REVIEW

Portal Vein Thrombosis

Sameer Parikh, MD,a Riddhi Shah, MD,a Prashant Kapoor, MDb
a
Department of Medicine, Baylor College of Medicine, Houston, Tex, bDepartment of Internal Medicine, Division of Hematology, Mayo
Clinic, Rochester, Minn.

ABSTRACT

Portal vein thrombosis is a condition not infrequently encountered by clinicians. It results from a
combination of local and systemic prothrombotic risk factors. The presentation of acute thrombosis varies
widely from an asymptomatic state to presence of life-threatening intestinal ischemia and infarction. In the
chronic stage, patients typically present with variceal bleeding or other complications of portal hyperten-
sion. Abdominal ultrasound color Doppler imaging has a 98% negative predictive value, and is considered
the imaging modality of choice in diagnosing portal vein thrombosis. Controlled clinical trials to assist with
clinical decision-making are lacking in both acute and chronic portal vein thrombosis. Oral anticoagulant
therapy is initiated if the risks of bleeding are low, but long-term anticoagulation is generally not
recommended in patients with concomitant hepatic cirrhosis. The roles of invasive therapeutic approaches
such as thrombolysis and transjugular intrahepatic portosystemic shunt continue to evolve. This review
conflates dissenting views into a rational approach of managing patients with portal vein thrombosis for the
general internist.
© 2010 Elsevier Inc. All rights reserved. • The American Journal of Medicine (2010) 123, 111-119

KEYWORDS: Anticoagulation; Cirrhosis; Coagulopathy; Myeloproliferative diseases

Portal vein thrombosis is a condition not infrequently en- the portal vein divides into right and left branches that
countered by internists. In acute portal vein thrombosis, continue to their respective hepatic lobes, ultimately emp-
patients may be asymptomatic or present with life-threaten- tying into hepatic sinusoids. Blood returns to the inferior
ing intestinal ischemia and infarction. In the chronic stage, vena cava via the hepatic veins (Figure 1). Portal vein
patients generally present with complications related to por- thrombosis is defined as a condition resulting from forma-
tal hypertension, such as variceal bleeding and hyper- tion of a blood clot in the extrahepatic portion of the portal
splenism. This review focuses on the etiopathogenesis, di- vein. Obstruction of the portal vein by a tumor is not
agnostic approach, and current management strategies for included in this definition, and its review is outside the
portal vein thrombosis. scope of this article.

ANATOMY INCIDENCE AND ETIOLOGY

The portal vein accounts for 75% of the blood supply to the Although both local and systemic prothrombotic factors are
liver. It is an 8-cm, valveless conduit originating from the implicated in causing portal vein thrombosis, cirrhosis of
confluence of the superior mesenteric and splenic veins the liver is considered an important predisposing condition.
posterior to the neck of the pancreas. In the porta hepatis, In patients with well-compensated cirrhosis, the incidence
of thrombosis reportedly varies between 0.6% and 16%.1,2
Funding: None. The incidence is higher in patients with advanced hepatic
Conflict of Interest: The authors have no conflicts of interest to dysfunction, particularly those with hepatocellular cancer,
declare relevant to this article. in whom it reaches approximately 35%. Its incidence in
Authorship: All authors had access to the data and played a role in patients without cirrhosis is not well described in the liter-
writing the manuscript. ature, but accounts for approximately 5%-10% of all cases
Requests for reprints should be addressed to Sameer Parikh, MD,
Department of Medicine, Baylor College of Medicine, One Baylor Plaza, of portal hypertension in the Western Hemisphere.3 In the
BCM Mailstop: 285, Houston, TX 77030. developing world, it may account for up to a third of
E-mail address: parikh@bcm.tmc.edu patients with portal hypertension, especially children, given

0002-9343/$ -see front matter © 2010 Elsevier Inc. All rights reserved.
doi:10.1016/j.amjmed.2009.05.023
112 The American Journal of Medicine, Vol 123, No 2, February 2010

the higher number of infectious complications that lead to age group.7 In adults, acute appendicitis, cholecystitis, acute
4
portal vein thrombosis. Notably, no sex preponderance is necrotizing pancreatitis, cholangitis, diverticulitis, and per-
observed. forated peptic ulcers all have been reported to cause portal
Although the mechanisms by which cirrhosis leads to vein thrombosis.8,9 Interestingly, in patients with bactere-
portal vein thrombosis are not entirely clear, decreased mia from bacteroides fragilis of an unknown source, there
portal blood flow and a decrease is a higher incidence of portal vein
in the level of natural anticoagu- thrombosis, presumably due to the
lants, including proteins C and S transient appearance of prothrom-
CLINICAL SIGNIFICANCE
and antithrombin III, have been botic anticardiolipin antibodies.10
implicated. The role of inherited ● Portal vein thrombosis results from a Inflammatory conditions such as
thrombophilia (such as Factor V combination of local and systemic pro- acute pancreatitis account for about
Leiden mutation and prothrombin thrombotic factors. 3%-5% of all cases of portal vein
20210 gene mutation) in the patho- thrombosis.7 Blunt abdominal trauma
genesis remains uncertain. Whereas ● New insights into its association with and surgical procedures, including
one study reported that up to 70% acquired prothrombotic conditions such open or laparoscopic cholecystec-
of all patients with portal vein as myeloproliferative disorders have tomy, splenectomy, gastric bypass
thrombosis and cirrhosis have an made it important for physicians to or- surgery, and colectomy could af-
underlying inherited hypercoagu- der necessary tests and follow patients fect the integrity of the portal ve-
lable state,5 another failed to con- carefully. nous system, thereby precipitating
firm this association.6 Thus, the portal vein thrombosis.11,12 As a
need to detect an underlying hyper- ● Long-term anticoagulant therapy is ini- result of inherent hypercoagulabil-
coagulable state in patients with cir- tiated if the risks of bleeding are low ity or direct extension of the tumor
rhosis is controversial, and based in patients without cirrhosis, but it is thrombus, intra-abdominal malig-
on the currently available scant generally not recommended in patients nancies such as hepatocellular
data, further investigations are with concomitant cirrhosis. cancer, gastric and pancreatic ad-
generally not recommended in enocarcinomas, and cholangiocar-
those with advanced cirrhosis, cinoma may lead to portal vein
who, by virtue of increased portal thrombosis.
pressure, reduced portal flow, and other local factors, are Systemic etiological factors encompass both congenital
already predisposed to excess thrombus formation. and acquired thrombophilic states. Pregnancy and oral con-
Infectious, inflammatory, and malignant conditions of traceptive use are acquired conditions associated with portal
the abdomen are the most commonly implicated local risk vein thrombosis. Increasingly, genetic polymorphisms such
factors for development of portal vein thrombosis (Figure 2). as Factor V Leiden and prothrombin 20210 gene mutation are
Umbilical sepsis triggered by omphalitis and umbilical vein being recognized as thrombogenic conditions associated with
cannulation is an important etiological factor in the neonatal splanchnic venous system clots. Acquired prothrombotic con-
ditions, especially myeloproliferative disorders (essential
thrombocythemia, polycythemia vera, and myelofibrosis
with myeloid metaplasia) might account for a large propor-
tion of cases previously diagnosed as “idiopathic” portal
vein thrombosis.13 The clinical features of an underlying
myeloproliferative disorder may become indiscernible if
they are masked by the concomitant presence of portal
hypertension-related hypersplenism and iron deficiency
anemia (from variceal bleeding). A recently described ac-
quired gain-of-function mutation in the gene encoding the
tyrosine-protein kinase, Janus Kinase 2, has been identified
as a specific marker for myeloproliferative diseases.14 This
mutation, easily detected with DNA sequencing or by poly-
merase chain reaction technique, has replaced bone marrow
biopsy as the initial diagnostic test for investigating patients
with portal vein thrombosis for an underlying myeloprolif-
erative disorder.15 Indeed, a number of studies have identi-
fied this mutation in up to 30% of patients with portal vein
thrombosis without overt clinical manifestations of myelo-
proliferative disorders and should prompt a referral to a
Figure 1 Normal anatomy of the portal vein and its tributaries. hematologist upon diagnosis.16 It is important to recognize
that the incidence of Janus Kinase 2 mutation is approxi-
Parikh et al Portal Vein Thrombosis 113

Figure 2 Etiology of portal vein thrombosis.

mately 95% in patients with polycythemia vera but is only vein thrombosis, refers to the development of biliary symp-
50% in patients with essential thrombocythemia and pri- toms as a result of the enlarged collateral veins on the
mary myelofibrosis. surface of the common bile duct resulting in either partial
or, rarely, complete bile duct obstruction. Patients might be
asymptomatic or present with jaundice, cholangitis, and
CLINICAL PRESENTATION abdominal pain.19
Acute Portal Vein Thrombosis
Portal vein thrombosis is considered acute when symptoms
develop ⬍60 days before presentation, and there is no
clinical, radiological, or endoscopic evidence of portal hy-
pertension or collateral circulation. Acute portal vein throm-
bosis commonly manifests as abdominal pain, fever, and
nausea. However, a number of patients might possibly be
asymptomatic or have nonspecific symptoms. In patients
with thrombus extending to the mesenteric veins, fatal con-
sequences including mesenteric ischemia, infarction, and
peritonitis with overwhelming sepsis can rapidly ensue.17

Chronic Portal Vein Thrombosis

Chronic portal vein thrombosis involves formation of nu-
merous hepatopetal collateral veins around the thrombosed
portal vein that allow blood flow from the patent proximal
portion of the portal vein to the distal portion—the so-called
“portal cavernoma” (Figure 3). Most patients at this stage
present with signs and symptoms of portal hypertension,
Figure 3 Pictorial depiction of hepatopetal collaterals around
including variceal bleeding, ascites, and hypersplenism.18 the thrombosed portal vein, the so-called “portal cavernoma.”
Portal cholangiopathy, a rare complication of chronic portal
114 The American Journal of Medicine, Vol 123, No 2, February 2010

Figure 4 (a) Ultrasound of the abdomen showing the liver and a normal patent portal vein.
(b) Doppler ultrasound of the abdomen showing normal hepatopetal flow in the portal vein.

DIAGNOSIS into its tributaries. The direction of blood flow is easily

Liver function tests are typically normal in patients with determined by color Doppler ultrasonography22 (Figure 4).
portal vein thrombosis who do not have concomitant cir- The obstruction in the portal vein may be partial or com-
rhosis. Routine blood tests such as complete blood count plete (Figure 5). Ultrasound with color Doppler imaging has
may reveal an elevated white blood cell count consistent a 98% negative predictive value and is the imaging modality
with an infection. Polycythemia or thrombocytosis should of choice in diagnosing portal vein thrombosis.17 Acute
prompt a review of the peripheral blood smear and a search portal vein thrombosis appears as a filling defect in the
for an underlying myeloproliferative disorder. For investi- contrast-enhanced lumen of the portal vein in CT studies
gation of hypercoagulable states, one should generally fol- (Figure 6). CT scan of the abdomen also might concurrently
low the guidelines established for diagnosing idiopathic detect malignant growths causing portal vein thrombosis.
unprovoked venous thromboembolism.20 The diagnosis of chronic portal vein thrombosis is estab-
For establishing the diagnosis, newer noninvasive mo- lished by demonstration of a portal cavernoma, which ap-
dalities such as ultrasonography with Doppler imaging, pears as a network of veins around porta hepatis (Figures 7, 8).
computed tomography (CT), and magnetic resonance (MR) MR studies of the abdomen also are extremely sensitive in
of the abdomen have supplanted the traditional invasive demonstrating occlusion of portal vein.21
tests of portal venography and superior mesenteric arteriog- In some patients with portal cavernomas, the dilated
raphy.21 Ultrasound examination demonstrates a hyper- veins may mimic cholangiocarcinoma or a pancreatic head
echoic material within the portal vein, at times extending mass. Endoscopic ultrasound and MR cholangiography ac-

Figure 5 (a) Doppler ultrasound of the abdomen demonstrating partial thrombosis of the
portal vein. (b) Doppler ultrasound of the abdomen demonstrating complete thrombosis of
the portal vein.
Parikh et al Portal Vein Thrombosis 115

Figure 6 (a) and (b) Computed tomography scan with contrast of the abdomen showing
a filling defect in the portal vein consistent with an acute portal vein thrombosis.

curately distinguish these from portal vein thrombosis.23 anticoagulation. However, data need to be confirmed pro-
Angiography, traditionally the definitive investigation in spectively before making treatment recommendations.
portal vein thrombosis, is generally reserved for preopera-
tive assessment if surgical intervention is planned. Acute Portal Vein Thrombosis in Patients
without Concomitant Cirrhosis
MANAGEMENT The goals of therapy for acute portal vein thrombosis are
2-fold: to establish complete patency of the portal vein,
Acute and Chronic Portal Vein Thrombosis in thereby preventing development of chronic portal vein
Patients with Cirrhosis thrombosis, and to reduce the risk of thrombus progres-
Determination of underlying cirrhosis of the liver should be sion into the mesenteric veins and prevent mesenteric
the foremost step in a patient with established portal vein ischemia and infarction. Randomized controlled studies
thrombosis (Figure 9). There is currently no evidence to on efficacy of most forms of therapy for portal vein
support the use of chronic anticoagulant therapy in either thrombosis are lacking. The use of unfractionated heparin
symptomatic or asymptomatic acute or chronic portal vein or, preferably, low-molecular-weight heparins such as
thrombosis patients with cirrhosis. Although the risk of enoxaparin or dalteparin, with subsequent transition to
bleeding due to reduced synthesis of coagulation factors and oral warfarin, is the most common approach to anticoag-
presence of varices is substantial, the benefits of recanaliz- ulation. The question of intensity of anticoagulation on
ing portal vein in asymptomatic cirrhotics with portal vein warfarin has not been addressed in randomized trials;
thrombosis are unknown.24 There might be a highly select although by extrapolation from data on venous thrombo-
group of patients with cirrhosis and very early thrombosis embolism, a target international normalized ratio of 2-3 is
with insignificant portal hypertension that may benefit from reasonable. The optimal duration of anticoagulation is

Figure 7 (a) Ultrasound of the abdomen showing portal cavernoma. (b) Doppler ultra-
sound of the abdomen showing numerous collaterals, the portal cavernoma.
116 The American Journal of Medicine, Vol 123, No 2, February 2010

Figure 8 (a) and (b) Computed tomography scan with contrast of the abdomen showing
numerous collaterals around the portal vein consistent with a portal cavernoma.

controversial and can range from 6 months to life-long Recanalization of the portal vein can be confirmed by
anticoagulation, depending on the etiology of portal vein Doppler ultrasound examination after 6 months of antico-
thrombosis. For most patients with a systemic etiology, agulation. Combined data from retrospective studies show
lifelong anticoagulation is generally recommended in the that the rate of recanalization after 6 months of anticoagu-
absence of contraindications.25 lation can vary from complete recanalization in 50% or

Figure 9 A proposed approach to the management of patients with portal vein thrombosis.
Parikh et al Portal Vein Thrombosis 117

partial recanalization in 40% to failure of recanalization in tive studies. The aims of management include: prevention
up to 10% of patients.26,27 In the single prospective study of and treatment of gastroesophageal variceal bleeding, pre-
105 patients with acute portal vein thrombosis, 44% of vention of recurrent thrombosis within the portal and the
patients achieved patency of portal venous flow, and mor- mesenteric veins, and analyzing the risks and benefits of
tality was 2% at 1-year follow-up.28 For patients with partial anticoagulation in a patient with thrombosis in the
or incomplete recanalization after 6 months of therapy, it is splanchnic venous system and bleeding from gastro-
unclear if prolonging anticoagulation would be of additional esophageal varices.
benefit. Spontaneous recanalization of the portal vein has Approximately 85%-90% of patients with chronic extra-
been reported in some patients, although it is difficult to hepatic portal venous obstruction have esophageal varices
identify these patients upon presentation. and about 30% have concomitant gastric varices.8 There are
Appropriate antibiotic therapy effective against Gram- no data on prevention of development of esophageal varices
negative and anaerobic bacteria should be administered ex- in patients with chronic portal vein thrombosis. In patients
peditiously to patients with infectious etiology. Although with medium- to large-sized varices as a consequence of
complete resolution of the thrombus on antibiotic therapy portal cavernoma, nonselective beta-blockers and endo-
alone has been reported, simultaneous anticoagulation is scopic therapies have reduced the incidence of both first and
recommended. In patients who develop mesenteric ischemia recurrent bleeding in a multivariate analysis in addition to
and intestinal infarction, emergent laparotomy with bowel prolonging survival.34,35 Indeed, in the absence of contra-
resection might be life-saving. However, the mortality as- indications, most patients with esophageal varices due to
sociated with this condition can exceed 70%. Appropriate chronic portal vein thrombosis should be on either pro-
anticoagulation also is warranted after successful surgical pranolol or nadolol, or undergo endoscopic variceal band
resection of the infarcted bowel.29 ligation.
The role of invasive therapies for acute portal vein A recently reported retrospective study of 27 adults
thrombosis is evolving, and evidence is mostly derived from showed that endoscopic band ligation of esophageal varices
small case series. Systemic or in situ thrombolysis, surgical due to chronic portal vein thrombosis was safe and effec-
thrombectomy, and placement of a transjugular intrahepatic tive. The rebleeding risk was 23% for the first year and 37%
portosystemic shunt (TIPS) have been reported to reduce at the end of 5 years.36 Until more evidence emerges, it is
the thrombotic burden in the portal vein.30 In a series of 20 reasonable to adhere to the same guidelines for treatment of
patients in whom transcatheter-directed thrombolysis of esophageal variceal bleeding in patients with chronic portal
portal vein was performed, only 3 patients had complete vein thrombosis as in patients with portal hypertension due
recanalization and 12 patients had partial recanalization. to hepatic cirrhosis. There is a theoretical concern of devel-
However, a major procedure-related complication occurred opment of portal vein thrombosis in patients receiving va-
in 60% of patients with procedure-related mortality of 5%.31 soconstrictors (terlipressin and octreotide) in the manage-
In another series of 33 patients, systemic thrombolysis with ment of acute variceal bleeding as a consequence of reduced
either streptokinase or recombinant tissue plasminogen ac- portal venous flow and stasis. Indeed, patients who develop
acute abdominal pain while receiving these infusions should
tivator led to complete resolution of blood flow in 10 pa-
be investigated for thrombosis in the splanchnic venous
tients. In the remaining 23 patients, only partial resolution
system with imaging studies.37
of thrombosis occurred.32 Surgical thrombectomy is gener-
Clinicians often face a dilemma in managing patients
ally indicated only if a patient undergoes laparotomy for
with chronic portal vein thrombosis without cirrhosis—
resection of the infracted bowel. In smaller series of pa-
should the patient receive anticoagulants for an extended
tients, restoration of portal venous blood flow with varying
period to reduce clot extension and prevent portal hyperten-
degrees of success has been achieved, either with TIPS
sion, or will anticoagulation exacerbate esophageal and gas-
alone or with combination of systemic and in situ throm-
tric variceal bleeding, leading to increased morbidity and
bolysis.33 Overall, percutaneous revascularization techniques
mortality? In the absence of clinically significant portal
appear promising and have been reported to be successful in
hypertension, that is, without evidence of ascites and varices
retrospective case series. Validation with special focus on
on endoscopic examination, it is reasonable to anticoagulate
procedural safety is required through well-conducted larger
patients. Unfortunately, no controlled trials exist to guide
randomized trials before routine recommendation in pa-
therapy in the remaining subset of patients that forms a large
tients with acute portal vein thrombosis. Irrespective of
majority. There have been a few retrospective analyses that
intervention with these invasive techniques, patients with a have shown that anticoagulation with warfarin is safe, re-
diagnosis of acute thrombosis require anticoagulation for at duces the risk of recurrent thrombosis, and might actually
least 3-6 months. improve survival.35,38 However, patient selection bias might
have tilted the balance in favor of anticoagulation. Until
Chronic Portal Vein Thrombosis in Patients more definitive studies are available, it seems rational to use
without Concomitant Cirrhosis anticoagulants in patients without any evidence of portal
As in acute portal vein thrombosis, evidence for manage- hypertension and those with documented hypercoagulable
ment-related issues is mostly available through retrospec- states. For patients who have undergone variceal band liga-
118 The American Journal of Medicine, Vol 123, No 2, February 2010

tion due to bleeding varices, an individual case-by-case 3. Valla DC, Condat B, Lebrec D. Spectrum of portal vein thrombosis in
decision for anticoagulation needs to be made. The role of the West. J Gastroenterol Hepatol. 2002;17(Suppl 3):S224-S227.
4. Sarin SK, Sollano JD, Chawla YK, et al. Consensus on extra-hepatic
shunt surgery in patients with chronic portal vein thrombo-
portal vein obstruction. Liver Int. 2006;26:512-519.
sis has not been studied adequately. There is no consensus 5. Amitrano L, Brancaccio V, Guardascione MA, et al. Inherited coag-
on the optimal timing and type of surgery. A number of ulation disorders in cirrhotic patients with portal vein thrombosis.
treatment options, including ursodeoxycholic acid, endo- Hepatology. 2000;31:345-348.
scopic retrograde cholangiopancreatography for retrieval of 6. Mangia A, Villani MR, Cappucci G, et al. Causes of portal venous
biliary stones, insertion of biliary stents to relieve obstruc- thrombosis in cirrhotic patients: the role of genetic and acquired
factors. Eur J Gastroenterol Hepatol. 2005;17:745-751.
tion, and TIPS have been tried with varying degrees of 7. Witte CL, Brewer ML, Witte MH, Pond GB. Protean manifestations of
success in portal cholangiopathy.39 pylethrombosis. A review of thirty-four patients. Ann Surg. 1985;202:
191-202.
8. Webb LJ, Sherlock S. The aetiology, presentation and natural history
PROGNOSIS of extra-hepatic portal venous obstruction. Q J Med. 1979;48:627-639.
The survival of patients with portal vein thrombosis and 9. Sheen CL, Lamparelli H, Milne A, et al. Clinical features, diagnosis
associated cirrhosis closely mimics those with cirrhosis and outcome of acute portal vein thrombosis. QJM. 2000;93:531-534.
alone, and is dependent on their Model for End-Stage Liver 10. Ni YH, Wang NC, Peng MY, et al. Bacteroides fragilis bacteremia
Disease score. Most noncirrhotic patients have a better out- associated with portal vein and superior mesentery vein thrombosis
secondary to antithrombin III and protein C deficiency: a case report.
come with survival dependent on the underlying etiological
J Microbiol Immun Infect. 2002;35:255-258.
factors.38 One-year survival varies from 80% to 95%, and 11. Abdelrazeq AS, Dwaik MA, Aldoori MI, et al. Laparoscopy-associ-
3-year survival from 75% to 90% in patients with chronic ated portal vein thrombosis: description of an evolving clinical syn-
portal vein thrombosis.26,40 Morbidity and mortality is as- drome. J Laparoendosc Adv Surg Tech A. 2006;16:9-14.
sociated mostly with increasing age, recurrent thrombosis, 12. Poultsides GA, Lewis WC, Feld R, et al. Portal vein thrombosis after
portal cholangiopathy, progression of the underlying my- laparoscopic colectomy: thrombolytic therapy via the superior mesen-
teric vein. Am Surg. 2005;71:856-860.
eloproliferative disease or its transformation into acute leu- 13. Chait Y, Condat B, Cazals-Hatem D, et al. Relevance of the criteria
kemia, and other unrelated causes. commonly used to diagnose myeloproliferative disorder in patients
with splanchnic vein thrombosis. Br J Haematol. 2005;129:553-560.
14. Kralovics R, Passamonti F, Buser AS, et al. A gain-of-function mu-
CONCLUSION tation of JAK2 in myeloproliferative disorders. N Engl J Med. 2005;
In summary, portal vein thrombosis represents an important 352:1779-1790.
and perplexing clinical problem, not infrequently encoun- 15. Primignani M, Barosi G, Bergamaschi G, et al. Role of the JAK2
tered by physicians, given the increased use of radiological mutation in the diagnosis of chronic myeloproliferative disorders in
investigations. Deeper insights into its association with my- splanchnic vein thrombosis. Hepatology. 2006;44:1528-1534.
16. Kiladjian JJ, Cervantes F, Leebeek FW, et al. The impact of JAK2 and
eloproliferative disorders and other prothrombotic states
MPL mutations on diagnosis and prognosis of splanchnic vein throm-
have made it imperative for physicians to remain cognizant bosis: a report on 241 cases. Blood. 2008;111:4922-4929.
of this condition. There are no randomized trials to guide 17. Gertsch P, Matthews J, Lerut J, et al. Acute thrombosis of the splanch-
therapy in acute and chronic portal vein thrombosis with nic veins. Arch Surg. 1993;128:341-345.
concomitant hepatic cirrhosis, and anticoagulation is gener- 18. Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review.
ally not recommended in this subset of patients. In noncir- Am J Med. 1992;92:173-182.
19. Chandra R, Kapoor D, Tharakan A, et al. Portal biliopathy. J Gastro-
rhotic portal vein thrombosis with underlying hypercoagu- enterol Hepatol. 2001;16:1086-1092.
lable states, convincing data favoring anticoagulation are 20. Kearon C, Kahn SR, Agnelli G, et al. Antithrombotic therapy for
increasingly becoming available. Considerable progress has venous thromboembolic disease: American College of Chest Physi-
been made in understanding the pathophysiology and man- cians Evidence-Based Clinical Practice Guidelines (8th Edition).
agement of portal vein thrombosis since its initial recogni- Chest. 2008;133(6 Suppl):454S-545S.
21. Haddad MC, Clark DC, Sharif HS, et al. MR, CT, and ultrasonography
tion. It is hoped that more evidence-based therapeutic op-
of splanchnic venous thrombosis. Gastrointest Radiol. 1992;17:34-40.
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in the diagnosis of cavernous transformation of the portal vein. J Clin
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ACKNOWLEDGMENT 23. Lai L, Brugge WR. Endoscopic ultrasound is a sensitive and specific
The authors wish to thank Ms. Yvette Pinales for her assis- test to diagnose portal venous system thrombosis (PVST). Am J Gas-
troenterol. 2004;99:40-44.
tance in the preparation of the manuscript and Sushil Sona-
24. Fimognari FL, Violi F. Portal vein thrombosis in liver cirrhosis. Intern
vane, MD, for providing images for this article. Emerg Med. 2008;3:213-218.
25. de Franchis R. Evolving consensus in portal hypertension. Report of
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