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Semin Thromb Hemost 2015; 41(05): 494-502
DOI: 10.1055/s-0035-1550439
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Splanchnic Vein Thrombosis

Dominique Valla
1   Hôpital Beaujon, AP-HP, Clichy-la-Garenne, Centre de Recherche de l'Inflammation Paris-Montmartre, Inserm and Université Paris- Diderot, Sorbonne-Paris Cité, Paris, France
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Publikationsdatum:
16. Juni 2015 (online)

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Abstract

Splanchnic vein thrombosis includes thrombosis of the hepatic venous system (Budd–Chiari syndrome) and thrombosis of the portal venous system. Both conditions share uncommon prothrombotic disorders as causal factors, among which myeloproliferative neoplasms rank first. Budd–Chiari syndrome presents with acute or chronic, asymptomatic or severe liver disease. Diagnosis depends on noninvasive imaging of the obstructed hepatic venous outflow tract. A spontaneously fatal course can be prevented by a stepwise approach: (1) anticoagulation therapy, specific therapy for underlying disease, and medical or endoscopic management of liver-related complications, (2) angioplasty/stenting in a second step, and (3) eventually the insertion of transjugular intrahepatic stent shunt or liver transplantation. Recent portal vein thrombosis mostly jeopardizes the gut. Early anticoagulation prevents thrombus extension but is incompletely successful in achieving recanalization. Chronic portal vein thrombosis is complicated by bleeding related to portal hypertension, which can be prevented by usual pharmacological and endoscopic means. The prevention of recurrent thrombosis is achieved by anticoagulation therapy the impact of which on the risk of bleeding remains unclear. Portal vein thrombosis in patients with cirrhosis is likely neither a direct consequence of nor a direct cause for liver disease progression. Therefore, the indications and effects of anticoagulation therapy for portal vein thrombosis in patients with cirrhosis remain uncertain.

Keywords

hepatic vein thrombosis - Budd–Chiari syndrome - portal vein thrombosis - cavernoma - TIPS
 

  • References

  • 1 Rajani R, Almer S. Incidence and prevalence rates in Budd-Chiari syndrome. Gut 2009; 58 (6) 889
    PubMedGoogle Scholar
  • 2 Rajani R, Björnsson E, Bergquist A , et al. The epidemiology and clinical features of portal vein thrombosis: a multicentre study. Aliment Pharmacol Ther 2010; 32 (9) 1154-1162
    CrossrefPubMedGoogle Scholar
  • 3 Ogren M, Bergqvist D, Björck M, Acosta S, Eriksson H, Sternby NH. Portal vein thrombosis: prevalence, patient characteristics and lifetime risk: a population study based on 23,796 consecutive autopsies. World J Gastroenterol 2006; 12 (13) 2115-2119
    CrossrefPubMedGoogle Scholar
  • 4 Plessier A, Darwish-Murad S, Hernandez-Guerra M , et al; European Network for Vascular Disorders of the Liver (EN-Vie). Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. Hepatology 2010; 51 (1) 210-218
    CrossrefPubMedGoogle Scholar
  • 5 Seijo S, Plessier A, Hoekstra J , et al; European Network for Vascular Disorders of the Liver. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology 2013; 57 (5) 1962-1968
    CrossrefPubMedGoogle Scholar
  • 6 Valla DC. Primary Budd-Chiari syndrome. J Hepatol 2009; 50 (1) 195-203
    CrossrefPubMedGoogle Scholar
  • 7 Chait Y, Condat B, Cazals-Hatem D , et al. Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol 2005; 129 (4) 553-560
    CrossrefPubMedGoogle Scholar
  • 8 Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood 2012; 120 (25) 4921-4928
    CrossrefPubMedGoogle Scholar
  • 9 Turon F, Cervantes F, Colomer D, Baiges A, Hernández-Gea V, Garcia-Pagán JC. Role of calreticulin mutations in the aetiological diagnosis of splanchnic vein thrombosis. J Hepatol 2015; 62 (1) 72-74
    CrossrefPubMedGoogle Scholar
  • 10 Tanaka M, Wanless IR. Pathology of the liver in Budd-Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. Hepatology 1998; 27 (2) 488-496
    CrossrefPubMedGoogle Scholar
  • 11 Cazals-Hatem D, Vilgrain V, Genin P , et al. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers. Hepatology 2003; 37 (3) 510-519
    CrossrefPubMedGoogle Scholar
  • 12 Hadengue A, Poliquin M, Vilgrain V , et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 1994; 106 (4) 1042-1047
    CrossrefPubMedGoogle Scholar
  • 13 Simonetto DA, Yang HY, Yin M , et al. Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology 2015; 61 (2) 648-659
    CrossrefPubMedGoogle Scholar
  • 14 Paradis V, Bièche I, Dargère D , et al. Quantitative gene expression in Budd-Chiari syndrome: a molecular approach to the pathogenesis of the disease. Gut 2005; 54 (12) 1776-1781
    CrossrefPubMedGoogle Scholar
  • 15 Plessier A, Rautou PE, Valla DC. Management of hepatic vascular diseases. J Hepatol 2012; 56 (Suppl. 01) S25-S38
    PubMedGoogle Scholar
  • 16 de Franchis R ; Baveno V Faculty. Revising consensus in portal hypertension: report of the Baveno V consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol 2010; 53 (4) 762-768
    CrossrefPubMedGoogle Scholar
  • 17 Sharma S, Texeira A, Texeira P, Elias E, Wilde J, Olliff SP. Pharmacological thrombolysis in Budd Chiari syndrome: a single centre experience and review of the literature. J Hepatol 2004; 40 (1) 172-180
    PubMedGoogle Scholar
  • 18 Rautou PE, Angermayr B, Garcia-Pagan JC , et al. Pregnancy in women with known and treated Budd-Chiari syndrome: maternal and fetal outcomes. J Hepatol 2009; 51 (1) 47-54
    CrossrefPubMedGoogle Scholar
  • 19 Moucari R, Rautou PE, Cazals-Hatem D , et al. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut 2008; 57 (6) 828-835
    CrossrefPubMedGoogle Scholar
  • 20 Valla DC, Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol 2000; 32 (5) 865-871
    CrossrefPubMedGoogle Scholar
  • 21 Acosta S, Alhadad A, Verbaan H, Ogren M. The clinical importance in differentiating portal from mesenteric venous thrombosis. Int Angiol 2011; 30 (1) 71-78
    PubMedGoogle Scholar
  • 22 Turnes J, García-Pagán JC, González M , et al. Portal hypertension-related complications after acute portal vein thrombosis: impact of early anticoagulation. Clin Gastroenterol Hepatol 2008; 6 (12) 1412-1417
    CrossrefPubMedGoogle Scholar
  • 23 Hall TC, Garcea G, Metcalfe M, Bilku D, Dennison AR. Management of acute non-cirrhotic and non-malignant portal vein thrombosis: a systematic review. World J Surg 2011; 35 (11) 2510-2520
    CrossrefPubMedGoogle Scholar
  • 24 Khanna R, Sarin SK. Non-cirrhotic portal hypertension - diagnosis and management. J Hepatol 2014; 60 (2) 421-441
    CrossrefPubMedGoogle Scholar
  • 25 Berzigotti A, García-Criado A, Darnell A, García-Pagán JC. Imaging in clinical decision-making for portal vein thrombosis. Nat Rev Gastroenterol Hepatol 2014; 11 (5) 308-316
    CrossrefPubMedGoogle Scholar
  • 26 Guérin F, Bidault V, Gonzales E, Franchi-Abella S, De Lambert G, Branchereau S. Meso-Rex bypass for extrahepatic portal vein obstruction in children. Br J Surg 2013; 100 (12) 1606-1613
    CrossrefPubMedGoogle Scholar
  • 27 Condat B, Pessione F, Helene Denninger M, Hillaire S, Valla D. Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy. Hepatology 2000; 32 (3) 466-470
    CrossrefPubMedGoogle Scholar
  • 28 Amitrano L, Guardascione MA, Scaglione M , et al. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol 2007; 102 (11) 2464-2470
    CrossrefPubMedGoogle Scholar
  • 29 Spaander MC, Hoekstra J, Hansen BE, Van Buuren HR, Leebeek FW, Janssen HL. Anticoagulant therapy in patients with non-cirrhotic portal vein thrombosis: effect on new thrombotic events and gastrointestinal bleeding. J Thromb Haemost 2013; 11 (3) 452-459
    CrossrefPubMedGoogle Scholar
  • 30 Condat B, Pessione F, Hillaire S , et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001; 120 (2) 490-497
    CrossrefPubMedGoogle Scholar
  • 31 Mínguez B, García-Pagán JC, Bosch J , et al. Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. Hepatology 2006; 43 (4) 707-714
    CrossrefPubMedGoogle Scholar
  • 32 Condat B, Vilgrain V, Asselah T , et al. Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology 2003; 37 (6) 1302-1308
    CrossrefPubMedGoogle Scholar
  • 33 Llop E, de Juan C, Seijo S , et al. Portal cholangiopathy: radiological classification and natural history. Gut 2011; 60 (6) 853-860
    CrossrefPubMedGoogle Scholar
  • 34 Orr DW, Harrison PM, Devlin J , et al. Chronic mesenteric venous thrombosis: evaluation and determinants of survival during long-term follow-up. Clin Gastroenterol Hepatol 2007; 5 (1) 80-86
    CrossrefPubMedGoogle Scholar
  • 35 Sarin SK, Gupta N, Jha SK , et al. Equal efficacy of endoscopic variceal ligation and propranolol in preventing variceal bleeding in patients with noncirrhotic portal hypertension. Gastroenterology 2010; 139 (4) 1238-1245
    CrossrefPubMedGoogle Scholar
  • 36 Merkel C, Bolognesi M, Bellon S , et al. Long-term follow-up study of adult patients with non-cirrhotic obstruction of the portal system: comparison with cirrhotic patients. J Hepatol 1992; 15 (3) 299-303
    CrossrefPubMedGoogle Scholar
  • 37 Janssen HL. Changing perspectives in portal vein thrombosis. Scand J Gastroenterol Suppl 2000; 232 (232) 69-73
    PubMedGoogle Scholar
  • 38 Wang L, Liu GJ, Chen YX, Dong HP, Wang LX. Sinistral portal hypertension: clinical features and surgical treatment of chronic splenic vein occlusion. Med Princ Pract 2012; 21 (1) 20-23
    CrossrefPubMedGoogle Scholar
  • 39 Butler JR, Eckert GJ, Zyromski NJ, Leonardi MJ, Lillemoe KD, Howard TJ. Natural history of pancreatitis-induced splenic vein thrombosis: a systematic review and meta-analysis of its incidence and rate of gastrointestinal bleeding. HPB (Oxford) 2011; 13 (12) 839-845
    CrossrefPubMedGoogle Scholar
  • 40 Tsochatzis EA, Senzolo M, Germani G, Gatt A, Burroughs AK. Systematic review: portal vein thrombosis in cirrhosis. Aliment Pharmacol Ther 2010; 31 (3) 366-374
    CrossrefPubMedGoogle Scholar
  • 41 Qi X, Han G, Fan D. Management of portal vein thrombosis in liver cirrhosis. Nat Rev Gastroenterol Hepatol 2014; 11 (7) 435-446
    CrossrefPubMedGoogle Scholar
  • 42 Nery F, Chevret S, Condat B , et al; Groupe d'Etude et de Traitement du Carcinome Hépatocellulaire. Causes and consequences of portal vein thrombosis in 1,243 patients with cirrhosis: Results of a longitudinal study. Hepatology 2015; 61 (2) 660-667
    CrossrefPubMedGoogle Scholar
  • 43 Maruyama H, Okugawa H, Takahashi M, Yokosuka O. De novo portal vein thrombosis in virus-related cirrhosis: predictive factors and long-term outcomes. Am J Gastroenterol 2013; 108 (4) 568-574
    CrossrefPubMedGoogle Scholar
  • 44 Luca A, Caruso S, Milazzo M , et al. Natural course of extrahepatic nonmalignant partial portal vein thrombosis in patients with cirrhosis. Radiology 2012; 265 (1) 124-132
    CrossrefPubMedGoogle Scholar
  • 45 Englesbe MJ, Schaubel DE, Cai S, Guidinger MK, Merion RM. Portal vein thrombosis and liver transplant survival benefit. Liver Transpl 2010; 16 (8) 999-1005
    CrossrefPubMedGoogle Scholar
  • 46 Englesbe MJ, Kubus J, Muhammad W , et al. Portal vein thrombosis and survival in patients with cirrhosis. Liver Transpl 2010; 16 (1) 83-90
    CrossrefPubMedGoogle Scholar
  • 47 Wanless IR, Wong F, Blendis LM, Greig P, Heathcote EJ, Levy G. Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension. Hepatology 1995; 21 (5) 1238-1247
    CrossrefPubMedGoogle Scholar
  • 48 Villa E, Cammà C, Marietta M , et al. Enoxaparin prevents portal vein thrombosis and liver decompensation in patients with advanced cirrhosis. Gastroenterology 2012; 143 (5) 1253-60.e1 , 4
    CrossrefPubMedGoogle Scholar
  • 49 Amitrano L, Guardascione MA, Brancaccio V , et al. Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis. J Hepatol 2004; 40 (5) 736-741
    CrossrefPubMedGoogle Scholar
  • 50 Francoz C, Valla D, Durand F. Portal vein thrombosis, cirrhosis, and liver transplantation. J Hepatol 2012; 57 (1) 203-212
    CrossrefPubMedGoogle Scholar
  • 51 Qi X, De Stefano V, Li H, Dai J, Guo X, Fan D. Anticoagulation for the treatment of portal vein thrombosis in liver cirrhosis: A systematic review and meta-analysis of observational studies. Eur J Intern Med 2015; 26 (1) 23-29
    CrossrefPubMedGoogle Scholar
  • 52 Luca A, Miraglia R, Caruso S , et al. Short- and long-term effects of the transjugular intrahepatic portosystemic shunt on portal vein thrombosis in patients with cirrhosis. Gut 2011; 60 (6) 846-852
    CrossrefPubMedGoogle Scholar
  • 53 Perarnau JM, Baju A, D'alteroche L, Viguier J, Ayoub J. Feasibility and long-term evolution of TIPS in cirrhotic patients with portal thrombosis. Eur J Gastroenterol Hepatol 2010; 22 (9) 1093-1098
    CrossrefPubMedGoogle Scholar