischemia,
ascending
cholangitis and
biliary cirrhosis
Portal vein There is no report Difficult Portal vein Postnatal utrasonography or
stenosis or atresia related to prenatal obstruction, enhanced computed

ultrasound diagnosis splenomegaly, tomography can be
of the abnormality variceal hemorrhage performed for follow-up, and
and portal symptomatic operative
hypertension could treatment are necessary
be the results of the
anomaly; the
prognosis is closely
related to clinical
symptoms
Portal vein There is no report Difficult Portal vein Postnatal ultrasonography or
hypoplasia related to prenatal hypoplasia is a enhanced computed
ultrasound diagnosis known comorbidity tomography can be
of the abnormality of biliary atresia, in performed for follow-up, and
addition to an symptomatic operative
enlarged hepatic treatment and liver
artery; primary transplant evaluation is
hypoplasia of the necessary
portal vein can cause
secondary portal
hypertension that
presented with the
severe but typical
clinical
manifestations of
ascites and hepatic
encephalopathy
Portal venous Ultrasonography Amenable Most congenital Perinatal management
aneurysm (PVA) demonstrates cases are primarily entails periodic
dilatation of the PV asymptomatic surveillance of the aneurysm
system through medical imaging,
Doppler ultrasonography is
the most useful method
Portal vein Type 1—portal vein Unnecessary Most patients are No special management is
variants trifurcation, where the asymptomatic required for isolated cases

MPV divides into three
branches: the LPV, the
RAPV and the RPPV
Type 2—the RPPV
arises directly from
the MPV as its first
branch
Type 3—the RAPV
originates from the
LPV
Type 4—the portal
vein gives only a
single right portal
branch in the liver
hilum, and the left PV
arises from the right
anterior segmental
branch
Transposition of There is no report Unnecessary Most patients are No special management is
the left and right related to prenatal asymptomatic needed in the case with no
portal vein ultrasound diagnosis clinical symptoms
of the abnormality

Type II: Intrahepatic The sonographic Amenable The prognosis of An IPRUV should prompt an
Anatomy and persistent right criteria included: (1) isolated intrahepatic extended anatomic survey
morphological umbilical vein an aberrant course of PRUV has a very low and a fetal cardiac
abnormalities (IPRUV) the PV toward the risk for an adverse evaluation; if the survey and
of the stomach; (2) the fetal neonatal outcome; cardiac anatomy are
umbilical vein gallbladder being the prognosis of reassuring, no further
medial to the UV; (3) non-isolated cases follow-up is needed; if
The UV fuses with the depends on the additional findings are
RPV instead of the severity of identified, genetic
LPV accompanied counseling and invasive
anomalies testing should be
considered

Duplication of the Ultrasonography Amenable Isolated DUV A DUV (intrahepatic PRUV)
umbilical vein demonstrates two (intrahepatic PRUV) should prompt an extended
(DUV) (IPRUV) UVs in a transverse has a better anatomic survey; no further
section of the fetal outcome, and the follow-up is needed for
abdomen, which drain prognosis of non- isolated cases; if additional
into the LPV and the isolated cases findings are identified,
RPV, respectively; the depends on the genetic counseling and
umbilical cord is also severity of additional invasive testing should be
visible in four vessels findings considered
Umbilical vein Sonographically, UVV Amenable The prognosis of A systematic structural
varix (UVV) appears as whole isolated case has a examination should be
(normal direction) course ectatic very low risk for an performed; no further
anechoic UV or limited adverse neonatal follow-up is needed for
mass, and color outcome isolated cases; genetic
Doppler sonography testing is recommended in
detects the the fetus with non-isolated
bidirectional turbulent UVV
flow, at the level of the
dilated segment of the
umbilical vein
Umbilical vein Prenatal ultrasound Necessary The pregnancy Close fetal ultrasonographic
constriction shows the diameter of complications surveillance is necessary
the UV is narrower include IUFD, IUGR
than the normal and oligohydramnios
range, and pulsed
Doppler detects high-
speed blood flow,
which can be as high
as 150 ~ 200 cm/s

Type III: Portal- IHPSS The prenatal Necessary Fetuses with IHPSS Hemodynamic surveillance
Vascular systemic ultrasound is shown have the highest live should be performed in fetal
connection shunts the connection with birth rate compared period; for the cases cannot
abnormalities (PSS) the IHPVS and the HV, to other types of be closed naturally, surgery
and the DV should be shunt fetuses, which was performed to repair the
focused can be naturally shunt
closed after delivery


EHPSS The antenatal Necessary The prognosis of Hemodynamics should be
ultrasound visualizes EHPSS depends on closely monitored before
the SpV and the SMV the size of shunt delivery
shunting into the IVC volume, the present
of associated
malformations, and
the development of
hemodynamic
imbalance; the
prognosis of most
cases of the
complete absence of
the IHPVS are poor
Umbilical– The prenatal Necessary USS is often Given the high prevalence of
systemic shunts ultrasound detects associated with congestive heart failure and
(USS) that the UV directly deficiency of the DV edema, hemodynamic
connects to the and dysplasia of surveillance should be
systemic circulation, portal venous performed in fetal period
such as the right system; the risk of
atrium, the IVC, the chromosome
renal vein, or the iliac abnormality and
vein other structure
malformations is
high; fetuses with
USS have the
poorest prognosis,
and the lowest rates
of live birth and
postnatal survival are
observed
Ductus venosus- Prenatal ultrasound Necessary The fetuses with the Genetic examination should
systemic shunts shows abnormal shunt DVSS are associated be recommended for fetuses
(DVSS) between the DV and with a high incidence with DVSS detection to
the abdominal IVC, of chromosomal

the hepatic vein or the malformation and a exclude chromosomal
coronary sinus low risk of other abnormalities
structural
malformations;
fetuses with isolated
DVSS have a good
prognosis and
normal liver function
Congenital Prenatal ultrasound Necessary The CHPAVF can The prenatal diagnosis of
hepatoportal shows single or lead to high out-put CHPAVF enables better
arteriovenous multiple direct heart failure with a planning of postpartum
fistula (CHPAVF) communications mortality rate of management
between the hepatic 50% ~ 90%
artery and the portal
vein branches;
additional findings
include hepatic artery
enlargement, portal
vein dilatation at the
site of fistula and
abdominal aorta
tapering beyond the
celiac artery
Isolated absence Prenatal ultrasound Necessary Isolated absence or Close surveillance of fetal
or atresia of demonstrates that the atresia of DV had hemodynamic changes is
ductus venosus DV is absent or good prognosis in recommended
presents as a thin 67.2% cases, and
band connecting the died in perinatal
LPV and the IVC, and period as a result of
that CDFI examination fetal edema in 15.6%
could not detect the cases
blood flow signal
Abnormal entry of Prenatal ultrasound Necessary Prognosis is Sonographic surveillance
the umbilical vein shows the UV depended on and amniocentesis was

into the portal connecting with the thrombus formation, counseled; close
vein MPV after entering the a persistent portal postpartum
abdominal cavity, and thrombosis can ultrasonographic follow-up
the confluence of the cause portal is necessary; the case
UV and the MPV cavernoma with without thrombosis can be
presents as an portal hypertension conservatively observed and
aneurismal dilatation. that led to followed up; if a thrombosis
There is a variety of esophageal varices is suspected, early surgery
variations of influent is proposed in order to avoid
blood vessel at the persistent portal thrombosis
joint, most are the and its specific
SMV and the SV complications
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1/11/23, 1:19 PM A new classification of congenital abnormalities of UPVS: sonographic appearances, screening strategy and clinical significance | Insights

nical significance | Insights into Imaging | Full Text

Insights into Imaging

Table 1 Ultrasonographic manifestations,

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

New Subtypes Prenatal Prenatal Clinical findings Perinatal management

Support and Contact Terms and conditions

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