Alimentary Pharmacology & Therapeutics
Review article: the modern management of portal vein thrombosis
Y. CHAWLA, A. DUSEJA & R. K. DHIMAN
Department of Hepatology, Post-
graduate Institute of Medical Educa-
tion & Research, Chandigarh, India
Correspondence to:
Prof. Y. Chawla, Department of
Hepatology, Second Floor, D Block,
Room No-6, Postgraduate Institute of
Medical Education & Research,
Chandigarh 160 012, India.
E-mail: ykchawla@gmail.com
Publication data
Submitted 12 May 2009
First decision 23 June 2009
Resubmitted 14 July 2009
Accepted 10 August 2009
Epub Accepted Article 12 August
2009
ª 2009 Blackwell Publishing Ltd
doi:10.1111/j.1365-2036.2009.04116.x
SUMMARY
Background
Portal vein thrombosis (PVT) is an important cause of portal hyperten-
sion. It may occur as such with or without associated cirrhosis and
hepatocellular carcinoma. Information on its management is scanty.
Aim
To provide an update on the modern management of portal vein throm-
bosis. Information on portal vein thrombosis in patients with and with-
out cirrhosis and hepatocellular carcinoma is also updated.
Methods
A pubmed search was performed to identify the literature using search
items portal vein thrombosis-aetiology and treatment and portal vein
thrombosis in cirrhosis and hepatocellular carcinoma.
Results
Portal vein thrombosis occurs because of local inflammatory conditions
in the abdomen and prothrombotic factors. Acute portal vein thrombosis
is usually symptomatic when associated with cirrhosis and ⁄ or superior
mesenteric vein thrombosis. Anticoagulation should be given for 3–6
months if detected early. If prothrombotic factors are identified, anti-
coagulation should be given lifelong. Chronic portal vein thrombosis
usually presents with well tolerated upper gastrointestinal bleed. It is
diagnosed by imaging, which demonstrates a portal cavernoma in place
of a portal vein. Anticoagulation does not have a definite role, but
bleeds can be treated with endotherapy or shunt surgery. Rarely liver
transplantation may be considered.
Conclusion
Role of anticoagulation in chronic portal vein thrombosis needs to be
further studied.
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881
882 Y . C H A W L A et al.
INTRODUCTION
Portal vein thrombosis (PVT) indicates thrombosis that
develops in the trunk of the portal vein including its
right and left intrahepatic branches. It may even
extend to the splenic or superior mesenteric veins or
towards the liver involving intrahepatic portal
branches. It occurs either in association with cirrhosis
or malignancy of liver or may happen without an
associated liver disease. The term has been used syn-
onymously with Extra Hepatic Portal Venous Obstruc-
tion (EHPVO).
It is an important cause of noncirrhotic prehepatic
portal hypertension all over the world. In India, it
accounts for almost 30% of all variceal bleeds and is
the leading cause of variceal bleeding in children.1
Over the last few years, it has been increasingly diag-
nosed by the widespread use of ultrasound Doppler.
The lifetime risk of getting PVT in the general popula-
tion is reported to be 1%.2
AETIOLOGY
The aetiology of PVT has changed over the years.
Many prothrombotic states and local abdominal condi-
tions leading to PVT have been identified (Table 1).
The earlier labelled ‘idiopathic’ cases now have been
shown to be associated with thrombophilic conditions
identified in approximately 60% of patients and an
additional local predisposing factor in 30% of cases.3–9
In some cases, multiple prothrombotic factors may be
associated in the development of PVT.7, 10–12 In one
study, one or more risk factors namely prothrombotic
state or abdominal inflammation was present in 87%
of patients.13
Amongst the thrombophilic states, primary myelo-
proliferative disorders (MPD) are common. They were
earlier diagnosed by spontaneous formation of ery-
throid colonies in bone marrow culture.14, 15 However,
with the identification of Janus kinase 2 (JAK 2)
V617F gene mutation, the diagnosis of myeloprolifera-
tive disorders as a cause of PVT has increased by 20%.
It is now recommended by the WHO as a major diag-
nostic criterion for the diagnosis of MPD.16–18 PVT
may even be the first manifestation of myeloprolifera-
tive disease. In the West, latent MPD has been reported
in 58% of patients with idiopathic PVT and 51% of
these developed overt MPD on follow-up.19
Other prothrombotic conditions that cause PVT
include paroxysmal nocturnal haemoglobinuria (PNH),
Table 1. Etiologies in the causation of PVT
Inherited prothrombotic disorders
Factor V Leiden mutation
Factor II gene mutation
Protein C deficiency
Protein S deficiency
Antithrombin III deficiency
Acquired thrombophilic disorders
Primary myeloproliferative disorders
Paroxysmal nocturnal haemoglobinuria
Antiphospholipid syndrome
Hyperhomocysteinemia
Increased factor VIII levels
Thrombin activatable fibrinolysis inhibitor (TAFI) gene
mutation
Local factors
Inflammatory lesions
Pancreatitis, diverticulitis, cholecystitis, appendicitis,
omphalitis, liver abscess
Portal vein injury
Splenectomy, laparoscopic colectomy, abdominal trauma,
portocaval shunts, intra-abdominal surgical procedures,
FNAC pancreatic masses, RFA for HCC
Other risk factors
High altitude
Cirrhotic liver, Budd Chiari syndrome
HCC, Pancreatic carcinoma
Oral contraceptives, pregnancy
CMV & Bacteroides fragilis infections
Post liver transplantation
Idiopathic 10–30%
antiphospholipid syndrome, inherited prothrombotic
disorders such as protein C, S and antithrombin III
deficiencies, factor V Leiden mutation, factor II muta-
tion (G20210A) and methylenetetrahydrofolate reduc-
tase (MTHR) gene mutation.4–6 These inherited
disorders namely protein C, S and antithrombin III
deficiencies may be a secondary phenomenon rather
than primary as they are produced from the liver and
may be affected in parenchymal liver disease. They
may ultimately be confirmed by investigating first-
degree relatives.4, 7, 20 Recently, mutation in thrombin-
activatable fibrinolysis inhibitor (TAFI) gene and high
levels of factor VIII have been shown to be associated
with risk of PVT.21, 22
Rarely in association with these thrombophilic con-
ditions, PVT has been described with cytomegalovirus
infection23 and following endoscopic sclerotherapy in
patients with cirrhosis.24 A strong link of Bacteroides
fragilis infection with PVT has also been demonstrated
possibly because of transient development of anti-
cardiolipin antibodies.25 Other conditions that are
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associated with PVT are use of oral contraceptives,
pregnancy, chronic inflammatory diseases and malig-
nancies in the background of the above prothrombotic
causes.3
The intraabdominal inflammatory conditions leading
to sepsis that cause PVT include pancreatitis, cholecys-
titis, cholangitis, appendicitis, liver abscess and local
injury to portal venous axis following splenectomy
including laparoscopic splenectomy, laparoscopic co-
lectomy, abdominal trauma, portocaval shunts & other
intraabdominal surgical procedures in association with
the above acquired or inherited prothrombotic condi-
tions.26–30 PVT may also occur after ablative therapy
for HCC and fine needle aspiration of pancreatic
mass.31–33
Portal vein thrombosis sometimes occurs after liver
transplantation at the anastomotic site because of
donor ⁄ recepient portal vein diameter mismatch.34, 35
Portal vein thrombosis is an important complication
of cirrhosis occurring in 0.6–16% of patients with
compensated cirrhosis and 35% in patients with
decompensation and HCC.36, 37 PVT may also occur
with Budd Chiari syndrome mainly as a result of stag-
nant portal venous flow and an underlying prothrom-
botic state.38 Recently, thrombosis of the portal and
mesenteric veins has been described presenting as a
medical emergency in troops posted at high altitudes
in India.39
Transient PVT has been reported in 23% of patients
with acute pancreatitis and 57% in those with pancre-
atic necrosis.40
In the Asia-Pacific region, PVT in children has been
attributed to omphalitis, neonatal umbilical sepsis
overt or unrecognized and umbilical vein cannula-
tion.41 However, a study has shown that although
umbilical venous catheter associated thrombosis is
common, spontaneous resolution occurs in most
cases.42
Therapeutic contralateral portal vein embolisation
before major hepatic resection is practised at a few
centres, where the future remnant liver volume is likely
to be less than 30% of total liver. With successful
therapeutic PVT of the contralateral site, there is
hypertrophy of functional residual liver by 47% after
4–8 weeks, which helps in prevention of liver failure.43
PRESENTATION
It is important to know the haemodynamics once a
patient develops PVT, as the clinical manifestations
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are dependent on them. Initially, there is ‘compensa-
tory arterial vasodilatation’ or ‘arterial rescue’ that sta-
bilizes the liver functions as demonstrated by
clamping of the portal vein, which simulates acute
PVT. This is followed by a ‘venous rescue’ that
involves development of venous collaterals bypassing
the obstructed segment within a few days and forming
a ‘cavernoma’ in 3–5 weeks.44–46
Clinically, PVT may be acute or chronic. Although
no time frame exists, to distinguish acute from chronic
PVT, it is usually considered acute if symptoms devel-
oped <60 days prior to hospital assessment.47 This
may not hold true always as patients with chronic
PVT may first present with upper GI bleeding. An easy
way to differentiate acute PVT from chronic PVT is
the absence of or insignificant portoportal collaterals
on imaging and no evidence of portal hypertension
including splenomegaly and oesophageal varices. The
proportion of patients who develop chronic PVT from
acute is also not clearly established.
In acute PVT, there is a sudden formation of throm-
bosis within the portal vein that leads to a complete or
partial obstruction of the portal vein. The acute epi-
sode may be asymptomatic or nonspecific or if it
involves the superior mesenteric vein, may be associ-
ated with abdominal pain and dyspeptic symptoms.
The severity of symptoms depends on the rapidity and
extent of venous thrombosis. Involvement of superior
mesenteric vein and the mesenteric venous arches may
lead to intestinal ischaemia, bowel infarction and ileus.
Patients may then present with haematochezia,
rebound tenderness, fever and ascites. Bowel infarction
is an important cause of mortality in patients with
thrombosis of portal venous system.26 Partial obstruc-
tion of portal vein may be associated with lesser
symptoms.
Patients with chronic PVT present with portal hyper-
tension related complications like a well tolerated
variceal bleed, splenomegaly, anaemia and thrombo-
cytopenia or incidental detection following an imaging
procedure. Some patients may not have oesophago-
gastric varices, but have ectopic varices at sites like
duodenum, anorectum, colon or gall-bladder. These
ectopic varices are significantly more common in
chronic PVT than in patients with cirrhosis48–50
(Table 2).
Patients may have abdominal discomfort as a conse-
quence of massive splenomegaly and growth retarda-
tion possibly because of resistance to growth hormone
function and reduced insulin like growth factors.51
884 Y . C H A W L A et al.
Table 2. Presentation of PVT
Acute PVT Chronic PVT
Abdominal pain, nausea, fever: Asymptomatic
may be asymptomatic Well tolerated UGI bleeds
Associated SMVT- Abdominal Splenomegaly
pain, Hypersplenism
Intestinal ischaemia, Growth retardation
Haematochezia Obstructive Jaundice
Imaging- Portal cavernoma with
Portal vein thrombosis on collaterals
doppler US
A spurt in growth after shunt surgery in children has
been observed.52 Another study, however, did not
show any growth impairment in children with PVT.53
Ascites is unusual except when it occurs after GI
bleeding or if there is associated cirrhosis, but is easy
to control with medical treatment.54 Overt encephalop-
athy is rare, but minimal hepatic encephalopathy may
occur because of portosystemic stunting.55–57
Other rare clinical manifestations include obstructive
jaundice, cholangitis and even choledocholithasis late
in the natural course of the disease because of pseudo-
sclerosing cholangitis or portal hypertensive biliopa-
thy.58–63
Portal vein thrombosis in childhood is most often
diagnosed at the late chronic stage and presents with
features of portal hypertension.1
DIAGNOSIS
The liver function is normal or near normal except if
PVT occurs in a patient with cirrhosis. Patients with
portal hypertensive biliopathy may show a rise in
alkaline phosphatase.
Liver grossly is normal in PVT, but may show atro-
phy and regenerative nodular hyperplasia, related to
apoptosis and compensatory arterial vasodilation in
chronic PVT.64–66
ULTRASOUND
Ultrasound is the investigation of choice. Demonstra-
tion of a portal cavernoma is suggestive of chronic
PVT and is usually associated with splenomegaly and
collaterals in relation to portal venous system. Ultra-
sound shows solid echoes within portal vein and
absent flow on pulsed Doppler.67 It is the least
expensive method, but sensitivity and specificity are
affected by interpatient variability and expertise of
the given radiologist.68 The diagnostic sensitivity and
specificity for Colour Doppler Ultrasound (CDUS) in
detecting portal vein thrombosis vary from 66% to
100%.69, 70
Endoscopic ultrasound has also been reported to be
a sensitive and specific test to diagnose portal vein
thrombosis.71–73
CT and MRI
The CT and MRI provide additional information such
as extension of thrombus, evidence of bowel infarction
and status of adjacent organs. On noncontrast
enhanced CT, portal vein thrombus is generally seen
to be isodense to adjacent soft tissue, but may be
hyperdense if it occurred within a month.74 Following
intravenous administration of iodinated contrast on
CT, a bland thrombus is seen as a low density, non-
enhancing defect within portal veins, while a tumour
thrombus enhances following contrast administration.
Moreover, dynamic CT shows a filling defect partially
or totally occluding the vessel lumen with rim
enhancement of the vessel wall.75
The sensitivity and specificity of MRI for detecting
main PVT are 100% and 98% respectively in patients
undergoing liver transplantation, the discordance
being caused by a decreased calibre of main portal
vein that may be interpreted as recanalised chronic
thrombus.76 MR portography has been shown to be
superior to colour Doppler US in detecting partial
thrombosis and occlusion of the main portal venous
vessels. It also identifies portosplenic collaterals and
portal venous vessels more adequately than colour
Doppler. MR portographic demonstration of portal
vein occlusion may be reported as normal on CDUS
and vice versa because of slow flow velocity in portal
vein.77 True fast imaging with steady state precession
(true FISP) with MRI has been shown to be a useful
adjunct to contrast enhanced MR angiography in
severely debilitated patients, where respiratory motion
may degrade the images or when the use of contrast
medium is not possible because of poor venous
access.78
The MR portography is valuable in determining
the resectability of neoplasm involving the portal
venous system79 and follow-up after therapeutic pro-
cedures80 including surgical splenorenal and meso-
caval shunts.
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The MR angiography has precluded the use of effec-
tive, but more invasive techniques like carbon dioxide
portography or intraarterial digital subtraction
angiography.81, 82
The PET–CT has been shown to be helpful in dis-
criminating between benign and malignant portal vein
thrombosis.83
Splenoportovenography
This investigation involves injecting dye in the splenic
pulp and visualizing the splenoportal venous axis,
which helps not only in diagnosing PVT but also in
identifying the patency of splenoportal axis for future
shunt surgery. With respect to patients treated by us in
the preUS ⁄ CT ⁄ MRI era, it proved to be a safe proce-
dure, which also helped detecting the portal pressure
and assessing the effect of drugs, as HVPG is fallacious
in PVT.84
Endoscopy
It is important to have endoscopy in patients with PVT
as portal hypertensive gastropathy is more often pres-
ent in the acute PVT with cancer or cirrhosis, while
large oesophageal varices are present more often in
patients with chronic PVT.13
Procoagulant workup
Once the diagnosis of PVT is made, extensive investi-
gation of prothrombotic disorders and local factors is
recommended.3, 11 especially for patients with a life
expectancy of more than 3–6 months and where anti-
coagulation is considered relevant.24
PROGNOSIS
Portal vein thrombosis has been classified into four
categories depending on the extent of thrombosis: Type
I-Thrombosis limited to portal vein Type, II-extension
into SMV but patent mesenteric vessels, Type III-diffuse
thrombosis of splanchnic venous system with large
collaterals and Type IV-extensive splanchnic venous
thrombosis but with only fine collaterals.85 This classi-
fication helps not only in decision on operability, but
also on the presentation and prognosis, as patients with
SMV involvement (Type IV) have the worse prognosis,
while patients with only the main PVT(Type 1) present
with either no symptoms or do so with a bleed.86
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Mortality in the past was 20–50% with acute portal
vein thrombosis and other splanchnic vessels, but
with an early diagnosis, increased clinical awareness,
improved diagnostic techniques and use of early anti-
coagulation, the 5-years survival rate has improved
to 85%.55, 87 Outcome of PVT is generally good and
mortality is primarily resulting from underlying cause
and less from consequences of portal hypertension.
Mortality is highest at 1 year in patients with cancer
or cirrhosis compared with those without (26% vs.
8%).26, 88 The chances of a bleed in a patient with
PVT and large varices is much less as compared with
a patient with cirrhosis (0.25% over 2 years vs. 20–
30% over 2 years follow-up).89 Bleeding-related mor-
tality in patients with PVT is much lower than in
patients with cirrhosis because of preserved liver
function.9, 11, 26, 90
A multivariate analysis performed on determinants
of survival in extra hepatic portal vein thrombosis
showed that advanced age, malignancy, cirrhosis,
mesenteric vein thrombosis, absence of abdominal
inflammation, serum levels of aminotransferase and
albumin are associated with reduced survival but not
with complications of portal hypertension.11Chronic
PVT did not show any extension of the thrombus as
shown by repeat imaging after as long as 10 years.91
TREATMENT
Acute PVT
The aim of the treatment is to reverse or prevent
advancement of thrombosis in the portal venous sys-
tem and to treat complications of established PVT.
Most of the management decisions have to be individ-
ualized depending on the local expertise, as there is a
lack of randomized controlled trials (Table 3).
Anticoagulation. Early initiation of anticoagulation
preferably within 30 days of symptoms is recom-
mended as no spontaneous recanalisation is reported
except in acute pancreatitis. Recanalisation decreases
from 69% when anticoagulation was instituted within
first week to 25% when instituted in 2nd week.8 Thirty
five percent of acute PVT shows recanalisation with
early anticoagulation.8, 9, 55 In another study, early
anticoagulation could achieve recanalisation in 12 of
27 (40%) patients without cirrhosis and malignancy
compared with none of the 11 patients who were not
886 Y . C H A W L A et al.

insufficient evidence for starting anticoagulation in

Table 3. Treatment strategy
patients with portal cavernoma, although recanalisa-
Acute PVT tion in partial PVT has been reported in patients with
Early anticoagulation 3–6 months, lifelong if cirrhosis.95
Thrombectomy, prothrombotic disorder
Anticoagulation has also been shown to reverse
Thromboaspiration present or family history
Thrombolytic therapy of venous thrombosis or biliary abnormalities because of acute portal vein
previous thrombotic thrombosis.96
episode
Chronic PVT
? Anticoagulation Thrombolytic therapy. In very recent portal vein
Endotherapy thrombosis, local thrombolytic therapy via a catheter
Shunt surgery Warren Zeppa introduced into portal vein either transhepatically or
Side-to-side splenorenal through transjugular approach may improve regional
shunt
clot lysis.97–100 Catheterization of SMA operatively
Rex shunt
TIPS Mesenteric-gonadal shunt and intra-arterial infusion of thrombolytic drugs like
Liver transplantation recombinant tissue plasminogen activator,101 uroki-
Portal hypertensive biliopathy nase and streptokinase have all been shown to have
Endoscopic sphincterotomy ⁄ Shunt followed by biliary gratifying results.102 In a series of 20 patients with
balloon dilation & stent intervention or if shunt acute or subacute portal and mesenteric vein throm-
not possible – hepatico
jejunostomy
bosis with severe symptoms, treatment with thrombo-
lytic therapy was initiated via transhepatic, common
femoral or SMA routes. Fifteen of the 20 patients
exhibited some degree of lysis of the thrombosis,
three having complete resolution, 12 partial and five
given anticoagulation. Varices appeared as early as
no resolution. Eighty five percent had resolution of
1 month after PVT.8 In yet another study, anticoagula-
symptoms, but major complications including bleed-
tion was able to achieve recanalisation of acute
ing developed in 60% patients. The investigators felt
splanchnic venous thrombosis in 45.4% of patients
thus that thrombolytic therapy should be reserved
and prevented them from recurrent thrombosis when
for patients with severe disease. Most centres
given lifelong.92 Recanalisation is less likely, if the
now tend to choose a more conservative therapeutic
thrombosis is extensive because of more than one pro-
strategy.99
thrombotic disorder and is associated with ascites.92
Early anticoagulation in mesenteric and portal vein
thrombosis minimizes serious complications like peri- Thrombectomy. Surgical thrombectomy is associated
tonitis because of bowel necrosis and also significantly with recurrence of thrombosis, surgical morbidity
decreases the development of oesophageal varices- and mortality and hence not recommended. Mechan-
related complications.8 Recanalisation occurs within ical thrombectomy by percutaneous transhepatic
4–6 months after anticoagulation; hence, these route has the advantage of rapidly removing throm-
patients should be kept on anticoagulation for at least bus in a recently developed PVT (<30 days),
6 months.1, 12 although its drawbacks include intimal or vascular
Long-term anticoagulation may be recommended in trauma to the portal vein, that may promote recur-
patients with identified prothrombotic disorders, recur- rent thrombosis.103 Percutaneous transhepatic throm-
rent episodes of thrombosis or family history of boaspiration within 72 h has been performed
venous thrombosis.93 Anticoagulation should be initi- successfully in some patients.104 Mechanical aspira-
ated with heparin & maintained for 2–3 week. Subcu- tion thromobectomy during TIPS placement has also
taneous low molecular weight heparin is as effective been attempted successfully.105 Pharmacological lysis
as intravenous heparin, which makes laboratory moni- of the thrombus with local urokinase and local
toring unnecessary with a lower risk of bleeding or removal of the clot allowed restoration of normal
immune thrombocytopenia.94 Later oral Vit K antago- blood flow to the liver in three patients who devel-
nists should be given to maintain an INR of 2–3. More oped PVT following liver transplantation. Balloon
controversy exists on its role in chronic PVT. There is dilation and placement of vascular stent are helpful

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in decreasing the risk of recurrent thrombosis where
a defective surgical technique is the reason for
thrombosis.106
Chronic PVT
Acute gastrointestinal bleeding due to varices is trea-
ted in a similar way as in cirrhotics. No studies have
addressed the role of primary prophylaxis in PVT
associated portal hypertension. There is concern of
extension of thrombosis with b-blockers as well as
vasopressors because of decrease in splanchnic blood
flow.89 No report has addressed this complication with
terlipressin; but, a case report does exist with use of
vasopressin.107 Two retrospective studies have sug-
gested that beta adrenergic blockade may play a role
in secondary prophylaxis as they reduce the risk of
rebleeding and improve survival after variceal bleed.26
Endoscopic variceal ligation is safe and highly
effective in children and adults with PVT.108, 109 Band
ligation plus sclerotherapy is considered better in
treating children with chronic PVT than EST alone, as
it requires fewer sessions and fewer complications.110
Our own study has shown that variceal obliteration
following endoscopic sclerotherapy opens up sponta-
neous shunts because of a possible increase in portal
pressure in 40% of patients, which in turn protects
these patients from further bleeds and recurrence of
varices.111
Shunt. Decompressive shunt surgery should be con-
sidered in cases with failed endotherapy, although it
needs to be borne in mind that 37% of patients with
PVT also have thrombosis of splenic and superior mes-
enteric vein.112 It is also indicated for correcting
symptomatic portal hypertensive biliopathy, symptom-
atic hypersplenism and ‘on demand’ one-time
treatment.
Warren Zeppa distal splenorenal shunts have been
shown to be effective in control of bleeding and long-
term survival in patients with PVT.113 Mesocaval
shunts may be necessary when Warren shunt is pre-
cluded. Our own study of side-to-side lienorenal
shunts demonstrated that it not only prevented rebleed
but also corrected the hypersplenism.114
Rex shunt (mesenteric left portal by pass) between
the superior mesenteric vein and left portal vein is
widely used now and has been considered more
physiological over other shunts, which do not return
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blood directly to the liver.115 Although commonly
performed in children, its use in adults needs to be
validated.116 The intrahepatic portal vein is assessed
by dissecting the terminal branches of the left portal
vein in the recessus of Rex between segments III
and IV of the left lobe of the liver. The vein should
be relatively large for adequate flow of blood. It
should be avoided if cirrhosis coexists. This bypass
requires an autologous vein graft. The internal jugu-
lar vein provides a suitable and easily obtainable
venous conduit. An autogenous saphenous vein, and
a cryopreserved graft has also been used at some
centres.117 A novel shunt approach when other
surgical options fail includes using the right or
left gonadal vein for shunting with mesenteric
veins.118–120
In a few cases with PVT, TIPS has been shown to be
successful121, 122 in treating patients with portal biliop-
athy and portal vein thrombosis complicating Budd
Chiari syndrome. In 23 of the 28 patients with com-
plete portal vein thrombosis, success was achieved in
73% including 6 of 9 patients with cavernomatous
transformation.121
Another study on TIPS performed on 15 patients
with cirrhosis and PVT leading to refractory ascites,
variceal haemorrhage and refractory pleural effusion
showed its success in 75% (3 ⁄ 4) patients with caverno-
matous transformation and 10 ⁄ 11 (94%) patients with
acute PVT with an overall survival of 87%. It may
thus be a treatment option in certain patients.123 TIPS
is unsuccessful if the lumen of thrombosed portal vein
is not catheterizable and cavernomatous vein is not
amenable to dilatation.
Non shunt surgery. Oesophageal transection with or
without splenectomy is less useful to control bleeding
because of a high risk of late rebleeding and reap-
pearance of varices, but can be resorted to as a non-
shunt option in patients with portosystemic
encephalopathy, hepatopulmonary syndrome or porto-
pulmonary syndrome.124 Moreover, splenectomy
destroys the opportunity to use the splenic vein later
for a shunt. In MPD, spleen becomes an organ for
extramedullary marrow formation and should be
preserved.112
Liver Transplant. Portal vein thrombosis was consid-
ered a major obstacle to liver transplantation which
led to increased surgical complexity and perioperative
888 Y . C H A W L A et al.
morbidity and mortality. Following the successful
Liver Transplant (LT) in the presence of PVT in 1985,
experience has increased in this condition.125 Liver
transplantation in patients with cirrhosis and portal
vein thrombosis is associated with greater operative
complexity including increased operative time transfu-
sion requirements, rethrombosis and reintervention,
but has no influence on overall morbidity and mortal-
ity.126, 127 Surgical techniques like thrombectomy,
thromboendovenectomy with venous reconstitution,
interposition of vein graft, portocaval liver transposi-
tion have all been performed including radiological
endovascular interventions to overcome venous
obstruction in the recipient.128
For liver transplantation, PVT has been classified
into four grades (Yerdel grading) i.e. Grade 1 PV mini-
mal or partially thrombosed less than 50% of the ves-
sel lumen, Grade 2 more than 50% occlusion of the
PV including total occlusion, Grade 3 complete throm-
bosis of both PV and proximal SMV and Grade 4 com-
plete thrombosis of PV as well as proximal and distal
SMV.129 While patients with Grade 1–2 PVT can be
adequately managed by terminal-to-terminal portal
vein anastomosis with or without thrombectomy, in
the absence of available distal SMV segments for PV
reconstruction, dilated branches of recipient portal
venous system could be selected as inflow vessels.
With Grade 4 PVT, classic porto-caval hemitransposi-
tion is a widely accepted approach. Forty two of the
465 Liver Transplants have been performed at a centre
with associated PVT. This study found a higher inci-
dence of rethrombosis (7.1% vs. 0.9%) and renal fail-
ure (16.7% vs. 8.5%) because of complex surgical
procedure damaging the portal vein thrombosis and
prolonged anhepatic phase.130
Liver Transplant is indicated in a rare patient with
life threatening complications of PVT not manageable
conservatively or by shunt surgery as in those with
encephalopathy, hypoxia or pulmonary artery hyper-
tension.124 Outcome in patients with PVT is dependent
on the pre-operative liver disease severity as patients
with a MELD of <15 and PVT had a decreased 1-year
survival as compared with those without PVT (57% vs.
89%), while those with a MELD > 15 and PVT had an
equal and slightly better survival vs. non-PVT patients
(1 year survival 91% vs. 75%) with only slightly
increased morbidity.131
Liver transplant has also been performed in a
patient with bile duct complications because of
cavernoma.132
PVT IN SPECIAL SITUATIONS
Portal hyptertensive biliopathy
Portal hypertensive biliopathy (PHB) constitutes abnor-
malities of entire biliary tract in patients with portal
hypertension. It is seen in 81–100% of patients with
extrahepatic portal venous obstruction and has occa-
sionally been reported in cirrhotics and noncirrhotic
portal fibrosis.133 Portal hypertensive biliopathy may
also occur in children.134
It may either be asymptomatic or be associated with
jaundice, pruritus, fever or abdominal pain. It is iden-
tified as abnormalities of extra and intrahepatic bile
ducts because of either ischaemia of bile ducts or com-
pression by choledochal varices. These abnormalities
appear as indentations and stenosis which affect the
main bile duct and mostly the left hepatic duct. The
left hepatic duct is involved more often and this may
be because of formation of prominent collaterals,
where the umbilical vein originates from the left
branch of the portal vein. Portal hypertensive biliopa-
thy is diagnosed with ERCP or MRCP, wherein the bili-
ary tree mimics bead like appearances of primary
sclerosing cholangitis. MR cholangiography coupled
with MR portography has shown that the cholangio-
graphic abnormalities result from impingement of bile
duct lumen by veins comprising the cavernoma. MRCP
should be the investigation of choice. ERCP, however,
should be performed only if therapy is indicated.61
Ultrasound plays a minor role in diagnosing portal
hypertensive biliopathy as CBD is obscured by high
level echoes in the porta and multiple collaterals may
conceal the bile duct, but helps in demonstrating gall-
bladder varices that are seen in 30–55% of patients
with PVT.50 Endoscopic ultrasound is useful in identi-
fying CBD varices in the wall of CBD in 76% and sur-
rounding it in 52%. The ones present in the wall result
in obstructive jaundice.72
For patients with portal hypertensive biliopathy and
choledocholithiasis, sphincterotomy and extraction of
stone with balloon catheter and stent placement are
indicated.135, 136 Endoscopic sphincterotomy in such
patients needs to be performed carefully as occasion-
ally it may lead to haemobilia and uncontrolled bleed-
ing.137, 138 In some severe cases with recurrent
cholangitis associated with biliary strictures, a porto-
systemic shunt for choledochal variceal decompression
should be performed first followed by definitive biliary
surgery namely hepaticojejunostomy.139, 140
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd
 R E V I E W : T H E M O D E R N M A N A G E M E N T O F P O R T A L V E I N T H R O M B O S I S 889
If shunt surgery is not possible for some reasons, a
biliary stent should be placed with periodic replace-
ment.133 TIPS has also been performed successfully in
treating PHB.121
PVT in cirrhosis and HCC
Portal vein thrombosis in cirrhosis has been reported
to occur in 11.2% of the 701 cirrhotics studied.
Forty three percent of these were asymptomatic. Of
the 45(57%) symptomatic patients, 31 presented with
a variceal bleed, 14 with abdominal pain, 10 of
whom had intestinal infarction. Most of these
patients were in CTP class B & C. Mutation of pro-
thrombin gene was found to be the only thrombo-
philic condition associated with it.36 Another study
on PVT in cirrhotics concluded that prothrombotic
mutations by themselves are not causative of PVT,
but sclerotherapy and previous abdominal surgery
favour development of PVT in two-thirds of cases,
but is elusive in others.141
HCC is commonly associated with PVT. It is asso-
ciated with worse survival and indicates advanced
disease. Advanced stage, higher CTP class, low serum
albumin and high AFP levels are predictive of PVT
in patients with HCC.142 In a study on thrombophilic
genetic factors in 94 patients with HCC with and
without PVT, the Odds Ratio was 3.85 for MTHFR
C6777TT with HCC vs. healthy controls. Prothrombin
gene G 20210A mutation was also more frequent
among HCC patients mainly with PVT thereby con-
cluding that all cirrhotics should be looked for
thrombophilic genetic factors to individualize
patients at risk for PVT in HCC.143 Survival in PVT
and HCC has been shown to be better in those with
normal AFP.144
Contrast enhanced US (CEUS) has been found to
be superior to colour doppler sonography, conven-
tional US and CT in thrombus detection and charac-
terization complicating hepatic malignancies.145 In a
study, CEUS detected thrombus in 100% and cor-
rectly characterized it in 98% of patients, while CT
detected thrombus in 68% and correctly character-
ized it in 68% of them, CEUS may thus be helpful
in staging HCC.146
Patients with PVT and small HCC can be safely
treated with RFA.147 Similarly, Yttrium -90 glass
microspheres (Theraspheres) appear to be safe and
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd
well tolerated in patients with portal vein thrombosis
without cavernous transformation.148, 149 Similarly,
conformal radiotherapy induced a 45.8% objective
response rate for PVT in HCC and may be consid-
ered an important treatment option.150 Patients with
PVT in the setting of HCC has a poor patient out-
come. Patients should be assessed preoperatively
whether the thrombus is associated with tumour
invasion or with stagnant flow. In a study of 12
consecutive patients who underwent liver transplan-
tation for HCC in the setting of PVT, 42% had no
evident portal vein invasion and only 17% had
tumour thrombosis. Only one-third experienced
tumour recurrence in first year post-transplant and
one-third became long-term survivors (median
36 months) with no evidence of tumour recur-
rence.150 Radiation therapy is considered the treat-
ment of choice for selected patients with HCC and
PVT especially for those with a favourable perfor-
mance status.151
CONCLUSION
Portal vein thrombosis is being increasingly recog-
nized with or without underlying liver disease.
Patients should be investigated for thrombophilic con-
ditions as a cause of PVT. Imaging picks up cases of
PVT and helps in distinguishing patients with acute
PVT from chronic PVT as is evidenced by formation of
portal cavernoma. Although real time sonography with
colour Doppler is adequate for diagnosis and charac-
terization, some patients may require contrast
enhanced US or MR angiography. In symptomatic
noncarvenomatous PVT, anticoagulation is recom-
mended with low molecular weight heparin and oral
anticoagulants. In patients with cavernomatous forma-
tion of portal vein or chronic PVT, decompressive
shunt surgery, preferably Rex shunt, should be per-
formed if endotherapy fails to prevent bleeding from
varices especially in children. Rarely, TIPS may be
attempted in expert hands and if all these measures
fail or patient has features of hepato pulmonary syn-
drome or porto pulmonary syndrome, liver transplan-
tation may be considered.
ACKNOWLEDGEMENT
Declaration of personal and funding interests: None.
890 Y . C H A W L A et al.
REFERENCES
1 Sarin SK, Sollano JD, Chawla YK, et al.
Members of the APASL Working Party
on Portal Hypertension. Consensus on
extra-hepatic portal vein obstruction.
Liver Int 2006; 26: 512–9.
2 Ogren M, Bergqvist D, Björck M, et al.
Portal vein thrombosis: prevalence,
patient characteristics and lifetime risk: a
population study based on 23,796 con-
secutive autopsies. World J Gastroenterol
2006; 12: 2115–9.
3 Denninger MH, Chaı̈t Y, Casadevall N,
et al. Cause of portal or hepatic venous
thrombosis in adults: the role of multiple
concurrent factors. Hepatology 2000; 31:
587–91.
4 Janssen HL, Meinardi JR, Vleggaar FP,
et al. Factor V Leiden mutation, pro-
thrombin gene mutation, and deficiencies
in coagulation inhibitors associated with
Budd-Chiari syndrome and portal vein
thrombosis: results of a case-control
study. Blood 2000; 96: 2364–8.
5 Bombeli T, Basic A, Fehr J. Prevalence of
hereditary thrombophilia in patients with
thrombosis in different venous systems.
Am J Hematol 2002; 70: 126–32.
6 Bhattacharyya M, Makharia G, Kannan
M, et al. Inherited prothrombotic defects
in Budd-Chiari syndrome and portal vein
thrombosis: a study from North India.
Am J Clin Pathol 2004; 121: 844–7.
7 Primignani M, Martinelli I, Bucciarelli P,
et al. Risk factors for thrombophilia in
extrahepatic portal vein obstruction.
Hepatology 2005; 41: 603–8.
8 Turnes J, Garcı́a-Pagán JC, González M,
et al. Portal hypertension-related
complications after acute portal vein
thrombosis: impact of early anticoagu-
lation. Clin Gastroenterol Hepatol 2008;
6: 1412–7.
9 Plessier A, Murad SD, Hernandez Guerra
M, et al. A prospective multicentric fol-
low up study on 105 patients with
acute portal vein thrombosis: results
from EN-VIE. Hepatology 2007; 46(sup-
pl): 310A.
10 Rosendaal FR. Venous thrombosis: a mul-
ticausal disease. Lancet 1999; 353: 1167–
73.
11 Janssen HL, Wijnhoud A, Haagsma EB,
et al. Extrahepatic portal vein thrombo-
sis: aetiology and determinants of sur-
vival. Gut 2001; 49: 720–4.
12 De Franchis R. Evolving consensus in
portal hypertension. Report of the Baveno
IV consensus workshop on methodology
of diagnosis and therapy in portal hyper-
tension. J Hepatol 2005; 43: 167–76.
13 Sogaard KK, Astrup LB, Vilstrup H, Gron-
baek H. Portal vein thrombosis; risk fac-
tors, clinical presentation and treatment.
BMC Gastroenterol 2007; 7: 34.
14 De Stefano V, Teofili L, Leone G, et al.
Spontaneous erythroid colony formation
as the clue to an underlying myeloprolif-
erative disorder in patients with
Budd-Chiari syndrome or portal vein
thrombosis. Semin Thromb Hemost 1997;
23: 411–8.
15 Chait Y, Condat B, Cazals-Hatem D,
et al. Relevance of the criteria com-
monly used to diagnose myeloprolifera-
tive disorder in patients with splanchnic
vein thrombosis. Br J Haematol 2005;
129: 553–60.
16 Kiladjian JJ, Cervantes F, Leebeek FW,
et al. The impact of JAK2 and MPL muta-
tions on diagnosis and prognosis of
splanchnic vein thrombosis: a report on
241 cases. Blood 2008; 111: 4922–9.
17 Patel RK, Lea NC, Heneghan MA, et al.
Prevalence of the activating JAK2 tyro-
sine kinase mutation V617F in the Budd-
Chiari syndrome. Gastroenterology 2006;
130: 2031–8.
18 Tefferi A, Thiele J, Orazi A, et al. Propos-
als and rationale for revision of the
World Health Organization diagnostic
criteria for polycythemia vera, essential
thrombocythemia, and primary myelofi-
brosis: recommendations from an ad hoc
international expert panel. Blood 2007;
110: 1092–7.
19 Valla D, Casadevall N, Huisse MG, et al.
Myeloproliferative disorders in portal
vein thrombosis in adults. Gastroenterol-
ogy 1998; 94: 1063–9.
20 Fisher NC, Wilde JT, Roper J, et al. Defi-
ciency of natural anticoagulant proteins
C, S, and antithrombin in portal vein
thrombosis: a secondary phenomenon?
Gut 2000; 46: 534–9.
21 De Bruijne EL, Murad SD, de Maat MP,
et al. Liver and Thrombosis Study Group.
Genetic variation in thrombin-activatable
fibrinolysis inhibitor (TAFI) is associated
with the risk of splanchnic vein thrombo-
sis. Thromb Haemost 2007; 97: 181–5.
22 Martinelli I, Primignani M, Aghemo A,
et al. High levels of factor VIII and risk
of extra-hepatic portal vein obstruction.
J Hepatol 2009; 50: 916–22.
23 Amitrano L, Guardascione MA, Scaglione
M, et al. Acute portal and mesenteric
thrombosis: unusual presentation of
cytomegalovirus infection. Eur J Gastro-
enterol Hepatol 2006; 18: 443–5.
24 Amitrano L, Brancaccio V, Guardascione
MA, et al. Portal vein thrombosis after
variceal endoscopic sclerotherapy in
cirrhotic patients: role of genetic
thrombophilia. Endoscopy 2002; 34:
535–8.
25 Liappis AP, Roberts AD, Schwartz AM,
et al. Thrombosis and infection: a case of
transient anti-cardiolipin antibody asso-
ciated with pylephlebitis. Am J Med Sci
2003; 325: 365–8.
26 Condat B, Pessione F, Hillaire S, et al.
Current outcome of portal vein thrombo-
sis in adults: risk and benefit of antico-
agulant therapy. Gastroenterology 2001;
120: 490–7.
27 Poultsides GA, Lewis WC, Feld R, et al.
Portal vein thrombosis after laparoscopic
colectomy: thrombolytic therapy via the
superior mesenteric vein. Am Surg 2005;
71: 856–60.
28 Bernades P, Baetz A, Lévy P, et al. Splenic
and portal venous obstruction in
chronic pancreatitis. A prospective
longitudinal study of a medical-surgical
series of 266 patients. Dig Dis Sci 1992;
37: 340–6.
29 Fujita F, Lyass S, Otsuka K, et al. Portal
vein thrombosis following splenectomy:
identification of risk factors. Am Surg
2003; 69: 951–6.
30 Bick RL. Coagulation abnormalities in
malignancy: a review. Semin Thromb
Hemost 1992; 18: 353–72.
31 Habu D, Nishiguchi S, Shiomi S, et al.
Portal vein thrombosis following
percutaneous ethanol injection therapy
for hepatocellular carcinoma. Indian
J Gastroenterol 2002; 21: 162–3.
32 Zheng RQ, Kudo M, Inui K, et al. Tran-
sient portal vein thrombosis caused by
radiofrequency ablation for hepatocellu-
lar carcinoma. J Gastroenterol 2003; 38:
101–3.
33 Matsumoto K, Yamao K, Ohashi K, et al.
Acute portal vein thrombosis after EUS-
guided FNA of pancreatic cancer: case
report. Gastrointest Endosc 2003; 57:
269.
34 Langnas AN, Marujo W, Stratta RJ, et al.
Vascular complications after orthotopic
liver transplantation. Am J Surg 1991;
161: 76–82.
35 Doria C, Marino IR. Acute portal vein
thrombosis secondary to donor ⁄ recipient
portal vein diameter mismatch after
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd

orthotopic liver transplantation: a case
report. Int Surg 2003; 88: 184–7.
36 Amitrano L, Guardascione MA, Brancac-
cio V, et al. Risk factors and clinical pre-
sentation of portal vein thrombosis in
patients with liver cirrhosis. J Hepatol
2004; 40: 736–41.
37 Belli L, Romani F, Sansalone CV, et al.
Portal thrombosis in cirrhotics. A retro-
spective analysis. Ann Surg 1986; 203:
286–91.
38 Murad SD, Valla DC, de Groen PC, et al.
Pathogenesis and treatment of Budd-
Chiari syndrome combined with portal
vein thrombosis. Am J Gastroenterol
2006; 101: 83–90.
39 Anand AC, Sashindran VK, Mohan L. Gas-
trointestinal problems at high altitude.
Trop Gastroenterol 2006; 27: 147–53.
40 Dörffel T, Wruck T, Rückert RI, et al.
Vascular complications in acute pancrea-
titis assessed by color duplex ultrasonog-
raphy. Pancreas 2000; 21: 126–33.
41 Yadav S, Dutta AK, Sarin SK. Do umbili-
cal vein catheterization and sepsis lead to
portal vein thrombosis? A prospective,
clinical, and sonographic evaluation.
J Pediatr Gastroenterol Nutr 1993; 17:
392–6.
42 Sakha SH, Rafeey M, Tarzamani MK. Por-
tal venous thrombosis after umbilical
vein catheterization. Indian J Gastro-
enterol 2007; 26: 283–4.
43 Giraudo G, Greget M, Oussoultzoglou E,
et al. Preoperative contralateral portal
vein embolisation before major hepatic
resection is a safe and efficient proce-
dure: a large single institution experi-
ence. Surgery 2008; 143: 476–82.
44 Henderson JM, Gilmore GT, Mackay GJ,
et al. Hemodynamics during liver trans-
plantation: the interactions between car-
diac output and portal venous and
hepatic arterial flows. Hepatology 1992;
16: 715–8.
45 Ohnishi K, Okuda K, Ohtsuki T, et al. For-
mation of hilar collaterals or cavernous
transformation after portal vein obstruc-
tion by hepatocellular carcinoma. Obser-
vations in ten patients. Gastroenterology
1984; 87: 1150–3.
46 De Gaetano AM, Lafortune M, Patriquin
H, et al. Cavernous transformation of the
portal vein: patterns of intrahepatic and
splanchnic collateral circulation detected
with Doppler sonography. AJR Am J
Roentgenol 1995; 165: 1151–5.
47 Malkowski P, Pawlak J, Michalowicz B,
et al. Thrombolytic treatment of portal
thrombosis. Hepatogastroenterology 2003;
50: 2098–100.
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd
R E V I E W : T H E M O D E R N M A N A G E M E N T O F P O R T A L V E I N T H R O M B O S I S 891
48 Ganguly S, Sarin SK, Bhatia V, Lahoti D.
The prevalence and spectrum of colonic
lesions in patients with cirrhotic and
noncirrhotic portal hypertension. Hepa-
tology 1995; 21: 1226–31.
49 Chawla Y, Dilawari JB. Anorectal varices
their frequency in cirrhotic and noncirrh-
otic portal hypertension. Gut 1991; 32:
309.
50 Chawla Y, Dilawari JB. Gall Bladder vari-
ces in portal vein thrombosis. AJR 1994;
162: 643–5.
51 Sarin SK, Bansal A, Sasan S, Nigam A.
Portal vein obstruction in children leads
to growth retardation. Hepatology 1992;
15: 229–33.
52 de Ville de Goyet J, Alberti D, Falchetti
D, et al. Treatment of extrahepatic portal
hypertension in children by mesenteric-
to-left portal vein bypass: a new physio-
logical procedure. Eur J Surg 1999; 165:
777–81.
53 Bellomo – Brandao MA, Morcillo AM,
Hessel G, et al. Growth assessment in
children with extrahepatic portal vein
obstruction and portal hypertension.
Ar Gastro 2003; 40: 247–50.
54 Ranjan M, Gupta R, Jain M, Malhotra V,
Sarin SK. Hepatic dysfunction in
patients with extrahepatic portal
venous obstruction. Liver Int 2003; 23:
434–9.
55 Condat B, Pessine T, Helene DM, Hillaire
S, Valla D. Recent portal or mesentric
venous thrombosis, increased recognition
and frequent recanalisatioon on antico-
agulant therapy. Hepatology 2000; 32:
466–70.
56 Minguer B, Farua Papan JC, Bosch J,
et al. Non cirrhotic portal vein thrombo-
sis exhibits neuropsychological & MR
changes consistent with minimal hepatic
encephalopathy. Hepatology 2006; 43:
707–14.
57 Gsoenervey M, Quero JC, DeBraign I,
et al. Subclinical hepatic encephalopathy
impairs daily functioning. Hepatology
1998; 28: 45–9.
58 Dilawari JB, Chawla Y. Pseudoscleoring
cholangitis in extrahepatic portal
venous obstruction. Gut 1992; 33: 272–
6.
59 Khuroo MS, Yattoo GN, Zasyar SA, et al.
Biliary abnormalities associated with
extrahepatic portal venous obstruction.
Hepatology 1993; 17: 807–13.
60 Dhiman RK, Puri D, Chawla Y, et al. Bili-
ary changes in extrahepatic portal venous
obstruction compression by collaterals or
ischemia? Gastrointest Endosc 1999; 50:
646–52.
61 Condat B, Vilgram V, Asselak T, et al.
Portal carvernoma associated cholangiop-
athy a clinical and MR cholangiography
coupled with MR portography imaging
study. Hepatology 2003; 37: 1302–8.
62 Malkhan GH, Bhatia ST, Bastin K, et al.
cholangiography associated with portal
hypertension diagnostic evaluation and
clinical implications. Gastroint Endos
1999; 49: 344–8.
63 Sezgin O, Oguz D, Attritas E, Saritas U,
Sahin B. Endoscopic management of bili-
ary obstruction carried by cavernous
transformation of portal vein. Gastroint
Endos 2003; 58: 602–8.
64 Kobayashi S, Ng CS, Kazama T, et al.
Hemodynamic and morphological
changes after portal vein embolisation:
different efforts central and peripheral
zones in the liver on multiphasic com-
puted tomography. J Comput Assist
Tomog 2004; 28: 804–10.
65 Vilgrain V, Condat B, Bureau C, et al.
Atrophy-hypertrophy complex in patients
with cavernous transformation of the
portal vein: CT evaluation. Radiology
2006; 241: 149–55.
66 Bilodeau M, Aubry MC, Houle R, et al.
Evaluation of hepatocytes injury follow-
ing partial ligation of the left portal vein.
J Hepatol 1999; 30: 29–37.
67 Van Gansbeke D, Avni EF, Delcour C,
et al. Sonographic features of portal vein
thrombosis. AJR Am J Roentgenol 1985;
144: 749–52.
68 Wang JT, Zhao HY, Liu YL. Portal vein
thrombosis. Hepatobiliary Pancreat Dis
Int 2005; 4: 515–8.
69 Tessler FN, Gehring BJ, Gomes AS, et al.
Diagnosis of portal vein thrombosis:
value of color Doppler imaging. AJR Am
J Roentgenol 1991; 157: 293–6.
70 Parvey HR, Eisenberg RL, Giyanani V,
et al. Duplex sonography of the portal
venous system: pitfalls and limitations.
AJR Am J Roentgenol 1989; 152: 765–
70.
71 Lai L, Brugge WR. Endoscopic ultrasound
is a sensitive and specific test to
diagnose portal venous system thrombo-
sis (PVST). Am J Gastroenterol 2004; 99:
40–4.
72 Palazzo l, Hochain P, Helmer C, et al.
Biliary varieces on endoscopic ultra-
sonography: clinical presentation ad
outcome. Endoscopy 2000; 32: 520–4.
73 Umpheren JL, Pecha RE, Urayama S.
Biliary stricture caused by portal bilo-
pathy. Diagnosis by EUS with Doppler
US. Gasterintest Endoscopy 2004; 60:
1021–4.
892 Y . C H A W L A et al.
74 Parvey HR, Raval B, Sandler CM. Portal
vein thrombosis: imaging findings. AJR
Am J Roentgenol 1994; 162: 77–81.
75 Lee HK, Park SJ, Yi BH, et al. Portal vein
thrombosis: CT features. Abdom Imaging
2008; 33: 72–9.
76 Shah TU, Semelka RC, Voultsinos V, et al.
Accuracy of magnetic resonance imaging
for preoperative detection of portal vein
thrombosis in liver transplant candidates.
Liver Transpl 2006; 12: 1682–8.
77 Cakmak O, Elmas N, Tamsel S, et al. Role
of contrast-enhanced 3D magnetic reso-
nance portography in evaluating portal
venous system compared with color
Doppler ultrasonography. Abdom Imaging
2008; 33: 65–71.
78 Smith CS, Sheehy N, McEniff N, et al.
Magnetic resonance portal venography:
use of fast-acquisition true FISP imag-
ing in the detection of portal vein
thrombosis. Clin Radiol 2007; 62:
1180–8.
79 Lin J, Zhou KR, Chen ZW, et al. Three-
dimensional contrast-enhanced MR angi-
ography in diagnosis of portal vein
involvement by hepatic tumors. World J
Gastroenterol 2003; 9: 1114–8.
80 Glockner JF, Forauer AR, Solomon H,
et al. Three-dimensional gadolinium-
enhanced MR angiography of vascular
complications after liver transplantation.
AJR Am J Roentgenol 2000; 174: 1447–
53.
81 Vlachogiannakos J, Patch D, Watkinson
A, et al. Carbon-dioxide portography: an
expanding role? Lancet 2000; 355: 987–
8.
82 Kreft B, Strunk H, Flacke S, et al. Detec-
tion of thrombosis in the portal venous
system: comparison of contrast-enhanced
MR angiography with intraarterial digital
subtraction angiography. Radiology 2000;
216: 86–92.
83 Sun L, Guan YS, Pan WM, et al. Highly
metabolic thrombus of the portal vein:
18F fluorodeoxyglucose positron emis-
sion tomography ⁄ computer tomography
demonstration and clinical significance
in hepatocellular carcinoma. World J
Gastroenterol 2008; 14: 1212–7.
84 Dilawari JB, Chawla YK, Raju GS, Kaur
U, Bambery P. Splenoportovenography in
portal hypertension: a safe outpatient
procedure. Can Assoc Radiol J 1990; 41:
146–8.
85 Jamieson NV. Changing perspectives in
portal vein thrombosis and liver trans-
plantation. Transplantation 2000; 69:
1772–4.
86 Kumar S, Sarr MG, Kamath PS. Mesen-
teric venous thrombosis. N Engl J Med
2001; 345: 1683–8.
87 Baril N, Wren S, Radin R, Ralla P, Stain S.
The role of anticoagulation in pylephlebi-
tis. Am J Surg 1996; 172: 449–52.
88 Hidajat N, Stobbe H, Griesshaber V, Felix
R, Schroder RJ. Imaging and radiological
interventions of portal vein thrombosis.
Acta Radiol 2005; 46: 336–43.
89 Webster GJ, Burroughs AK, Riordan SM.
Review article: portal vein thrombosis –
new insights into aetiology and manage-
ment. Aliment Pharmacol Ther 2005; 21:
1–9.
90 Merkel C, Bolognesi M, Bellon S, et al. Long
term follow up study of adult patients with
noncirrhotis obstruction of the portal sys-
tem comparison with cirrhotic patients.
J Hepatol 1992; 15: 299–303.
91 Chawla YK, Dilawari JB. Portographic
changes with time in patients with extra-
hepatic portal venous obstruction. J Gas-
troenterol Hepatol 1988; 3: 421–4.
92 Amitrano L, Guardascione MA, Scaglione
M, et al. A Prognostic factors in non-
cirrhotic patients with splanchnic vein
thromboses. Am J Gastroenterol 2007;
102: 2464–70.
93 Garcia Pagan JC, Guerra MH, Bosch J.
Extrahepatic portal vein thrombosis.
Seminars in Liver diseases 2008; 28:
282–92.
94 Dolorich LR, Ginsberg JS, Douketis JD,
Holbrook AM, Chealig A. metaanalysis
comparing low molecular wt heparin
with unfractionated heparin in treatment
of venous thrombolism examining
some unanswered questions regarding
location of treatment product use and
dosing frequency. Arch Int Med 2000;
160: 181–8.
95 Francoz C, Belghiti J, Vilgrain V, et al.
Splanchnic vein thrombosis in candidates
for liver transplantation: usefulness of
screening and anticoagulation. Gut 2005;
54: 691–7.
96 Louvet A, Texier F, Dharancy S, et al.
Anticoagulation therapy may sense bili-
ary abnormalities due to acute portal
thrombosis. DDS 2006; 2006: 5111–7.
97 Lopeia JE, Correa G, Brazzini A, et al.
Percutaneous, transhepatic treatment of
symptomatic mesenteric venous thrombo-
sis. J Vasc Surg 2002; 36: 1058–67.
98 Aytekin C, Boyvat F, Kurt A, Yologhuz
coskun M. Catheter directed thrombolysis
with transjugular access in portal vein
thrombosis secondary to pancreatitis. Eur
J Radiol 2001; 39: 80–2.
99 Hollingshead M, Burke CT, Mauro MA,
Weetis SM, Dixon RG, Jaugus PF. Trans-
catheter thrombolytic therapy for acute
mesenteric and portal vein thrombosis.
J Vasc Inter Radiol 2005; 16: 651–61.
100 Kercher KW, Sing RF, Watson KW, et al.
Transhepatic thrombolysis in acute portal
vein thrombosis after laproscopic sple-
nectomy. Sung Laproso Endo Percutan
Tech 2002; 12: 131–6.
101 Henao EA, Bohamon WT, Silva MB Jr.
Treatment of portal vein thrombosis with
relative superior mesenteric artery
infusion of recombinant tissue plasmino-
gen activator. J Vase Surg 2003; 38:
1411–5.
102 Tateishi A, Mitsui M, Oki T, et al. Exten-
sive mesenteric vein and portal vein
thrombosis significantly treated by thro-
moltis and anticoagulation. J Gastroen-
terol Hepatol 2001; 16: 1429–33.
103 Uflacher R. Ajpheat of percutaneous
mechanical thromboectomy in TIPS and
PVT Tech. Vas Inter Radiol 2003; 6: 59–
69.
104 Oğuzkurt P, Tercan F, Ince E, Ezer SS,
Hiçsönmez A. Percutaneous treatment of
portal vein thrombosis in a child who
has undergone splenectomy. J Pediatr
Surg 2008; 43: e29–32.
105 Ferro C, Rossi CG, Bovio G, Dahamane
M, Centenaro M. TIPS mechanical aspira-
tion thembectory and directt thromboly-
sis in the treatment of acute portal and
superior mesenteric vein thrombosis. Car-
diovas Intervent Radiol 2007; 30: 1070–
4.
106 Adani GL, Baccarani U, Risaliti A, et al.
Percutaneous transhepatic portography
for the treatment of early portal vein
thrombosis after surgery. Cardiovasc In-
tervent Radiol 2007; 30: 1222–6.
107 Brearley S, Hawker PC, Dykes PW,
Keighley MR. A lethal complication of
peripheral vein vasopressin infusion.
Hepatogastroenterology 1985; 32: 224–5.
108 Celińska-Cedro D, Teisseyre M, Woyna-
rowski M, et al. Endoscopic ligation of
esophageal varices for prophylaxis of
first bleeding in children and adolescents
with portal hypertension: preliminary
results of a prospective study. J Pediatr
Surg 2003; 38: 1008–11.
109 Spaander MC, Murad SD, van Buuren HR,
et al. Endoscopic treatment of esophag-
ogastric variceal bleeding in patients
with noncirrhotic extrahepatic portal
vein thrombosis: a long-term follow-up
study. Gastrointest Endosc 2008; 67:
821–7.
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd

110 Poddar U, Thapa BR, Singh K. Band liga-
tion plus sclerotherapy versussclerothera-
py alone in children with extrahepatic
portal venous obstruction. J Clin Gastro-
enterol 2005; 39: 626–9.
111 Dilawari JB, Raju GS, Chawla YK.
Development of large spleno-adreno-
renal shunt after endoscopic sclero-
therapy. Gastroenterology 1989; 97:
421–6.
112 Wolff M, Hirner A. Current state of por-
tosystemic shunt surgery. Langenbecks
Arch Surg 2003; 388: 141–9.
113 Livingstone AS, Koniaris LG, Perez EA,
et al. 507 Warren-Zeppa distal splenore-
nal shunts: a 34-year experience. Ann
Surg 2006; 243: 884–92.
114 Sharma BC, Singh RP, Chawla YK, et al.
Effect of shunt surgery on spleen size,
portal pressure and oesophageal varices
in patients with non-cirrhotic portal
hypertension. J Gastroenterol Hepatol
1997; 12: 582–4.
115 Dasgupta R, Roberts E, Superina RA, Kim
PC. Effectiveness of Rex shunt in the
treatment of portal hypertension. J Pedi-
atr Surg 2006; 41: 108–12.
116 Query JA, Sandler AD, Sharp WJ. Use of
autogenous saphenous vein as a
conduit for mesenterico-left portal
vein bypass. J Pediatr Surg 2007; 42:
1137–40.
117 Krebs-Schmitt D, Briem-Richter A, Grab-
horn E, et al. Effectiveness of Rex shunt
in children with portal hypertension
following liver transplantation or with
primary portal hypertension. Pediatr
Transplant 2009; 13: 540–4.
118 Kim HB, Pomposelli JJ, Lillehei CW, et al.
Mesogonadal shunts for extrahepatic
portal vein thrombosis and variceal
hemorrhage. Liver Transpl 2005; 11:
1389–94.
119 Warren WD, Henderson JM, Millikan WJ,
et al. Management of variceal bleeding
in patients with noncirrhotic portal
vein thrombosis. Ann Surg 1988; 207:
623–34.
120 Orloff MJ, Orloff MS, Girard B, Orloff SL.
Bleeding esophagogastric varices from
extrahepatic portal hypertension:
40 years’ experience with portal-systemic
shunt. J Am Coll Surg 2002; 194: 717–
28.
121 Senzolo M, Tibbals J, Cholongitas E,
et al. Transjugular intrahepatic portosys-
temic shunt for portal vein thrombosis
with and without cavernous transforma-
tion. Aliment Pharmacol Ther 2006; 23:
767–75.
Aliment Pharmacol Ther 30, 881–894
ª 2009 Blackwell Publishing Ltd
R E V I E W : T H E M O D E R N M A N A G E M E N T O F P O R T A L V E I N T H R O M B O S I S 893
122 Bilbao JI, Elorz M, Vivas I, et al. Transju-
gular intrahepatic portosystemic shunt
(TIPS) in the treatment of venous symp-
tomatic chronic portal thrombosis in
non-cirrhotic patients. Cardiovasc Inter-
vent Radiol 2004; 27: 474–80.
123 Van Ha TG, Hodge J, Funaki B, et al.
Transjugular intrahepatic portosystemic
shunt placement in patients with cirrhosis
and concomitant portal vein thrombosis.
Cardiovasc Intervent Radiol 2006; 29:
785–90.
124 Superina R, Shneider B, Emre S, et al.
Surgical guidelines for the management
of extra-hepatic portal vein obstruction.
Pediatr Transplant 2006; 10: 908–13.
125 Shaw BW Jr, Iwatsuki S, Bron K, et al.
Portal vein grafts in hepatic transplanta-
tion. Surg Gynecol Obstet 1985; 161: 66–
8.
126 Lladó L, Fabregat J, Castellote J, et al.
Management of portal vein thrombosis in
liver transplantation: influence on mor-
bidity and mortality. Clin Transplant
2007; 2: 716–21.
127 Lendoire J, Raffin G, Cejas N, et al. Liver
transplantation in adult patients with
portal vein thrombosis: risk factors, man-
agement and outcome. HPB (Oxford)
2007; 9: 352–6.
128 Harmanci O, Bayraktar Y. Portal hyper-
tension due to portal venous thrombosis:
etiology, clinical outcomes. World J Gas-
troenterol 2007; 13: 2535–40.
129 Yerdel MA, Gunson B, Mirza D, et al.
Portal vein thrombosis in adults undergo-
ing liver transplantation : risk factors,
screening, management and outcome.
Transplantation 2000; 69: 1873–81.
130 Tao YF, Teng F, Wang ZX, et al. Liver
transplant recipients with portal vein
thrombosis: a single center retrospective
study. Hepatobiliary Pancreat Dis Int
2009; 8: 34–9.
131 Doenecke A, Tsui TY, Zuelke C, et al.
Pre-existent portal vein thrombosis in
liver transplantation: influence of pre-
operative disease severity. Clin Trans-
plant 2009; 19, Feb 19, PubMed.
132 Hajdu CH, Murakami T, Diflo T, et al. Int-
rahepatic portal cavernoma as an indica-
tion for liver transplantation. Liver
Transpl 2007; 13: 1312–6.
133 Dhiman RK, Behera A, Chawla YK, et al.
Portal hypertensive biliopathy. Gut 2007;
56: 1001–8.
134 Gauthier-Villars M, Franchi S, Gauthier
F, et al. Cholestasis in children with por-
tal vein obstruction. J Pediatr 2005; 146:
568–73.
135 Thervet L, Faulques B, Pissas A, et al.
Endoscopic management of obstructive
jaundice due to portal cavernoma. Endos-
copy 1993; 25: 423–5.
136 Bhatia V, Jain AK, Sarin SK. Choledocho-
lithiasis associated with portal biliopathy
in patients with extrahepatic portal vein
obstruction: management with endo-
scopic sphincterotomy. Gastrointest En-
dosc 1995; 42: 178–81.
137 Tighe M, Jacobson I. Bleeding from bile
duct varices: an unexpected hazard dur-
ing therapeutic ERCP. Gastrointest En-
dosc 1996; 43: 250–2.
138 Mutignani M, Shah SK, Bruni A, et al.
Endoscopic treatment of extrahepatic bile
duct strictures in patients with portal
biliopathy carries a high risk of haemo-
bilia: report of 3 cases. Dig Liver Dis
2002; 34: 587–91.
139 Chaudhary A, Dhar P, Sarin SK, et al.
Bile duct obstruction due to portal biliop-
athy in extrahepatic portal hypertension:
surgical management. Br J Surg 1998;
85: 326–9.
140 Vibert E, Azoulay D, Aloia T, et al.
Therapeutic strategies in symptomatic
portal biliopathy. Ann Surg 2007; 246:
97–104.
141 Mangia A, Villani MR, Cappucci G, et al.
Causes of portal venous thrombosis in
cirrhotic patients: the role of genetic and
acquired factors. Eur J Gastroenterol
Hepatol 2005; 17: 745–51.
142 Connolly GC, Chen R, Hyrien O, et al.
Incidence, risk factors and consequences
of portal vein and systemic thrombosesin
hepatocellular carcinoma. Thromb Res
2008; 122: 299–306.
143 D’Amico M, Pasta L, Sammarco P.
MTHFR C677TT, PAI1 4G-4G, V Leiden
Q506, and prothrombin G20210A in
hepatocellular carcinoma with and with-
out portal vein thrombosis. J Thromb
Thrombolysis 2008; 28: 70–3.
144 Carr BI, Buch SC, Kondragunta V, Panc-
oska P, Branch RA. Tumor and liver
determinants of prognosis in unresectable
hepatocellular carcinoma: a case cohort
study. J Gastroenterol Hepatol 2008; 23:
1259–66.
145 Rossi S, Rosa L, Ravetta V, et al. Con-
trast-enhanced versus conventional and
color Doppler sonography for the detec-
tion of thrombosis of the portal and
hepatic venous systems. AJR Am J
Roentgenol 2006; 186: 763–73.
146 Rossi S, Ghittoni G, Ravetta V, et al. Con-
trast-enhanced ultrasonography and
spiral computed tomography in the
894 Y . C H A W L A et al.

detection and characterization of portal (Therasphere) for the treatment of unre- of hepatocellular carcinoma and
vein thrombosis complicating hepatocel- sectable hepatocellular carcinoma in portal vein thrombosis: a challenging
lular carcinoma. Eur Radiol 2008; 18: patients with portal vein thrombosis. dilemma? Dig Dis Sci 2008; 53: 1994–
1749–56. J Vasc Interv Radiol 2004; 15: 335–45. 9.
147 Neeman Z, Libutti SK, Patti JW, Wood 149 Kulik LM, Carr BI, Mulcahy MF, et al. 151 Huang YJ, Hsu HC, Wang CY, et al. The
BJ. Percutaneous radiofrequency ablation Safety and efficacy of 90 Y radiotherapy treatment responses in cases of radiation
of hepatocellular carcinoma in the pres- for hepatocellular carcinoma with and therapy to portal vein thrombosis in
ence of portal vein thrombosis. Clin without portal vein thrombosis. Hepatolo- advanced hepatocellular carcinoma. Int J
Imaging 2003; 27: 417–20. gy 2008; 47: 71–81. Radiat Oncol Biol Phys 2009; 73: 1155–
148 Salem R, Lewandowski R, Roberts C, et al. 150 Sotiropoulos GC, Radtke A, Schmitz KJ, 63.
Use of Yttrium-90 glass microspheres et al. Liver transplantation in the setting

Aliment Pharmacol Ther 30, 881–894

ª 2009 Blackwell Publishing Ltd