Abstract
The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis; the simultaneous involvement of additional regions is frequent, and clinical presentations and risk factors may be shared. The annual incidence of BCS and isolated mesenteric vein thrombosis is less than one per million individuals, while the incidence of EHPVO is about four per million; autopsy studies, however, suggest higher numbers. Current advances in non-invasive vascular imaging allow for the identification of chronic or asymptomatic forms. Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloproliferative neoplasms (MPN) are the leading systemic cause of splanchnic vein thrombosis, and are diagnosed in half the BCS patients and one-third of the EHPVO patients. The molecular marker JAK2 V617F is detectable in a large majority of patients with overt MPN, and up to 40% of patients without overt MPN. Inherited thrombophilia is present in at least one-third of the patients, and the factor V Leiden or the prothrombin G20210A mutations are the most common mutations found in BCS or EHPVO patients, respectively. Multiple factors are present in approximately one-third of the patients with BCS and two-thirds of the patients with portal vein thrombosis. Immediate anticoagulation with heparin is used to treat patients acutely. Upon clinical deterioration, catheter-directed thrombolysis or transjugular intrahepatic portosystemic shunt is used in conjunction with anticoagulation. Long-term oral anticoagulation with vitamin K-antagonists (VKA) is recommended in all BCS patients, and in the patients with a permanent prothrombotic state associated with an unprovoked EHPVO. In patients with an unprovoked EHPVO and no prothrombotic conditions, or in those with a provoked EHPVO, anticoagulant treatment is recommended for a minimum of 3–6 months.
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Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC, European Group for the Study of Vascular Disorders of the Liver (2003) Budd-Chiari syndrome: a review by an expert panel. J Hepatol 38:364–371
Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M, Jafri W, Kumar A, Kudo M, Lesmana LA, Sharma BC, Shiha G, de Janaka Silva H, Members of the APASL Working Party on Portal Hypertension (2006) Consensus on extra-hepatic portal vein obstruction. Liver Int 26:512–519
Valla D (2004) Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol 19:204–211
Almdal TP, Sorensen TI (1991) Incidence of parenchymal liver diseases in Denmark, 1981 to 1985: analysis of hospitalization registry data. The Danish Association for the Study of the Liver. Hepatology 13:650–655
Rajani R, Melin T, Björnsson E, Broomé U, Sangfelt P, Danielsson A, Gustavsson A, Grip O, Svensson H, Lööf L, Wallerstedt S, Almer SH (2009) Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival—an 18-year experience. Liver Int 29:253–259
Eapen CE, Mammen T, Moses V, Shyamkumar NK (2007) Changing profile of Budd-Chiari syndrome in India. Indian J Gastroenterol 26:77–81
Ogren M, Bergqvist D, Björck M, Acosta S, Eriksson H, Sternby NH (2006) Portal vein thrombosis: prevalence, patient characteristics and lifetime risk: a population study based on 23, 796 consecutive autopsies. World J Gastroenterol 12:2115–2119
Acosta S, Alhadad A, Svensson P, Ekberg O (2008) Epidemiology, risk and prognostic factors in mesenteric venous thrombosis. Br J Surg 95:1245–1251
Acosta S, Ogren M, Sternby NH, Bergqvist D, Björck M (2005) Mesenteric venous thrombosis with transmural intestinal infarction: a population-based study. J Vasc Surg 41:59–63
Dentali F, Ageno W, Witt D, Malato A, Clark N, Garcia D, McCool K, Siragusa S, Dyke S, Crowther M, WARPED consortium (2009) Natural history of mesenteric venous thrombosis in patients treated with vitamin K antagonists: a multi-centre, retrospective cohort study. Thromb Haemost 102:501–504
Darvish Murad S, Valla DC, de Groen PC, Zeitoun G, Hopmans JA, Haagsma EB, van Hoek B, Hansen BE, Rosendaal FR, Janssen HL (2004) Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 39:500–508
Mentha G, Giostra E, Majno PE, Bechstein WO, Neuhaus P, O’Grady J, Praseedom RK, Burroughs AK, Le Treut YP, Kirkegaard P, Rogiers X, Ericzon BG, Hockerstedt K, Adam R, Klempnauer J (2006) Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol 44:520–528
Darvish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, Trebicka J, Morard I, Lasser L, Heller J, Hadengue A, Langlet P, Miranda H, Primignani M, Elias E, Leebeek FW, Rosendaal FR, Garcia-Pagan JC, Valla DC, Janssen HL, EN-Vie (European Network for Vascular Disorders of the Liver) (2009) Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 151:167–175
Senzolo M, Cholongitas EC, Patch D, Burroughs AK (2005) Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome. Nat Clin Pract Gastroenterol Hepatol 2:182–190
Hadengue A, Poliquin M, Vilgrain V, Belghiti J, Degott C, Erlinger S, Benhamou JP (1994) The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 106:1042–1047
Zeitoun G, Escolano S, Hadengue A, Azar N, Younsi ElM, Mallet A, Boudet MJ, Hay JM, Erlinger S, Benhamou JP, Belghiti J, Valla D (1999) Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology 30:84–89
Darvish Murad S, Valla DC, de Groen PC, Zeitoun G, Haagsma EB, Kuipers EJ, Janssen HL (2006) Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis. Am J Gastroenterol 101:83–90
Kamath P (2006) Budd–Chiari syndrome: radiologic findings. Liver Transp 12:S21–S22
Condat B, Valla D (2006) Nonmalignant portal vein thrombosis in adults. Nat Clin Pract Gastroenterol Hepatol 3:505–515
Condat B, Pessione F, Helene Denninger M, Hillaire S, Valla D (2000) Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy. Hepatology 32:466–470
Janssen HL, Wijnhoud A, Haagsma EB, van Uum SH, van Nieuwkerk CM, Adang RP, Chamuleau RA, van Hattum J, Vleggaar FP, Hansen BE, Rosendaal FR, van Hoek B (2001) Extrahepatic portal vein thrombosis: aetiology and determinants of survival. Gut 49:720–724
Tessler FN, Gehring BJ, Gomes AS, Perrella RR, Ragavendra N, Busuttil RW, Grant EG (1991) Diagnosis of portal vein thrombosis: value of color Doppler imaging. AJR Am J Roentgenol 157:293–296
Bradbury MS, Kavanagh PV, Chen MY, Weber TM, Bechtold RE (2002) Noninvasive assessment of portomesenteric venous thrombosis: current concepts and imaging strategies. J Comput Assist Tomogr 26:392–404
Kumar S, Sarr MG, Kamath PS (2001) Mesenteric vein thrombosis. N Engl J Med 345:1683–1688
Denninger MH, Chaït Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, Erlinger S, Briere J, Valla D (2000) Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 31:587–591
Janssen HL, Meinardi JR, Vleggaar FP, van Uum SH, Haagsma EB, van Der Meer FJ, van Hattum J, Chamuleau RA, Adang RP, Vandenbroucke JP, van Hoek B, Rosendaal FR (2000) Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. Blood 96:2364–2368
Primignani M, Barosi G, Bergamaschi G, Gianelli U, Fabris F, Reati R, Dell’Era A, Bucciarelli P, Mannucci PM (2006) Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology 44:1528–1534
Plessier A, Darwish Murad S, Hernandez Guerra M, European Network for Vascular Disorders of the Liver (EN-Vie) et al (2009) Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. Hepatology 51:210–218
De Stefano V, Teofili L, Leone G, Michiels JJ (1997) Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with Budd-Chiari syndrome or portal vein thrombosis. Semin Thromb Hemost 23:411–418
Dahabreh IJ, Zoi K, Giannouli S, Zoi C, Loukopoulos D, Voulgarelis M (2009) Is JAK2 V617F mutation more than a diagnostic index? A meta-analysis of clinical outcomes in essential thrombocythemia. Leuk Res 33:67–73
Fiorini A, Chiusolo P, Rossi E, Za T, De Ritis DG, Ciminello A, Leone G, De Stefano V (2009) Absence of the JAK2 exon 12 mutations in patients with splanchnic venous thrombosis and without overt myeloproliferative neoplasms. Am J Hematol 84:126–127
Dentali F, Squizzato A, Brivio L, Appio L, Campiotti L, Crowther M, Grandi AM, Ageno W (2009) JAK2V617F mutation for the early diagnosis of Ph-myeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis. Blood 113:5617–5623
Marchetti M, Castoldi E, Spronk HM, van Oerle R, Balducci D, Barbui T, Rosing J, Ten Cate H, Falanga A (2008) Thrombin generation and activated protein C resistance in patients with essential thrombocythemia and polycythemia vera. Blood 112:4061–4068
Martinelli I, Primignani M, Aghemo A, Reati R, Bucciarelli P, Fabris F, Battaglioli T, Dell’Era A, Mannucci PM (2009) High levels of factor VIII and risk of extra-hepatic portal vein obstruction. J Hepatol 50:916–922
Dentali F, Galli M, Gianni M, Ageno W (2008) Inherited thrombophilic abnormalities and risk of portal vein thrombosis. A meta-analysis. Thromb Haemost 99:675–682
Ziakas PD, Poulou LS, Rokas GI, Bartzoudis D, Voulgarelis M (2007) Thrombosis in paroxysmal nocturnal hemoglobinuria: sites, risks, outcome. An overview. J Thromb Haemost 5:642–645
Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M (1997) Budd-Chiari syndrome: a common complication of Behçet’s disease. Am J Gastroenterol 92:858–862
Rautou PE, Plessier A, Bernuau J, Denninger MH, Moucari R, Valla D (2009) Pregnancy: a risk factor for Budd-Chiari syndrome? Gut 58:606–608
Valla D, Le MG, Poynard T, Zucman N, Rueff B, Benhamou JP (1986) Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. Gastroenterology 90:807–811
Stamou KM, Toutouzas KG, Kekis PB, Nakos S, Gafou A, Manouras A, Krespis E, Katsaragakis S, Bramis J (2006) Prospective study of the incidence and risk factors of postsplenectomy thrombosis of the portal, mesenteric, and splenic veins. Arch Surg 141:663–669
Fimognari FL, Violi F (2008) Portal vein thrombosis in liver cirrhosis. Intern Emerg Med 3:213–218
Valla DC (2008) Budd-Chiari syndrome and veno-occlusive disease/sinusoidal obstruction syndrome. Gut 57:1469–1478
Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, Condat B, Farges O, Chagneau C, de Ledinghen V, Francoz C, Sauvanet A, Vilgrain V, Belghiti J, Durand F, Valla D (2006) Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 44:1308–1316
Sharma S, Texeira A, Texeira P, Elias E, Wilde J, Olliff SP (2004) Pharmacological thrombolysis in Budd-Chiari syndrome: a single centre experience and review of the literature. J Hepatol 40:172–180
Smalberg JH, Spaander MV, Jie KS, Pattynama PM, van Buuren HR, van den Berg B, Janssen HL, Leebeek FW (2008) Risks and benefits of transcatheter thrombolytic therapy in patients with splanchnic venous thrombosis. Thromb Haemost 100:1084–1088
Liu FY, Wang MQ, Fan QS, Duan F, Wang ZJ, Song P (2009) Interventional treatment for symptomatic acute–subacute portal and superior mesenteric vein thrombosis. World J Gastroenterol 15:5028–5034
Garcia-Pagán JC, Heydtmann M, Raffa S, Plessier A, Murad S, Fabris F, Vizzini G, Abraldes JG, Olliff S, Nicolini A, Luca A, Primignani M, Janssen HL, Valla D, Elias E, Bosch J, Group Budd-Chiari syndrome-transjugular intrahepatic portosystemic shunt (2008) TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology 135:808–815
Senzolo M, Patch D, Miotto D, Ferronato C, Cholongitas E, Burroughs AK (2008) Interventional treatment should be incorporated in the algorithm for the management of patients with portal vein thrombosis. Hepatology 48:1352–1353
Condat B, Pessione F, Hillaire S, Denninger MH, Guillin MC, Poliquin M, Hadengue A, Erlinger S, Valla D (2001) Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 120:490–497
Amitrano L, Guardascione MA, Scaglione M, Pezzullo L, Sangiuliano N, Armellino MF, Manguso F, Margaglione M, Ames PR, Iannaccone L, Grandone E, Romano L, Balzano A (2007) Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol 102:2464–2470
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De Stefano, V., Martinelli, I. Splanchnic vein thrombosis: clinical presentation, risk factors and treatment. Intern Emerg Med 5, 487–494 (2010). https://doi.org/10.1007/s11739-010-0413-6
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DOI: https://doi.org/10.1007/s11739-010-0413-6