Clinical Radiology 73 (2018) 610e624

Contents lists available at ScienceDirect

Clinical Radiology
journal homepage: www.clinicalradiologyonline.net

Review

Role of radiological imaging and interventions

in management of BuddeChiari syndrome
C.J. Das a, *, M. Soneja b, S. Tayal b, A. Chahal a, S. Srivastava c, A. Kumar c,
U. Baruah d
a
Department of Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi e 110029, India
b
Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi e 110029, India
c
Department of Gastroenterology, GB Pant Hospital, New Delhi e 110002, India
d
Department of Anaesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi e 110029, India

article in formation
BuddeChiari syndrome (BCS) is a clinical condition resulting from impaired hepatic venous
Article history: drainage, in which there is obstruction to the hepatic venous outflow at any level from the
Received 21 September 2017 small hepatic veins to the junction of the inferior vena cava and the right atrium leading to
Accepted 8 February 2018 hepatic congestion. The diagnosis of BCS is based on imaging, which can be gathered from non-
invasive investigations such as ultrasonography coupled with venous Doppler, triphasic
computed tomography (CT) and magnetic resonance imaging (MRI). Apart from diagnosis,
various interventional radiology procedures aid in the successful management of this syn-
drome. In this article, we present various imaging features of BCS along with various inter-
ventional procedures that are used to treat this diverse condition.
Ó 2018 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

Introduction
BuddeChiari syndrome (BCS) is a clinical condition in
which there is obstruction to the hepatic venous outflow at
any level from the small hepatic veins to the junction of the
inferior vena cava (IVC) and the right atrium.1 In China, the
incidence of BCS was found to be 0.88/million per year with
an estimated prevalence of 7.69/million.2 This syndrome is
common in the 30e40 years age group with equal sex
predilection.3e6 In the Asian population there is a predi-
lection towards the terminal portion of IVC or a
combination of both IVC and hepatic veins, while pure he-
patic vein involvement is more frequently observed in the
West.2,7,8
Anatomically it is classified depending on the type of
venous involvement. Type I is limited to the IVC. Lesions
within the hepatic veins are classified as type II. It can be
further divided based on the length of segment involve-
ment. Segment occlusions of <4 cm are classified as Type IIa
and >4 cm as Type IIb. Involvement of both IVC and hepatic
veins is classified under BCS type III.9
Aetiology

Primary BCS is associated with various thrombophilic

* Guarantor and correspondent: C. J. Das, Present address. Department of
Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi e
110029, India. Tel.: þ91 11 26254390; fax: þ91 11 26588663.
E-mail address: dascj@yahoo.com (C.J. Das).
disorders, but it can also be multifactorial in origin (Fig 1).
Membranous obstructionehepatic vena cava syndrome is
an acquired condition, which is considered a separate entity

https://doi.org/10.1016/j.crad.2018.02.003
0009-9260/Ó 2018 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 611

Budd Chiari
syndrome

Idiopathic (16- Extrinsic

Intrinsic cause
35%)* compression

NeoplasƟc
HCC
Post phlebiƟs InfecƟve HepaƟc
stenosis RCC
ThromboƟc abscesses
(Membranous) Adrenal CC
HepaƟc cysts
(amoeboid) Sarcomas of IVC
Right atrial
myxoma

Inherited Acquired
Factor v leiden AnƟphospholipid
mutaƟon syndrome
Prothrombin PNH
g20210a hyperhomocysƟnemia
mutaƟon,
MyeloproliferaƟve
Protein c disorders
deficiency,
Oral contracepƟve pill
Protein s intake pregnancy
deficiency
Post liver
AnƟthrombin III transplantaƟon
deficiency

Figure 1 Common causes of BCS.4,13,14

from BCS. This condition is secondary to a bacterial Diagnosis

infection-induced thrombophlebitis, which eventually
transforms into a thick localised stenosis or obstruction.
This is more prevalent in lower socioeconomic groups with
poor hygiene conditions in South Africa and Asian
countries.7,10e12
Clinical presentation
The underlying pathophysiology behind BCS is increased
portal vein and hepatic sinusoid pressures with simulta-
neous decrease in portal venous flow. The result of
increased portal pressure is formation of ascites and venous
collaterals. Chronic increase in portal pressure with
decreased perfusion by portal flow slowly leads to hypoxic
cell death with centrilobular necrosis followed by fibrosis,
which eventually culminates into cirrhosis. Over a period of
time regenerative nodules may be seen in the periportal
area. In case of hepatic venous outflow obstruction, caudate
lobe hypertrophy is seen in up to 50% of the chronic cases
due to its direct drainage into the IVC. This hypertrophy
can further compromise the IVC flow by causing direct
compression or IVC stenosis.15
BCS should be suspected in patients who present with
clinical features of ascites, hepatomegaly, and abdominal
pain lacking features of chronic liver disease. Confirmation
of the diagnosis of BCS itself requires radiological evidence,
which can be gathered from non-invasive investigations
such as ultrasonography coupled with venous Doppler, tri-
phasic computed tomography (CT) and magnetic resonance
imaging (MRI). Familiarity with the imaging features of BCS
can aid radiological diagnoses and guide appropriate pa-
tient management.
Sonography
Ultrasound offers a simple and affordable method to
diagnose cases of BCS offering a sensitivity and specificity of
about 85%.16 In the acute phase of BCS, findings include an
enlarged liver with or without altered echogenicity pro-
duced by infarction and haemorrhage. Characteristics of
hepatic vein in BCS on ultrasound are variable echogenicity
of the lumen, visualisation of a thrombus, proximal dilata-
tion secondary to stenosis (Fig 2). A classical spider-web
612 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624

Figure 2 (a) Ultrasound image showing short segment stenosis of right hepatic vein extending to the ostium with proximal dilatation (long
arrow) and presence of collaterals (black arrow). (b) Doppler ultrasound image showing aliasing at the stenotic segment with increased velocity
and loss of triphasic pattern seen in the patent hepatic vein. (c) Ultrasound image of a different patient showing non-visualisation of the right
hepatic vein and is replaced by a fibrous, echogenic cord (white arrow). (d) Doppler ultrasound image showing an enlarged caudate lobe and
caudate vein (white arrow).

appearance near the hepatic vein ostia in the absence of
normal hepatic vein may be seen. Another specific obser-
vation is replacement of the hepatic vein by a fibrous,
echogenic cord (Fig 2). Focal obliteration of the lumen may
point towards a membranous web. Due to its unique
drainage, the caudate lobe remains spared with sonography
showing its hypertrophy confirmed by the measuring the
calibre of the caudate vein (>3 mm; Fig 2).9,17 As a result of
this, the IVC may be compressed. The IVC may be inde-
pendently involved with localised or long-segment nar-
rowing secondary to a thrombus or web. Small and tortuous
intrahepatic or sub-capsular collaterals are seen in almost
80% of the cases, which strongly favour the diagnosis of
chronic BCS.16,18 These collaterals have a characteristic
comma-shaped appearance (Fig 2). Doppler assessment
reveals changes in flow, such as monophasic or absent flow,
reversal of flow, turbulence in the hepatic veins as against a
triphasic pattern seen in patent hepatic veins, which is
attributable to the cardiac and respiratory cycle (Fig 2).
Another characteristic finding includes absent or flat he-
patic vein wave form without fluttering. The evaluation is
incomplete without reviewing the portal vein flow and
patency. The portal vein shows slow hepatofugal flow (<11
cm/s). Other features of BCS include ascites, splenomegaly,
coarse hepatic echotexture with or without large regener-
ative nodules, and portal hypertension.19 Sonography is also
useful in the follow-up after initial evaluation and treat-
ment (Fig 3).
CT
Triple-phase CT is another useful tool to diagnose BCS. It
is also useful when imaging of the vascular anatomy is
required in cases where transjugular intrahepatic porto-
systemic stent shunting (TIPSS) is planned.15 In the acute
phase, CT shows the absence of hepatic vein opacification,
but false-positive or indeterminable results can occur due
insufficient time delay after contrast medium injection. A
hypertrophied caudate lobe may be found in 75% of the
cases.18 On CT, the liver may have a mottled appearance
along with late enhancement of the periphery of the liver
and around the hepatic veins (Fig 4). This mottled appear-
ance results from central enhancement in the liver, espe-
cially at the level of the caudate lobe accompanied by hypo-
attenuation in the periphery of the liver owing to sinusoidal
and portal vein stasis.20,21
Findings of chronic BCS on CT are shrunken liver (Fig 4)
sparing the caudate lobe with intrahepatic collaterals.
These collateralised routes seen in BCS are the
left renalehemiazygos pathway, inferior phrenice
pericardiophrenic collaterals, and superficial collaterals of
the abdominal wall.20,22,23 Regenerative nodules may
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 613

Figure 3 (a) Pre-procedure sonography coupled with Doppler ultrasound showing a patent IVC. (b) Segmental narrowing of all hepatic veins in a
case of BCS. This patient underwent right hepatic vein angioplasty and stenting and follow-up sonography (c) and Doppler (d) showed a patent
stent.

appear hyperdense in both arterial and portal phase imag-
ing as these areas have preserved hepatic venous outflow
with decreased portal flow. These nodules are usually
multiple in numbers and their size range from 0.5 to 4 cm.
An important differential for such nodules is hepatocellular
carcinoma (HCC), which can be differentiated on CT. HCC
shows arterial enhancement with washout in portal phase
compared to hyper or iso-attenuation in case of regenera-
tive nodules.24 Hepatic artery enlargement can also be seen
as it compensates for the diminished portal flow. Stasis of
blood flow in the portal vein can produce portal vein
thrombosis.21
MRI
The MRI signal intensity is decided by the variations in
perfusion, necrosis, hypertrophy, and atrophy.21 It offers a
method to accurately diagnose acute and chronic BCS. In
the acute phase, MRI shows decreased T1-weighted and
slightly elevated T2-weighted signals in the central liver
accompanied with uneven increased arterial enhancement.
These signals are altered in the subacute phase where the
above-mentioned changes are now seen more in the pe-
riphery. In chronic BCS, MRI shows obstruction of the IVC,
collaterals, and a spider-web network pattern (Fig 5):
however, it is not as valuable as Doppler sonography to
expose collaterals as well as the direction of the flow. Spin-
echo and gradient-echo sequences and intravenous gado-
linium injection assist in visualisation of obstructed he-
patic veins and the IVC.25 Hypertrophy of spared segments
is seen leading to dysmorphic liver. The affected liver
segments may show enhancement on delayed post-
gadolinium images.26
614 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624

Figure 4 Contrast-enhanced CT in acute BCS showing an enlarged caudate which appeared mottled in the arterial phase (a) along with late
enhancement of the periphery of liver in the venous phase (b). (c) Contrast-enhanced CT showing a shrunken and dysmorphic liver in chronic
BCS.

The compensatory increase in hepatic arterial flow due nodular regenerative hyperplasia (NRH) and large regen-
to diminished portal flow encourages the development of erative nodules (LRN). In case of LRNs, because of the higher
regenerative nodules and the aberrant bile ducts in these copper load, the signals are usually hyperintense in T1 and
nodules. Two varieties of nodules exist, which include iso-to hypointense in T2 due to their increased regenerative

Figure 5 MRI images showing decreased T1-weighted (a) and slightly elevated T2 weighted (b, c) signals along with hypertrophied caudate,
intrahepatic collaterals (black arrow) and narrowed IVC (white arrow). (d) Contrast-enhanced 3D MR angiogram showing non-visualisation of
the IVC with presence of multiple bilateral paravertebral collaterals.
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 615

nature.27,28 A diagnosis of HCC associated with BCS can be
made on MRI also. Unenhanced MRI shows isointense or
hypointense lesions as compared to the parenchyma. These
malignant nodules show irregular and heterogeneous
enhancement in the arterial phase and a portal phase
washout. Conversely, benign lesions are multiple in num-
ber, smaller sized, regular, and homogeneously enhancing
on the arterial phase with slight hyperintensity in portal
phase.29
MR angiography is a useful technique to demarcate the
IVC and its course in detail. Contrast-enhanced three-
dimensional (3D) MR angiography (Fig 5) serves as an
important tool in establishing the diagnosis and treatment
planning of BCS.30e32
Management
The objective of the treatment is relieving hepatic
venous outflow obstruction and improving liver perfusion.
The mode of therapy is governed by prognostic factors,
potential for parenchymal recovery, surgical risk, and
availability of live donor as well as the stage of disease at
which the patient presents.33e35
The various steps of management are outlined in Fig 6.
The first step includes, initiating treatment of the underly-
ing condition as early as possible. In case of acute presen-
tation due to a recent thrombosis, thrombolysis may be
considered as the initial treatment of choice. Angioplasty
offers better outcomes if a short segment is involved;
however, it may be associated with recurrence. Other
decompression procedures include TIPSS, which bypasses
the liver and relieves the elevated portal pressures. Surgical
shunts are the mainstay in case all the above techniques fail,
but are associated with increased morbidity and are seldom
performed these days. Liver transplantation is the final step
in the management of BCS. In addition one must pay
attention to the early detection of complications, such as
portal hypertension, HCC, and other myeloproliferative
disorders. Attempts to correct the underlying nutritional
status must also be made. A stepwise approach had been
designed to direct the treatment of BCS and various studies
have evaluated the efficacy of the Plessier’s algorithm
approach.36,37
A single study has mentioned the definition of response
to treatment in which a set of six parameters are used36: (1)
absence of clinically detectable ascites, with normal serum
sodium and creatinine levels, in the absence of diuretic
therapy, or on low-dose diuretics (spironolactone 75 mg/
d or furosemide 40 mg/d) and moderate NaCl intake (6 g/d);
(2) increase in coagulation factor V to a level >40% of
normal value; (3) decrease in conjugated serum bilirubin to
a level <15 mol/l; (4) absence of first or recurrent portal
hypertension-related bleeding on primary or secondary
prophylaxis with non-selective beta-blockers or with
endoscopic therapy; (5) absence of spontaneous bacterial
infection; and (6) BMI 20 kg/m2 after subtraction of ascites
and oedema. Complete response was defined when all of
the six criteria were met and stable. Technical success was
defined as follows: for recanalisation with or without
stenting, when the pressure gradient across the IVC or

Budd chiari syndrome

Acute Subacute/Chronic Frank Cirrhosis

presentaon presentaon

Short segment Long segment

Ancoagulaon Thrombolysis stenosis/occlusion occlusion

Angioplasty +- Liver
Stenng Tansplantaon

Systemic Catheter
directed TIPSS
Thrombolysis thrombolysis

Figure 6 Management of BCS.

616 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624
hepatic vein stenosis fell to <5 mmHg; and for TIPSS, when
the porto-hepatic pressure gradient decreased to a level
<12 mmHg.36
Medical management
Medical therapy alone is recommended for patients with
no ongoing hepatic necrosis, relatively normal liver func-
tion results, and easily controllable ascites. Indicators of
poor prognosis include coagulopathy, encephalopathy, and
the hepatorenal syndrome, which deserve rapid relief of
hepatic venous obstruction.
Anticoagulation
The first step in the management requires administration
of indefinite anticoagulation despite lack of prospective
randomised controlled trials of anticoagulation in patients
with BCS.1 Based on data suggesting increase incidence of
Heparin-induced thrombocytopenia (HIT) with unfractio-
nated heparin more than low-molecular weight heparin, it
may be advisable to avoid unfractionated heparin.36 To evade
the risks associated with anticoagulants, a recent retro-
spective study in post-liver transplant patients showed that
hydroxyurea and aspirin were considered safer and effective
than warfarin in patients with BCS caused by Myeloprolif-
erative disorder (MPD).38 In a newly diagnosed case low-
molecular weight heparin should be started in view of its
rapid onset of action.39 Long-term anticoagulation is also
usually recommended post-therapeutic interventions such
as TIPSS, surgical portosystemic shunt, or liver transplant as
most patients have an underlying prothrombotic disorder.
The optimal level of anticoagulation in patients with BCS
needs evaluation and whether the conventional targets used,
namely anti Xa level 0.5e0.8 IU/ml for heparins and an INR of
2e3 for vitamin K antagonists, are appropriate for patients
with BCS receiving these agents.40,41
Thrombolysis
Catheter-assisted thrombolysis not only decreases sys-
temic exposure to the drug but also provides a route to
manage lesions such as venous webs or stenosis via an-
gioplasty or stenting.1,41 The preferred thrombolytic agent is
tissue plasminogen activator, which can be delivered via the
transjugular or transfemoral route just proximal to or
within the thrombosed hepatic vein. If combined with an-
gioplasty and/or stenting, better success rates are achieved
especially in patients with an acute thrombus. The difficulty
lies in identifying cases with acute obstruction but if
radiological investigations can confirm the diagnosis then
prompt treatment provides a good outcome.42
In a recent study by Zhang et al., complete dissolution of
thrombus was seen in nine out of 13 patients with BCS
complicated by thrombosis that underwent directed
thrombolysis. The efficacy of thrombolysis can be deter-
mined by the age of the thrombus (acute/subacute) as well as
the application of a side whole catheter for contiguous
thrombolysis.16,17 The sidehole catheter enables direct in-
jection into the clot, thus augmenting the drug concentration
and allowing reduced drug dosage.43,44 In a recent study
thrombolysis with pre-dilatation of the IVC was found to be
safe and efficacious as well as superior to thrombolysis alone
in cases of BCS with old IVC thrombosis.45 Mechanical
thrombus aspiration may be clubbed with IVC pre-dilatation
and thrombolysis, but this technique may not be applicable
for patients with segmental obstruction of the IVC or
massive thrombosis involving the entire IVC.46
Angioplasty and stenting
Balloon angioplasty and stenting offer an alternate
method to restore hepatic venous patency provided the
anatomy is amenable to the procedure. Establishing flow
even in one of the main three hepatic veins is ample for
clinical resolution of symptoms. Angioplasty (Fig 7) is
considered effective especially in cases of central and short
segment occlusion with patent hepatic vein segment. Stent
placement may be employed in case of re-stenosis or failure
of balloon angioplasty or in long-segment involvement
(Fig 8) A transjugular, femoral, or even ultrasound-guided
direct transhepatic approach is employed to gain access to
the vein. Determining the anatomical detail of the hepatic
veins before the procedure is crucial in identifying the most
appropriate hepatic vein to be targeted. Intrahepatic
obstruction or presence of collateral “spider webs” deem
the veins unsuitable for recanalisation.42
Recanalisation with angioplasty is useful in patients with
membranous/short segment occlusion of the hepatic vein.
Balloon dilatation alone of the IVC is usually effective for
opening of the stricture (Fig 9) or membranous web (Fig 10).
Stenting is deemed necessary mainly in cases of persistent
stricture despite dilatation (Fig 11). A transfemoral
approach is preferred in case of the IVC whereas hepatic
venous obstruction is accessed via the transjugular route, or
in rare cases, the percutaneous transhepatic approach. A
hepatic vein having a straight course, echo-free lumen, and
good calibre (7e8 mm in adults) is considered suitable for
interventions (Fig 2). In cases with combined IVC and he-
patic vein obstruction, it is challenging to negotiate across
the strictured segment and multiple approaches have to be
attempted to recanalise both the segments.24
Various studies from eastern populations describe
favourable outcomes with endovascular techniques in the
management of BCS. Zhang et al. along with other studies
reported satisfactory long-term patency using direct large
balloon dilation of the IVC and/or stent placement followed
by postoperative anticoagulation in patients with an old IVC
thrombus. Rates of re-occlusion were higher in patients
with segmental occlusion of the IVC as well as in absence of
postoperative anticoagulation.47e50
Ding et al. advocated angioplasty especially with a large
balloon for short segment occlusion of the hepatic vein.
Stent placement was considered predominantly in cases of
restenosis after repeat angioplasty or in case of long
segment involvement. As compared to TIPSS, angioplasty
was preferred as it was a safer procedure, minimally inva-
sive, achieved physiological hepatic blood flow with low
rate of procedure-related complications and re-occlusion. A
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 617

Figure 7 (a) Ultrasound image shows long segment proximal occlusion of right hepatic vein, mid-hepatic vein, and left hepatic vein. (b) After
failed mid-hepatic vein cannulation via the jugular route, mid-hepatic vein cannulation was performed via ipsilateral femoral access and
contrast run shows multiple collaterals. (c) Balloon angioplasty was performed showing a persistent wedge and (d) post-angioplasty run showed
complete disappearance of the collaterals.

balloon to hepatic vein diameter ratio of 1.5:1 to 2:1 was
deemed safer and optimal for narrowed hepatic venous
dilation. A dreaded complication of Percutaneous Trans-
hepatic Balloon Angioplasty (PTBA) includes hepatic vein
laceration and/or perforation by the large balloon and/or J-
type self-made blunt needle during the procedure and its
use was thus advised with great caution.51 It is inferred that
restenosis after angioplasty may be governed by factors
such as venous recoil, a low-flow state, thrombogenicity,
and impairment of the vein wall during the procedure as
well as the small size of balloon.51 The technical success rate
of angioplasty is especially high in patients with isolated
IVC obstruction.52 The success rate of angioplasty in cases of
pure hepatic vein occlusion is ambiguous40; however, data
from European centres suggest that angioplasty may not as
popular for treatment of BCS as compared to results
available from China. This could be explained by the pres-
ence of a distinct mechanism of hepatic venous obstruction
in the eastern population where hepatic vein stenosis is
more common.37 Membranous or short segment obstruc-
tion of the IVC or hepatic vein is more common in the East
whereas thrombosis particularly multiple segment
involvement is frequent in the West. This probably explains
widespread use of angioplasty in the Eastern population as
compared to the West.4,15,53
TIPSS
TIPSS placement involves creating an alternate pathway
across the portal vein and IVC for hepatic venous drainage,
thereby decompressing the congested liver. It has been re-
ported as being a successful treatment option in BCS.54,55
618 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624

Figure 8 (a) Contrast run from jugular access shows a patent IVC. (b) Contrast run post-right hepatic vein cannulation shows multiple collaterals
along with dilated distal and an occluded proximal right hepatic vein. (c) Balloon angioplasty of the right hepatic vein was performed followed by
placement of a 3710 mm balloon-mounted stent. (d) Note the non-opacification of collaterals in the post-stenting run. (e) Follow-up ultrasound
images show a normal-calibre distal right hepatic vein along with normal flow with the central venous waveform in the stented segment (f).

Figure 9 (a) Access via the femoral route showed narrowing of the suprarenal IVC (short arrow) with markedly dilated hemiazygos system (long
arrow). (b) Multiple intrahepatic collaterals in right paravertebral locations are also seen. (c) The stricture was negotiated and was dilated by a
4020 mm balloon. (d) Post-angioplasty run showed patent lumen of IVC with disappearance of collaterals.
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 619

Figure 10 (aeb) Ultrasound images showing short-segment narrowing of the IVC close to the cavo-atrial junction. Access via the jugular (c) and
femoral (d) route showed an abrupt cut-off of the IVC just distal to cavo-atrial junction. (e) The stricture was negotiated and was dilated using a
4020 mm balloon. (f) Post-angioplasty run showed a patent lumen of IVC with residual wedge suggesting membranous obstruction. (g) Ul-
trasound image showed opening of the IVC lumen.

Based on a study investigating the long-term outcome
following TIPSS from 1988 to 2008, an overall survival of
92% was seen and none of those patients subjected to TIPSS
required liver transplantation. Thus, as per the results, it
may be beneficial to use TIPSS as a first-line treatment
instead of awaiting failure of previous treatment steps.
TIPSS is more effective particularly when early intervention
and a high level of interventional experience is
available.36,56
In a recent study, TIPSS was implemented in 62 patients
(39.5%) as a rescue therapy after failure of medical and
minimally invasive treatments (angioplasty/stenting/
thrombolysis). The 5-year Orthotopic Liver transplantation
(OLT) free survival in these patients was 72%, which corre-
lates with the results of previous studies. The outcome
demonstrated by the study did not vary depending on the
timing of the TIPSS insertion. This suggests that patients can
be kept on close monitoring to detect those who fail to
respond to medical or minimally invasive treatments before
initiating procedures such as TIPSS.
This study also validated that the BCS-TIPS Prognostic
index(PI) score (based on International normalized ratio,
bilirubin and age) score >7 was the only independent factor
associated with poor survival and OLT-free survival after
TIPSS as previously reported.37,54 Tripathi et al. reported
excellent outcomes with TIPSS over a mean follow-up of
nearly 7 years. TIPSS was successful in reducing portal
pressure and preventing variceal bleeding and ascites in
99% of patients. The overall survival rates at 1 year and 5
years following TIPSS were 92% and 80%, respectively. All
patients received anticoagulation post-TIPSS. TIPSS was
associated with significant episodes of bleeding while 15%
had post-TIPSS encephalopathy, which was self-limiting in
most cases.57 These results likely are attributed to the high
level of experience in placing TIPSS at these centres. It can
be concluded that TIPSS should be considered first in those
failing medical management and recanalisation therapies
before proceeding to OLT, but a subgroup exists among
these patients in whom it might be preferable to choose
OLT, which is characterised by a high BCS-TIPS PI score.57,58
A recent meta-analysis by Zhang et al. evaluated the
outcomes of interventional treatment for BCS and found
that the success rate based on the pooled results with the
use of TIPSS was 96.4% (95% confidence interval [CI]:
94.2e98.6%). The rate of vascular stenosis after 1 year of the
intervention for TIPSS was 12% (95% CI: 8e16%). The pooled
result of the 1-year and 5-year survival rate post-TIPSS as
per the analysis was 87.3% (83.2e91.3%) and 72.1%
(67.2e77%), respectively.59 TIPSS is often the preferred
method with a good midterm outcome to ameliorate the
clinical features, especially in those with chronic BCS.24
TIPSS placement is not only recommended in patients
with failed thrombolytic therapy, with poor hepatic reserve,
with an occluded IVC, but also in acute emergent condi-
tions. TIPSS serves to provide a bridge to liver trans-
plantation and the shunt provides ample time to allow for
development of collaterals.42,60,61 In case of occurrence of
variceal bleeding in acute and chronic BCS, TIPSS may be
contemplated as the first line of treatment.24
TIPSS in comparison to surgical shunts is associated with
far less morbidity and mortality. TIPSS also allows bypassing
of caval stenosis unlike the surgical shunts by providing
outflow into the suprahepatic IVC.62 It also offers the
advantage of being a viable choice of treatment in cases of
620 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624

Figure 11 (a) Access via the femoral route showed complete narrowing of the suprahepatic IVC. (b) The stricture was negotiated and (c) was
dilated by a 4020 mm balloon. (d) Post-angioplasty image shows a patent lumen of the IVC. (e) As this patient had previously failed angio-
plasty, IVC stenting was performed. (f) Contrast-enhanced CT showed a patent IVC stent.

concomitant portal vein thrombosis.63 It decreases the need
for liver donors allowing the benefit of these grafts for other
liver diseases.36,64
TIPSS is not without its limitations, most importantly that
of shunt stenosis or thrombosis. Long-term shunt patency is
one of the most common problems and most patients with
BCS require long-term anticoagulation.65 These cases may
require follow-up interventions to maintain patency. The use
of polytetrafluoroethylene-covered stents has significantly
improved the patency rates as compared to uncovered stents
(67% at 1 year versus 19%). They are also associated with a
lower incidence of clinically significant events compared
with uncovered stents.66,67 TIPSS is also a technically difficult
procedure especially in view of lack of normal hepatic veins.
Interestingly, TIPSS is technically more difficult in patients
with BCS in contrast to cirrhosis and success rate varies from
80% in BCS versus >90% in cirrhosis in experienced cen-
tres.68,69 TIPSS has also been seen to be associated with a
higher rate of bleeding complications when performed in
patients with BCS as compared to patients of chronic liver
disease. The success and the complication rates following
TIPSS are also affected by the learning curve effect.36,70
Another potential complication associated with TIPSS is
misplacement or migration of TIPSS into the portal vein,
which can have adverse consequences if the patient goes on
to transplantation.34
In some cases of chronic BCS there may not be a visible
site of confluence of hepatic vein and IVC, such that TIPSS
may not be technically feasible. In such a scenario use of
direct porto-caval shunt may be employed (Fig 12). DIPS
(direct intraparenchymal portosystemic shunt) is a direct
cavo-portal shunt utilised in BCS when there is long-
segment occlusion of all the hepatic veins flush with the
ostium. DIPS is a technically challenging procedure as it
entails making a longer intraparenchymal tract. These pa-
tients typically have an enlarged caudate lobe, complicating
access to the portal vein and causing intrahepatic caval
compression. The average length of the intraparenchymal
tract in adult patients is often >6 cm.69 Multiple techniques
have been described for DIPS including usage of
 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624 621

Figure 12 (a) DIPS: contrast-enhanced image post-cannulation of the portal vein puncturing through the right wall of IVC using a Ross Uchida
transjugular puncture set shows portal venous opacification. (b) A guidewire was advanced until it reached the inferior mesenteric vein over
which a centimetre sizing catheter was placed and a contrast run was taken to measure the length of the stent (c). (d) The parenchymal tract was
dilated using a 1010 cm balloon followed by covered stent (1010 cm) placement in the parenchymal tract and uncovered stent in portal vein
overlapping each other (e). (f) Post-stenting contrast run showed complete opacification of the shunt.

intravascular ultrasound70 transabdominal ultrasound for
direct in-line percutaneous puncture of portal vein and
IVC71 or simultaneous utilisation of fluoroscopy and trans-
abdominal ultrasound in an oblique sagittal plane.69 Some
interventional radiologists demonstrate great expertise in
the DIPS procedure with fluoroscopy times of <10 minutes
and procedural time <1 hour and they have practically
replaced TIPSS by DIPS placement.70 On the contrary, the
present authors only use DIPS as a last resort. Although
there are no consensus guidelines to date, we advise that
only experienced operators who perform >10 TIPSS pro-
cedures per year with an experience exceeding 5 years
should attempt a DIPS placement owing to its inherent
technical complexity. The mere length of the tract decreases
the chances, thus increasing the attempts taken for a suc-
cessful puncture of portal vein branch. The intra-
parenchymal tract length can be minimised by accessing
the IVC below the level of the hepatic vein confluence.69 In
the authors’ experience, the expertise of an interventional
radiologist with ultrasound plays a crucial role in this
setting where a simultaneous visualisation of the needle
tract in fluoroscopy and transabdominal ultrasound in the
oblique parasagittal plane plays a major role for safe and
successful puncture. Utmost care should be taken to punc-
ture the portal veins within the intraparenchymal segment,
which avoids torrential intraperitoneal haemorrhage. A
100% success rate with the use of DIPS has been reported in
patients with BCS in small studies.72 The patency rates are
comparable to that of TIPSS with PTFE covered stent-grafts.
Venography is preferred over ultrasound to monitor for
DIPS stent graft patency. Acute liver failure is the most
feared complication of DIPS along with
haemoperitoneum.73,74
Liver transplantation
Liver transplantation is considered the treatment of
choice in patients with fulminant hepatic failure and in
cases with advanced liver cirrhosis. It is often the last resort
in the management of BCS in which all other treatment
techniques have failed. Patients of a younger age group,
with inherited pro-thrombotic conditions, shunt dysfunc-
tion should also be considered for liver transplantation.75
Segev et al. investigated the United States database
(United Network for Organ Sharing [UNOS] registry) of
patients who underwent liver transplantation for BCS from
622 C.J. Das et al. / Clinical Radiology 73 (2018) 610e624
1987 to 2006.76 The 1- and 3-year survival rates were 82%
and 76%, respectively, with a 90-day mortality of 15%. De-
terminants of poor patient survival included a longer cold
ischaemic time (>12 hours), preoperative life support, and
retransplantation. The use of TIPSS prior to transplantation
did not affect the patient outcome.3
In a study by Ulrich et al., patients with milder disease
were included with a ChildePugh score A or B, which
resulted in a 5-year survival rate of 89.4% not different from
the survival rate of untreated ChildePugh class A. The sur-
vival rate and graft function after OLT in BCS patients was
found to be superior to those who received a transplant for
other indications.77 In Asian countries due to the dearth of
deceased-donor liver grafts, living-donor liver trans-
plantation (LDLT) has been the mainstay for patients with
end-stage liver disease and acute liver failure, including
BCS.78 The Japanese Liver Transplant Society on the
nationwide LDLT registry in Japan reported 41 cases of BCS.
The 1-, 3-, 5-, and 10-year cumulative patient survival rates
after LDLT for BCS were 89%, 84%, 81%, and 81%, respec-
tively.79 With the routine use of the MELD score and ad-
vances in liver transplantation technology survival after OLT
in the management of BCS has improved in recent years.42
Anticoagulation is required as thrombotic complications
can recur post-transplantation; however, it is associated
with a high incidence of bleeding and thrombotic compli-
cations can occur despite anticoagulation, Thus the bene-
fit:hazard ratio of routine anticoagulation in patients
undergoing transplantation for BCS should be improved.36
Conclusion
To conclude, BCS has a diverse aetiology and presenta-
tion. Obstruction of the hepatic venous outflow at any level
from the small hepatic veins to the junction of the IVC and
the right atrium can lead to BCS. Ultrasonography coupled
with venous Doppler, triphasic CT, and MRI play significant
role in its diagnosis whereas interventional radiological
procedures, such as angioplasty and TIPSS, are vital for its
management. Liver transplantation is indicated in patients
with fulminant hepatic failure, advanced liver cirrhosis, and
in cases in which all other treatment methods have failed.
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