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Conclusion
Role of anticoagulation in chronic portal vein thrombosis needs to be
further studied.
Portal vein thrombosis (PVT) indicates thrombosis that Inherited prothrombotic disorders
develops in the trunk of the portal vein including its Factor V Leiden mutation
right and left intrahepatic branches. It may even Factor II gene mutation
Protein C deficiency
extend to the splenic or superior mesenteric veins or Protein S deficiency
towards the liver involving intrahepatic portal Antithrombin III deficiency
branches. It occurs either in association with cirrhosis Acquired thrombophilic disorders
or malignancy of liver or may happen without an Primary myeloproliferative disorders
associated liver disease. The term has been used syn- Paroxysmal nocturnal haemoglobinuria
Antiphospholipid syndrome
onymously with Extra Hepatic Portal Venous Obstruc-
Hyperhomocysteinemia
tion (EHPVO). Increased factor VIII levels
It is an important cause of noncirrhotic prehepatic Thrombin activatable fibrinolysis inhibitor (TAFI) gene
portal hypertension all over the world. In India, it mutation
accounts for almost 30% of all variceal bleeds and is Local factors
the leading cause of variceal bleeding in children.1 Inflammatory lesions
Pancreatitis, diverticulitis, cholecystitis, appendicitis,
Over the last few years, it has been increasingly diag- omphalitis, liver abscess
nosed by the widespread use of ultrasound Doppler. Portal vein injury
The lifetime risk of getting PVT in the general popula- Splenectomy, laparoscopic colectomy, abdominal trauma,
tion is reported to be 1%.2 portocaval shunts, intra-abdominal surgical procedures,
FNAC pancreatic masses, RFA for HCC
Other risk factors
AETIOLOGY High altitude
Cirrhotic liver, Budd Chiari syndrome
The aetiology of PVT has changed over the years. HCC, Pancreatic carcinoma
Many prothrombotic states and local abdominal condi- Oral contraceptives, pregnancy
tions leading to PVT have been identified (Table 1). CMV & Bacteroides fragilis infections
The earlier labelled ‘idiopathic’ cases now have been Post liver transplantation
Idiopathic 10–30%
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 antiphospholipid syndrome, inherited prothrombotic
In some cases, multiple prothrombotic factors may be disorders such as protein C, S and antithrombin III
associated in the development of PVT.7, 10–12 In one deficiencies, factor V Leiden mutation, factor II muta-
study, one or more risk factors namely prothrombotic tion (G20210A) and methylenetetrahydrofolate reduc-
state or abdominal inflammation was present in 87% tase (MTHR) gene mutation.4–6 These inherited
of patients.13 disorders namely protein C, S and antithrombin III
Amongst the thrombophilic states, primary myelo- deficiencies may be a secondary phenomenon rather
proliferative disorders (MPD) are common. They were than primary as they are produced from the liver and
earlier diagnosed by spontaneous formation of ery- may be affected in parenchymal liver disease. They
throid colonies in bone marrow culture.14, 15 However, may ultimately be confirmed by investigating first-
with the identification of Janus kinase 2 (JAK 2) degree relatives.4, 7, 20 Recently, mutation in thrombin-
V617F gene mutation, the diagnosis of myeloprolifera- activatable fibrinolysis inhibitor (TAFI) gene and high
tive disorders as a cause of PVT has increased by 20%. levels of factor VIII have been shown to be associated
It is now recommended by the WHO as a major diag- with risk of PVT.21, 22
nostic criterion for the diagnosis of MPD.16–18 PVT Rarely in association with these thrombophilic con-
may even be the first manifestation of myeloprolifera- ditions, PVT has been described with cytomegalovirus
tive disease. In the West, latent MPD has been reported infection23 and following endoscopic sclerotherapy in
in 58% of patients with idiopathic PVT and 51% of patients with cirrhosis.24 A strong link of Bacteroides
these developed overt MPD on follow-up.19 fragilis infection with PVT has also been demonstrated
Other prothrombotic conditions that cause PVT possibly because of transient development of anti-
include paroxysmal nocturnal haemoglobinuria (PNH), cardiolipin antibodies.25 Other conditions that are
associated with PVT are use of oral contraceptives, are dependent on them. Initially, there is ‘compensa-
pregnancy, chronic inflammatory diseases and malig- tory arterial vasodilatation’ or ‘arterial rescue’ that sta-
nancies in the background of the above prothrombotic bilizes the liver functions as demonstrated by
causes.3 clamping of the portal vein, which simulates acute
The intraabdominal inflammatory conditions leading PVT. This is followed by a ‘venous rescue’ that
to sepsis that cause PVT include pancreatitis, cholecys- involves development of venous collaterals bypassing
titis, cholangitis, appendicitis, liver abscess and local the obstructed segment within a few days and forming
injury to portal venous axis following splenectomy a ‘cavernoma’ in 3–5 weeks.44–46
including laparoscopic splenectomy, laparoscopic co- Clinically, PVT may be acute or chronic. Although
lectomy, abdominal trauma, portocaval shunts & other no time frame exists, to distinguish acute from chronic
intraabdominal surgical procedures in association with PVT, it is usually considered acute if symptoms devel-
the above acquired or inherited prothrombotic condi- oped <60 days prior to hospital assessment.47 This
tions.26–30 PVT may also occur after ablative therapy may not hold true always as patients with chronic
for HCC and fine needle aspiration of pancreatic PVT may first present with upper GI bleeding. An easy
mass.31–33 way to differentiate acute PVT from chronic PVT is
Portal vein thrombosis sometimes occurs after liver the absence of or insignificant portoportal collaterals
transplantation at the anastomotic site because of on imaging and no evidence of portal hypertension
donor ⁄ recepient portal vein diameter mismatch.34, 35 including splenomegaly and oesophageal varices. The
Portal vein thrombosis is an important complication proportion of patients who develop chronic PVT from
of cirrhosis occurring in 0.6–16% of patients with acute is also not clearly established.
compensated cirrhosis and 35% in patients with In acute PVT, there is a sudden formation of throm-
decompensation and HCC.36, 37 PVT may also occur bosis within the portal vein that leads to a complete or
with Budd Chiari syndrome mainly as a result of stag- partial obstruction of the portal vein. The acute epi-
nant portal venous flow and an underlying prothrom- sode may be asymptomatic or nonspecific or if it
botic state.38 Recently, thrombosis of the portal and involves the superior mesenteric vein, may be associ-
mesenteric veins has been described presenting as a ated with abdominal pain and dyspeptic symptoms.
medical emergency in troops posted at high altitudes The severity of symptoms depends on the rapidity and
in India.39 extent of venous thrombosis. Involvement of superior
Transient PVT has been reported in 23% of patients mesenteric vein and the mesenteric venous arches may
with acute pancreatitis and 57% in those with pancre- lead to intestinal ischaemia, bowel infarction and ileus.
atic necrosis.40 Patients may then present with haematochezia,
In the Asia-Pacific region, PVT in children has been rebound tenderness, fever and ascites. Bowel infarction
attributed to omphalitis, neonatal umbilical sepsis is an important cause of mortality in patients with
overt or unrecognized and umbilical vein cannula- thrombosis of portal venous system.26 Partial obstruc-
tion.41 However, a study has shown that although tion of portal vein may be associated with lesser
umbilical venous catheter associated thrombosis is symptoms.
common, spontaneous resolution occurs in most Patients with chronic PVT present with portal hyper-
cases.42 tension related complications like a well tolerated
Therapeutic contralateral portal vein embolisation variceal bleed, splenomegaly, anaemia and thrombo-
before major hepatic resection is practised at a few cytopenia or incidental detection following an imaging
centres, where the future remnant liver volume is likely procedure. Some patients may not have oesophago-
to be less than 30% of total liver. With successful gastric varices, but have ectopic varices at sites like
therapeutic PVT of the contralateral site, there is duodenum, anorectum, colon or gall-bladder. These
hypertrophy of functional residual liver by 47% after ectopic varices are significantly more common in
4–8 weeks, which helps in prevention of liver failure.43 chronic PVT than in patients with cirrhosis48–50
(Table 2).
Patients may have abdominal discomfort as a conse-
PRESENTATION
quence of massive splenomegaly and growth retarda-
It is important to know the haemodynamics once a tion possibly because of resistance to growth hormone
patient develops PVT, as the clinical manifestations function and reduced insulin like growth factors.51
The MR angiography has precluded the use of effec- Mortality in the past was 20–50% with acute portal
tive, but more invasive techniques like carbon dioxide vein thrombosis and other splanchnic vessels, but
portography or intraarterial digital subtraction with an early diagnosis, increased clinical awareness,
angiography.81, 82 improved diagnostic techniques and use of early anti-
The PET–CT has been shown to be helpful in dis- coagulation, the 5-years survival rate has improved
criminating between benign and malignant portal vein to 85%.55, 87 Outcome of PVT is generally good and
thrombosis.83 mortality is primarily resulting from underlying cause
and less from consequences of portal hypertension.
Mortality is highest at 1 year in patients with cancer
Splenoportovenography
or cirrhosis compared with those without (26% vs.
This investigation involves injecting dye in the splenic 8%).26, 88 The chances of a bleed in a patient with
pulp and visualizing the splenoportal venous axis, PVT and large varices is much less as compared with
which helps not only in diagnosing PVT but also in a patient with cirrhosis (0.25% over 2 years vs. 20–
identifying the patency of splenoportal axis for future 30% over 2 years follow-up).89 Bleeding-related mor-
shunt surgery. With respect to patients treated by us in tality in patients with PVT is much lower than in
the preUS ⁄ CT ⁄ MRI era, it proved to be a safe proce- patients with cirrhosis because of preserved liver
dure, which also helped detecting the portal pressure function.9, 11, 26, 90
and assessing the effect of drugs, as HVPG is fallacious A multivariate analysis performed on determinants
in PVT.84 of survival in extra hepatic portal vein thrombosis
showed that advanced age, malignancy, cirrhosis,
mesenteric vein thrombosis, absence of abdominal
Endoscopy
inflammation, serum levels of aminotransferase and
It is important to have endoscopy in patients with PVT albumin are associated with reduced survival but not
as portal hypertensive gastropathy is more often pres- with complications of portal hypertension.11Chronic
ent in the acute PVT with cancer or cirrhosis, while PVT did not show any extension of the thrombus as
large oesophageal varices are present more often in shown by repeat imaging after as long as 10 years.91
patients with chronic PVT.13
TREATMENT
Procoagulant workup
Acute PVT
Once the diagnosis of PVT is made, extensive investi-
gation of prothrombotic disorders and local factors is The aim of the treatment is to reverse or prevent
recommended.3, 11 especially for patients with a life advancement of thrombosis in the portal venous sys-
expectancy of more than 3–6 months and where anti- tem and to treat complications of established PVT.
coagulation is considered relevant.24 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).
PROGNOSIS
Portal vein thrombosis has been classified into four
categories depending on the extent of thrombosis: Type Anticoagulation. Early initiation of anticoagulation
I-Thrombosis limited to portal vein Type, II-extension preferably within 30 days of symptoms is recom-
into SMV but patent mesenteric vessels, Type III-diffuse mended as no spontaneous recanalisation is reported
thrombosis of splanchnic venous system with large except in acute pancreatitis. Recanalisation decreases
collaterals and Type IV-extensive splanchnic venous from 69% when anticoagulation was instituted within
thrombosis but with only fine collaterals.85 This classi- first week to 25% when instituted in 2nd week.8 Thirty
fication helps not only in decision on operability, but five percent of acute PVT shows recanalisation with
also on the presentation and prognosis, as patients with early anticoagulation.8, 9, 55 In another study, early
SMV involvement (Type IV) have the worse prognosis, anticoagulation could achieve recanalisation in 12 of
while patients with only the main PVT(Type 1) present 27 (40%) patients without cirrhosis and malignancy
with either no symptoms or do so with a bleed.86 compared with none of the 11 patients who were not
in decreasing the risk of recurrent thrombosis where blood directly to the liver.115 Although commonly
a defective surgical technique is the reason for performed in children, its use in adults needs to be
thrombosis.106 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
Chronic PVT
and IV of the left lobe of the liver. The vein should
Acute gastrointestinal bleeding due to varices is trea- be relatively large for adequate flow of blood. It
ted in a similar way as in cirrhotics. No studies have should be avoided if cirrhosis coexists. This bypass
addressed the role of primary prophylaxis in PVT requires an autologous vein graft. The internal jugu-
associated portal hypertension. There is concern of lar vein provides a suitable and easily obtainable
extension of thrombosis with b-blockers as well as venous conduit. An autogenous saphenous vein, and
vasopressors because of decrease in splanchnic blood a cryopreserved graft has also been used at some
flow.89 No report has addressed this complication with centres.117 A novel shunt approach when other
terlipressin; but, a case report does exist with use of surgical options fail includes using the right or
vasopressin.107 Two retrospective studies have sug- left gonadal vein for shunting with mesenteric
gested that beta adrenergic blockade may play a role veins.118–120
in secondary prophylaxis as they reduce the risk of In a few cases with PVT, TIPS has been shown to be
rebleeding and improve survival after variceal bleed.26 successful121, 122 in treating patients with portal biliop-
Endoscopic variceal ligation is safe and highly athy and portal vein thrombosis complicating Budd
effective in children and adults with PVT.108, 109 Band Chiari syndrome. In 23 of the 28 patients with com-
ligation plus sclerotherapy is considered better in plete portal vein thrombosis, success was achieved in
treating children with chronic PVT than EST alone, as 73% including 6 of 9 patients with cavernomatous
it requires fewer sessions and fewer complications.110 transformation.121
Our own study has shown that variceal obliteration Another study on TIPS performed on 15 patients
following endoscopic sclerotherapy opens up sponta- with cirrhosis and PVT leading to refractory ascites,
neous shunts because of a possible increase in portal variceal haemorrhage and refractory pleural effusion
pressure in 40% of patients, which in turn protects showed its success in 75% (3 ⁄ 4) patients with caverno-
these patients from further bleeds and recurrence of matous transformation and 10 ⁄ 11 (94%) patients with
varices.111 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
Shunt. Decompressive shunt surgery should be con- is not catheterizable and cavernomatous vein is not
sidered in cases with failed endotherapy, although it amenable to dilatation.
needs to be borne in mind that 37% of patients with
PVT also have thrombosis of splenic and superior mes-
Non shunt surgery. Oesophageal transection with or
enteric vein.112 It is also indicated for correcting
without splenectomy is less useful to control bleeding
symptomatic portal hypertensive biliopathy, symptom-
because of a high risk of late rebleeding and reap-
atic hypersplenism and ‘on demand’ one-time
pearance of varices, but can be resorted to as a non-
treatment.
shunt option in patients with portosystemic
Warren Zeppa distal splenorenal shunts have been
encephalopathy, hepatopulmonary syndrome or porto-
shown to be effective in control of bleeding and long-
pulmonary syndrome.124 Moreover, splenectomy
term survival in patients with PVT.113 Mesocaval
destroys the opportunity to use the splenic vein later
shunts may be necessary when Warren shunt is pre-
for a shunt. In MPD, spleen becomes an organ for
cluded. Our own study of side-to-side lienorenal
extramedullary marrow formation and should be
shunts demonstrated that it not only prevented rebleed
preserved.112
but also corrected the hypersplenism.114
Rex shunt (mesenteric left portal by pass) between
the superior mesenteric vein and left portal vein is Liver Transplant. Portal vein thrombosis was consid-
widely used now and has been considered more ered a major obstacle to liver transplantation which
physiological over other shunts, which do not return led to increased surgical complexity and perioperative
If shunt surgery is not possible for some reasons, a well tolerated in patients with portal vein thrombosis
biliary stent should be placed with periodic replace- without cavernous transformation.148, 149 Similarly,
ment.133 TIPS has also been performed successfully in conformal radiotherapy induced a 45.8% objective
treating PHB.121 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-
PVT in cirrhosis and HCC
come. Patients should be assessed preoperatively
Portal vein thrombosis in cirrhosis has been reported whether the thrombus is associated with tumour
to occur in 11.2% of the 701 cirrhotics studied. invasion or with stagnant flow. In a study of 12
Forty three percent of these were asymptomatic. Of consecutive patients who underwent liver transplan-
the 45(57%) symptomatic patients, 31 presented with tation for HCC in the setting of PVT, 42% had no
a variceal bleed, 14 with abdominal pain, 10 of evident portal vein invasion and only 17% had
whom had intestinal infarction. Most of these tumour thrombosis. Only one-third experienced
patients were in CTP class B & C. Mutation of pro- tumour recurrence in first year post-transplant and
thrombin gene was found to be the only thrombo- one-third became long-term survivors (median
philic condition associated with it.36 Another study 36 months) with no evidence of tumour recur-
on PVT in cirrhotics concluded that prothrombotic rence.150 Radiation therapy is considered the treat-
mutations by themselves are not causative of PVT, ment of choice for selected patients with HCC and
but sclerotherapy and previous abdominal surgery PVT especially for those with a favourable perfor-
favour development of PVT in two-thirds of cases, mance status.151
but is elusive in others.141
HCC is commonly associated with PVT. It is asso-
CONCLUSION
ciated with worse survival and indicates advanced
disease. Advanced stage, higher CTP class, low serum Portal vein thrombosis is being increasingly recog-
albumin and high AFP levels are predictive of PVT nized with or without underlying liver disease.
in patients with HCC.142 In a study on thrombophilic Patients should be investigated for thrombophilic con-
genetic factors in 94 patients with HCC with and ditions as a cause of PVT. Imaging picks up cases of
without PVT, the Odds Ratio was 3.85 for MTHFR PVT and helps in distinguishing patients with acute
C6777TT with HCC vs. healthy controls. Prothrombin PVT from chronic PVT as is evidenced by formation of
gene G 20210A mutation was also more frequent portal cavernoma. Although real time sonography with
among HCC patients mainly with PVT thereby con- colour Doppler is adequate for diagnosis and charac-
cluding that all cirrhotics should be looked for terization, some patients may require contrast
thrombophilic genetic factors to individualize enhanced US or MR angiography. In symptomatic
patients at risk for PVT in HCC.143 Survival in PVT noncarvenomatous PVT, anticoagulation is recom-
and HCC has been shown to be better in those with mended with low molecular weight heparin and oral
normal AFP.144 anticoagulants. In patients with cavernomatous forma-
Contrast enhanced US (CEUS) has been found to tion of portal vein or chronic PVT, decompressive
be superior to colour doppler sonography, conven- shunt surgery, preferably Rex shunt, should be per-
tional US and CT in thrombus detection and charac- formed if endotherapy fails to prevent bleeding from
terization complicating hepatic malignancies.145 In a varices especially in children. Rarely, TIPS may be
study, CEUS detected thrombus in 100% and cor- attempted in expert hands and if all these measures
rectly characterized it in 98% of patients, while CT fail or patient has features of hepato pulmonary syn-
detected thrombus in 68% and correctly character- drome or porto pulmonary syndrome, liver transplan-
ized it in 68% of them, CEUS may thus be helpful tation may be considered.
in staging HCC.146
Patients with PVT and small HCC can be safely
ACKNOWLEDGEMENT
treated with RFA.147 Similarly, Yttrium -90 glass
microspheres (Theraspheres) appear to be safe and Declaration of personal and funding interests: None.
orthotopic liver transplantation: a case 48 Ganguly S, Sarin SK, Bhatia V, Lahoti D. 61 Condat B, Vilgram V, Asselak T, et al.
report. Int Surg 2003; 88: 184–7. The prevalence and spectrum of colonic Portal carvernoma associated cholangiop-
36 Amitrano L, Guardascione MA, Brancac- lesions in patients with cirrhotic and athy a clinical and MR cholangiography
cio V, et al. Risk factors and clinical pre- noncirrhotic portal hypertension. Hepa- coupled with MR portography imaging
sentation of portal vein thrombosis in tology 1995; 21: 1226–31. study. Hepatology 2003; 37: 1302–8.
patients with liver cirrhosis. J Hepatol 49 Chawla Y, Dilawari JB. Anorectal varices 62 Malkhan GH, Bhatia ST, Bastin K, et al.
2004; 40: 736–41. their frequency in cirrhotic and noncirrh- cholangiography associated with portal
37 Belli L, Romani F, Sansalone CV, et al. otic portal hypertension. Gut 1991; 32: hypertension diagnostic evaluation and
Portal thrombosis in cirrhotics. A retro- 309. clinical implications. Gastroint Endos
spective analysis. Ann Surg 1986; 203: 50 Chawla Y, Dilawari JB. Gall Bladder vari- 1999; 49: 344–8.
286–91. ces in portal vein thrombosis. AJR 1994; 63 Sezgin O, Oguz D, Attritas E, Saritas U,
38 Murad SD, Valla DC, de Groen PC, et al. 162: 643–5. Sahin B. Endoscopic management of bili-
Pathogenesis and treatment of Budd- 51 Sarin SK, Bansal A, Sasan S, Nigam A. ary obstruction carried by cavernous
Chiari syndrome combined with portal Portal vein obstruction in children leads transformation of portal vein. Gastroint
vein thrombosis. Am J Gastroenterol to growth retardation. Hepatology 1992; Endos 2003; 58: 602–8.
2006; 101: 83–90. 15: 229–33. 64 Kobayashi S, Ng CS, Kazama T, et al.
39 Anand AC, Sashindran VK, Mohan L. Gas- 52 de Ville de Goyet J, Alberti D, Falchetti Hemodynamic and morphological
trointestinal problems at high altitude. D, et al. Treatment of extrahepatic portal changes after portal vein embolisation:
Trop Gastroenterol 2006; 27: 147–53. hypertension in children by mesenteric- different efforts central and peripheral
40 Dörffel T, Wruck T, Rückert RI, et al. to-left portal vein bypass: a new physio- zones in the liver on multiphasic com-
Vascular complications in acute pancrea- logical procedure. Eur J Surg 1999; 165: puted tomography. J Comput Assist
titis assessed by color duplex ultrasonog- 777–81. Tomog 2004; 28: 804–10.
raphy. Pancreas 2000; 21: 126–33. 53 Bellomo – Brandao MA, Morcillo AM, 65 Vilgrain V, Condat B, Bureau C, et al.
41 Yadav S, Dutta AK, Sarin SK. Do umbili- Hessel G, et al. Growth assessment in Atrophy-hypertrophy complex in patients
cal vein catheterization and sepsis lead to children with extrahepatic portal vein with cavernous transformation of the
portal vein thrombosis? A prospective, obstruction and portal hypertension. portal vein: CT evaluation. Radiology
clinical, and sonographic evaluation. Ar Gastro 2003; 40: 247–50. 2006; 241: 149–55.
J Pediatr Gastroenterol Nutr 1993; 17: 54 Ranjan M, Gupta R, Jain M, Malhotra V, 66 Bilodeau M, Aubry MC, Houle R, et al.
392–6. Sarin SK. Hepatic dysfunction in Evaluation of hepatocytes injury follow-
42 Sakha SH, Rafeey M, Tarzamani MK. Por- patients with extrahepatic portal ing partial ligation of the left portal vein.
tal venous thrombosis after umbilical venous obstruction. Liver Int 2003; 23: J Hepatol 1999; 30: 29–37.
vein catheterization. Indian J Gastro- 434–9. 67 Van Gansbeke D, Avni EF, Delcour C,
enterol 2007; 26: 283–4. 55 Condat B, Pessine T, Helene DM, Hillaire et al. Sonographic features of portal vein
43 Giraudo G, Greget M, Oussoultzoglou E, S, Valla D. Recent portal or mesentric thrombosis. AJR Am J Roentgenol 1985;
et al. Preoperative contralateral portal venous thrombosis, increased recognition 144: 749–52.
vein embolisation before major hepatic and frequent recanalisatioon on antico- 68 Wang JT, Zhao HY, Liu YL. Portal vein
resection is a safe and efficient proce- agulant therapy. Hepatology 2000; 32: thrombosis. Hepatobiliary Pancreat Dis
dure: a large single institution experi- 466–70. Int 2005; 4: 515–8.
ence. Surgery 2008; 143: 476–82. 56 Minguer B, Farua Papan JC, Bosch J, 69 Tessler FN, Gehring BJ, Gomes AS, et al.
44 Henderson JM, Gilmore GT, Mackay GJ, et al. Non cirrhotic portal vein thrombo- Diagnosis of portal vein thrombosis:
et al. Hemodynamics during liver trans- sis exhibits neuropsychological & MR value of color Doppler imaging. AJR Am
plantation: the interactions between car- changes consistent with minimal hepatic J Roentgenol 1991; 157: 293–6.
diac output and portal venous and encephalopathy. Hepatology 2006; 43: 70 Parvey HR, Eisenberg RL, Giyanani V,
hepatic arterial flows. Hepatology 1992; 707–14. et al. Duplex sonography of the portal
16: 715–8. 57 Gsoenervey M, Quero JC, DeBraign I, venous system: pitfalls and limitations.
45 Ohnishi K, Okuda K, Ohtsuki T, et al. For- et al. Subclinical hepatic encephalopathy AJR Am J Roentgenol 1989; 152: 765–
mation of hilar collaterals or cavernous impairs daily functioning. Hepatology 70.
transformation after portal vein obstruc- 1998; 28: 45–9. 71 Lai L, Brugge WR. Endoscopic ultrasound
tion by hepatocellular carcinoma. Obser- 58 Dilawari JB, Chawla Y. Pseudoscleoring is a sensitive and specific test to
vations in ten patients. Gastroenterology cholangitis in extrahepatic portal diagnose portal venous system thrombo-
1984; 87: 1150–3. venous obstruction. Gut 1992; 33: 272– sis (PVST). Am J Gastroenterol 2004; 99:
46 De Gaetano AM, Lafortune M, Patriquin 6. 40–4.
H, et al. Cavernous transformation of the 59 Khuroo MS, Yattoo GN, Zasyar SA, et al. 72 Palazzo l, Hochain P, Helmer C, et al.
portal vein: patterns of intrahepatic and Biliary abnormalities associated with Biliary varieces on endoscopic ultra-
splanchnic collateral circulation detected extrahepatic portal venous obstruction. sonography: clinical presentation ad
with Doppler sonography. AJR Am J Hepatology 1993; 17: 807–13. outcome. Endoscopy 2000; 32: 520–4.
Roentgenol 1995; 165: 1151–5. 60 Dhiman RK, Puri D, Chawla Y, et al. Bili- 73 Umpheren JL, Pecha RE, Urayama S.
47 Malkowski P, Pawlak J, Michalowicz B, ary changes in extrahepatic portal venous Biliary stricture caused by portal bilo-
et al. Thrombolytic treatment of portal obstruction compression by collaterals or pathy. Diagnosis by EUS with Doppler
thrombosis. Hepatogastroenterology 2003; ischemia? Gastrointest Endosc 1999; 50: US. Gasterintest Endoscopy 2004; 60:
50: 2098–100. 646–52. 1021–4.
74 Parvey HR, Raval B, Sandler CM. Portal 86 Kumar S, Sarr MG, Kamath PS. Mesen- 99 Hollingshead M, Burke CT, Mauro MA,
vein thrombosis: imaging findings. AJR teric venous thrombosis. N Engl J Med Weetis SM, Dixon RG, Jaugus PF. Trans-
Am J Roentgenol 1994; 162: 77–81. 2001; 345: 1683–8. catheter thrombolytic therapy for acute
75 Lee HK, Park SJ, Yi BH, et al. Portal vein 87 Baril N, Wren S, Radin R, Ralla P, Stain S. mesenteric and portal vein thrombosis.
thrombosis: CT features. Abdom Imaging The role of anticoagulation in pylephlebi- J Vasc Inter Radiol 2005; 16: 651–61.
2008; 33: 72–9. tis. Am J Surg 1996; 172: 449–52. 100 Kercher KW, Sing RF, Watson KW, et al.
76 Shah TU, Semelka RC, Voultsinos V, et al. 88 Hidajat N, Stobbe H, Griesshaber V, Felix Transhepatic thrombolysis in acute portal
Accuracy of magnetic resonance imaging R, Schroder RJ. Imaging and radiological vein thrombosis after laproscopic sple-
for preoperative detection of portal vein interventions of portal vein thrombosis. nectomy. Sung Laproso Endo Percutan
thrombosis in liver transplant candidates. Acta Radiol 2005; 46: 336–43. Tech 2002; 12: 131–6.
Liver Transpl 2006; 12: 1682–8. 89 Webster GJ, Burroughs AK, Riordan SM. 101 Henao EA, Bohamon WT, Silva MB Jr.
77 Cakmak O, Elmas N, Tamsel S, et al. Role Review article: portal vein thrombosis – Treatment of portal vein thrombosis with
of contrast-enhanced 3D magnetic reso- new insights into aetiology and manage- relative superior mesenteric artery
nance portography in evaluating portal ment. Aliment Pharmacol Ther 2005; 21: infusion of recombinant tissue plasmino-
venous system compared with color 1–9. gen activator. J Vase Surg 2003; 38:
Doppler ultrasonography. Abdom Imaging 90 Merkel C, Bolognesi M, Bellon S, et al. Long 1411–5.
2008; 33: 65–71. term follow up study of adult patients with 102 Tateishi A, Mitsui M, Oki T, et al. Exten-
78 Smith CS, Sheehy N, McEniff N, et al. noncirrhotis obstruction of the portal sys- sive mesenteric vein and portal vein
Magnetic resonance portal venography: tem comparison with cirrhotic patients. thrombosis significantly treated by thro-
use of fast-acquisition true FISP imag- J Hepatol 1992; 15: 299–303. moltis and anticoagulation. J Gastroen-
ing in the detection of portal vein 91 Chawla YK, Dilawari JB. Portographic terol Hepatol 2001; 16: 1429–33.
thrombosis. Clin Radiol 2007; 62: changes with time in patients with extra- 103 Uflacher R. Ajpheat of percutaneous
1180–8. hepatic portal venous obstruction. J Gas- mechanical thromboectomy in TIPS and
79 Lin J, Zhou KR, Chen ZW, et al. Three- troenterol Hepatol 1988; 3: 421–4. PVT Tech. Vas Inter Radiol 2003; 6: 59–
dimensional contrast-enhanced MR angi- 92 Amitrano L, Guardascione MA, Scaglione 69.
ography in diagnosis of portal vein M, et al. A Prognostic factors in non- 104 Oğuzkurt P, Tercan F, Ince E, Ezer SS,
involvement by hepatic tumors. World J cirrhotic patients with splanchnic vein Hiçsönmez A. Percutaneous treatment of
Gastroenterol 2003; 9: 1114–8. thromboses. Am J Gastroenterol 2007; portal vein thrombosis in a child who
80 Glockner JF, Forauer AR, Solomon H, 102: 2464–70. has undergone splenectomy. J Pediatr
et al. Three-dimensional gadolinium- 93 Garcia Pagan JC, Guerra MH, Bosch J. Surg 2008; 43: e29–32.
enhanced MR angiography of vascular Extrahepatic portal vein thrombosis. 105 Ferro C, Rossi CG, Bovio G, Dahamane
complications after liver transplantation. Seminars in Liver diseases 2008; 28: M, Centenaro M. TIPS mechanical aspira-
AJR Am J Roentgenol 2000; 174: 1447– 282–92. tion thembectory and directt thromboly-
53. 94 Dolorich LR, Ginsberg JS, Douketis JD, sis in the treatment of acute portal and
81 Vlachogiannakos J, Patch D, Watkinson Holbrook AM, Chealig A. metaanalysis superior mesenteric vein thrombosis. Car-
A, et al. Carbon-dioxide portography: an comparing low molecular wt heparin diovas Intervent Radiol 2007; 30: 1070–
expanding role? Lancet 2000; 355: 987– with unfractionated heparin in treatment 4.
8. of venous thrombolism examining 106 Adani GL, Baccarani U, Risaliti A, et al.
82 Kreft B, Strunk H, Flacke S, et al. Detec- some unanswered questions regarding Percutaneous transhepatic portography
tion of thrombosis in the portal venous location of treatment product use and for the treatment of early portal vein
system: comparison of contrast-enhanced dosing frequency. Arch Int Med 2000; thrombosis after surgery. Cardiovasc In-
MR angiography with intraarterial digital 160: 181–8. tervent Radiol 2007; 30: 1222–6.
subtraction angiography. Radiology 2000; 95 Francoz C, Belghiti J, Vilgrain V, et al. 107 Brearley S, Hawker PC, Dykes PW,
216: 86–92. Splanchnic vein thrombosis in candidates Keighley MR. A lethal complication of
83 Sun L, Guan YS, Pan WM, et al. Highly for liver transplantation: usefulness of peripheral vein vasopressin infusion.
metabolic thrombus of the portal vein: screening and anticoagulation. Gut 2005; Hepatogastroenterology 1985; 32: 224–5.
18F fluorodeoxyglucose positron emis- 54: 691–7. 108 Celińska-Cedro D, Teisseyre M, Woyna-
sion tomography ⁄ computer tomography 96 Louvet A, Texier F, Dharancy S, et al. rowski M, et al. Endoscopic ligation of
demonstration and clinical significance Anticoagulation therapy may sense bili- esophageal varices for prophylaxis of
in hepatocellular carcinoma. World J ary abnormalities due to acute portal first bleeding in children and adolescents
Gastroenterol 2008; 14: 1212–7. thrombosis. DDS 2006; 2006: 5111–7. with portal hypertension: preliminary
84 Dilawari JB, Chawla YK, Raju GS, Kaur 97 Lopeia JE, Correa G, Brazzini A, et al. results of a prospective study. J Pediatr
U, Bambery P. Splenoportovenography in Percutaneous, transhepatic treatment of Surg 2003; 38: 1008–11.
portal hypertension: a safe outpatient symptomatic mesenteric venous thrombo- 109 Spaander MC, Murad SD, van Buuren HR,
procedure. Can Assoc Radiol J 1990; 41: sis. J Vasc Surg 2002; 36: 1058–67. et al. Endoscopic treatment of esophag-
146–8. 98 Aytekin C, Boyvat F, Kurt A, Yologhuz ogastric variceal bleeding in patients
85 Jamieson NV. Changing perspectives in coskun M. Catheter directed thrombolysis with noncirrhotic extrahepatic portal
portal vein thrombosis and liver trans- with transjugular access in portal vein vein thrombosis: a long-term follow-up
plantation. Transplantation 2000; 69: thrombosis secondary to pancreatitis. Eur study. Gastrointest Endosc 2008; 67:
1772–4. J Radiol 2001; 39: 80–2. 821–7.
110 Poddar U, Thapa BR, Singh K. Band liga- 122 Bilbao JI, Elorz M, Vivas I, et al. Transju- 135 Thervet L, Faulques B, Pissas A, et al.
tion plus sclerotherapy versussclerothera- gular intrahepatic portosystemic shunt Endoscopic management of obstructive
py alone in children with extrahepatic (TIPS) in the treatment of venous symp- jaundice due to portal cavernoma. Endos-
portal venous obstruction. J Clin Gastro- tomatic chronic portal thrombosis in copy 1993; 25: 423–5.
enterol 2005; 39: 626–9. non-cirrhotic patients. Cardiovasc Inter- 136 Bhatia V, Jain AK, Sarin SK. Choledocho-
111 Dilawari JB, Raju GS, Chawla YK. vent Radiol 2004; 27: 474–80. lithiasis associated with portal biliopathy
Development of large spleno-adreno- 123 Van Ha TG, Hodge J, Funaki B, et al. in patients with extrahepatic portal vein
renal shunt after endoscopic sclero- Transjugular intrahepatic portosystemic obstruction: management with endo-
therapy. Gastroenterology 1989; 97: shunt placement in patients with cirrhosis scopic sphincterotomy. Gastrointest En-
421–6. and concomitant portal vein thrombosis. dosc 1995; 42: 178–81.
112 Wolff M, Hirner A. Current state of por- Cardiovasc Intervent Radiol 2006; 29: 137 Tighe M, Jacobson I. Bleeding from bile
tosystemic shunt surgery. Langenbecks 785–90. duct varices: an unexpected hazard dur-
Arch Surg 2003; 388: 141–9. 124 Superina R, Shneider B, Emre S, et al. ing therapeutic ERCP. Gastrointest En-
113 Livingstone AS, Koniaris LG, Perez EA, Surgical guidelines for the management dosc 1996; 43: 250–2.
et al. 507 Warren-Zeppa distal splenore- of extra-hepatic portal vein obstruction. 138 Mutignani M, Shah SK, Bruni A, et al.
nal shunts: a 34-year experience. Ann Pediatr Transplant 2006; 10: 908–13. Endoscopic treatment of extrahepatic bile
Surg 2006; 243: 884–92. 125 Shaw BW Jr, Iwatsuki S, Bron K, et al. duct strictures in patients with portal
114 Sharma BC, Singh RP, Chawla YK, et al. Portal vein grafts in hepatic transplanta- biliopathy carries a high risk of haemo-
Effect of shunt surgery on spleen size, tion. Surg Gynecol Obstet 1985; 161: 66– bilia: report of 3 cases. Dig Liver Dis
portal pressure and oesophageal varices 8. 2002; 34: 587–91.
in patients with non-cirrhotic portal 126 Lladó L, Fabregat J, Castellote J, et al. 139 Chaudhary A, Dhar P, Sarin SK, et al.
hypertension. J Gastroenterol Hepatol Management of portal vein thrombosis in Bile duct obstruction due to portal biliop-
1997; 12: 582–4. liver transplantation: influence on mor- athy in extrahepatic portal hypertension:
115 Dasgupta R, Roberts E, Superina RA, Kim bidity and mortality. Clin Transplant surgical management. Br J Surg 1998;
PC. Effectiveness of Rex shunt in the 2007; 2: 716–21. 85: 326–9.
treatment of portal hypertension. J Pedi- 127 Lendoire J, Raffin G, Cejas N, et al. Liver 140 Vibert E, Azoulay D, Aloia T, et al.
atr Surg 2006; 41: 108–12. transplantation in adult patients with Therapeutic strategies in symptomatic
116 Query JA, Sandler AD, Sharp WJ. Use of portal vein thrombosis: risk factors, man- portal biliopathy. Ann Surg 2007; 246:
autogenous saphenous vein as a agement and outcome. HPB (Oxford) 97–104.
conduit for mesenterico-left portal 2007; 9: 352–6. 141 Mangia A, Villani MR, Cappucci G, et al.
vein bypass. J Pediatr Surg 2007; 42: 128 Harmanci O, Bayraktar Y. Portal hyper- Causes of portal venous thrombosis in
1137–40. tension due to portal venous thrombosis: cirrhotic patients: the role of genetic and
117 Krebs-Schmitt D, Briem-Richter A, Grab- etiology, clinical outcomes. World J Gas- acquired factors. Eur J Gastroenterol
horn E, et al. Effectiveness of Rex shunt troenterol 2007; 13: 2535–40. Hepatol 2005; 17: 745–51.
in children with portal hypertension 129 Yerdel MA, Gunson B, Mirza D, et al. 142 Connolly GC, Chen R, Hyrien O, et al.
following liver transplantation or with Portal vein thrombosis in adults undergo- Incidence, risk factors and consequences
primary portal hypertension. Pediatr ing liver transplantation : risk factors, of portal vein and systemic thrombosesin
Transplant 2009; 13: 540–4. screening, management and outcome. hepatocellular carcinoma. Thromb Res
118 Kim HB, Pomposelli JJ, Lillehei CW, et al. Transplantation 2000; 69: 1873–81. 2008; 122: 299–306.
Mesogonadal shunts for extrahepatic 130 Tao YF, Teng F, Wang ZX, et al. Liver 143 D’Amico M, Pasta L, Sammarco P.
portal vein thrombosis and variceal transplant recipients with portal vein MTHFR C677TT, PAI1 4G-4G, V Leiden
hemorrhage. Liver Transpl 2005; 11: thrombosis: a single center retrospective Q506, and prothrombin G20210A in
1389–94. study. Hepatobiliary Pancreat Dis Int hepatocellular carcinoma with and with-
119 Warren WD, Henderson JM, Millikan WJ, 2009; 8: 34–9. out portal vein thrombosis. J Thromb
et al. Management of variceal bleeding 131 Doenecke A, Tsui TY, Zuelke C, et al. Thrombolysis 2008; 28: 70–3.
in patients with noncirrhotic portal Pre-existent portal vein thrombosis in 144 Carr BI, Buch SC, Kondragunta V, Panc-
vein thrombosis. Ann Surg 1988; 207: liver transplantation: influence of pre- oska P, Branch RA. Tumor and liver
623–34. operative disease severity. Clin Trans- determinants of prognosis in unresectable
120 Orloff MJ, Orloff MS, Girard B, Orloff SL. plant 2009; 19, Feb 19, PubMed. hepatocellular carcinoma: a case cohort
Bleeding esophagogastric varices from 132 Hajdu CH, Murakami T, Diflo T, et al. Int- study. J Gastroenterol Hepatol 2008; 23:
extrahepatic portal hypertension: rahepatic portal cavernoma as an indica- 1259–66.
40 years’ experience with portal-systemic tion for liver transplantation. Liver 145 Rossi S, Rosa L, Ravetta V, et al. Con-
shunt. J Am Coll Surg 2002; 194: 717– Transpl 2007; 13: 1312–6. trast-enhanced versus conventional and
28. 133 Dhiman RK, Behera A, Chawla YK, et al. color Doppler sonography for the detec-
121 Senzolo M, Tibbals J, Cholongitas E, Portal hypertensive biliopathy. Gut 2007; tion of thrombosis of the portal and
et al. Transjugular intrahepatic portosys- 56: 1001–8. hepatic venous systems. AJR Am J
temic shunt for portal vein thrombosis 134 Gauthier-Villars M, Franchi S, Gauthier Roentgenol 2006; 186: 763–73.
with and without cavernous transforma- F, et al. Cholestasis in children with por- 146 Rossi S, Ghittoni G, Ravetta V, et al. Con-
tion. Aliment Pharmacol Ther 2006; 23: tal vein obstruction. J Pediatr 2005; 146: trast-enhanced ultrasonography and
767–75. 568–73. spiral computed tomography in the
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