Advertisement

Acute Portal Vein Thrombosis: Aetiopathogenesis, Diagnosis and Management

  • Sahaj Rathi
  • Radha K. Dhiman
Chapter
Part of the GI Surgery Annual book series (GISA, volume 22)

Abstract

Portal vein thrombosis (PVT) refers to the occlusion of the trunk of the portal vein (PV) by formation of a luminal thrombus. This may extend into one or both of its intrahepatic branches, or its tributaries—the splenic and the superior mesenteric vein. The thrombus may be complete, occluding the whole lumen; or partial, sparing a peripheral stream to allow flow of blood. Occlusion of the PV by tumoral invasion is usually classified separately as a malignant thrombus.

Keywords

Portal Vein Thrombosis Superior Mesenteric Vein Paroxysmal Nocturnal Haemoglobinuria Myeloproliferative Disorder Complete Recanalization 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Arora NK, Lodha R, Gulati S, Gupta AK, Mathur P, Joshi MS, et al. Portal hypertension in north Indian children. Indian J Pediatr 1998;65:585–91.CrossRefPubMedGoogle Scholar
  2. 2.
    Chawla Y, Duseja A, Dhiman RK. Review article: The modern management of portal vein thrombosis. Aliment Pharmacol Ther 2009;30:881–94.CrossRefPubMedGoogle Scholar
  3. 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;12:2115–19.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Basit SA, Stone CD, Gish R. Portal vein thrombosis. Clin Liver Dis 2015;19:199–221.CrossRefPubMedGoogle Scholar
  5. 5.
    Nonami T, Yokoyama I, Iwatsuki S, Starzl TE. The incidence of portal vein thrombosis at liver transplantation. Hepatology 1992;16:1195–8.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Llovet JM, Bruix J. Prospective validation of the Cancer of the Liver Italian Program (CLIP) score: A new prognostic system for patients with cirrhosis and hepatocellular carcinoma. Hepatology 2000;32:679–80.CrossRefPubMedGoogle Scholar
  7. 7.
    Pirisi M, Avellini C, Fabris C, Scott C, Bardus P, Soardo G, et al. Portal vein thrombosis in hepatocellular carcinoma: Age and sex distribution in an autopsy study. J Cancer Res Clin Oncol 1998;124:397–400.CrossRefPubMedGoogle Scholar
  8. 8.
    Rabe C, Pilz T, Klostermann C, Berna M, Schild HH, Sauerbruch T, et al. Clinical characteristics and outcome of a cohort of 101 patients with hepatocellular carcinoma. World J Gastroenterol 2001;7:208–15.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Amitrano L, Guardascione MA, Brancaccio V, Margaglione M, Manguso F, Iannaccone L, et al. Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis. J Hepatol 2004;40:736–41.CrossRefPubMedGoogle Scholar
  10. 10.
    Belli L, Romani F, Sansalone CV, Aseni P, Rondinara G. Portal thrombosis in cirrhotics. A retrospective analysis. Ann Surg 1986;203:286–91.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    DeLeve LD, Valla DC, Garcia-Tsao G; American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009;49:1729–64.CrossRefPubMedGoogle Scholar
  12. 12.
    Yadav S, Dutta AK, Sarin SK. Do umbilical vein catheterization and sepsis lead to portal vein thrombosis? A prospective, clinical, and sonographic evaluation. J Pediatr Gastroenterol Nutr 1993;17:392–6.CrossRefPubMedGoogle Scholar
  13. 13.
    Janssen HL, Meinardi JR, Vleggaar FP, van Uum SH, Haagsma EB, van Der Meer FJ, et al. 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 2000;96:2364–8.PubMedGoogle Scholar
  14. 14.
    Bombeli T, Basic A, Fehr J. Prevalence of hereditary thrombophilia in patients with thrombosis in different venous systems. Am J Hematol 2002;70:126–32.CrossRefPubMedGoogle Scholar
  15. 15.
    Bhattacharyya M, Makharia G, Kannan M, Ahmed RP, Gupta PK, Saxena R. Inherited prothrombotic defects in Budd–Chiari syndrome and portal vein thrombosis: A study from North India. Am J Clin Pathol 2004;121:844–7.CrossRefPubMedGoogle Scholar
  16. 16.
    Primignani M, Martinelli I, Bucciarelli P, Battaglioli T, Reati R, Fabris F, et al. Risk factors for thrombophilia in extrahepatic portal vein obstruction. Hepatology 2005;41:603–8.CrossRefPubMedGoogle Scholar
  17. 17.
    Turnes J, García-Pagán JC, González M, Aracil C, Calleja JL, Ripoll C, et al. Portal hypertension-related complications after acute portal vein thrombosis: Impact of early anticoagulation. Clin Gastroenterol Hepatol 2008;6:1412–17.CrossRefPubMedGoogle Scholar
  18. 18.
    Denninger MH, Chaït Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, et al. Cause of portal or hepatic venous thrombosis in adults: The role of multiple concurrent factors. Hepatology 2000;31:587–91.CrossRefPubMedGoogle Scholar
  19. 19.
    Brière JB. Budd–Chiari syndrome and portal vein thrombosis associated with myeloproliferative disorders: Diagnosis and management. Semin Thromb Hemost 2006;32:208–18.CrossRefPubMedGoogle Scholar
  20. 20.
    De Stefano V, Teofili L, Leone G, Michiels JJ. 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 1997;23:411–18.CrossRefPubMedGoogle Scholar
  21. 21.
    Chait Y, Condat B, Cazals-Hatem D, Rufat P, Atmani S, Chaoui D, et al. Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol 2005;129:553–60.CrossRefPubMedGoogle Scholar
  22. 22.
    Kiladjian JJ, Cervantes F, Leebeek FW, Marzac C, Cassinat B, Chevret S, et al. The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: A report on 241 cases. Blood 2008;111:4922–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Patel RK, Lea NC, Heneghan MA, Westwood NB, Milojkovic D, Thanigaikumar M, et al. Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd–Chiari syndrome. Gastroenterology 2006;130:2031–8.CrossRefPubMedGoogle Scholar
  24. 24.
    Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA, et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: Recommendations from an ad hoc international expert panel. Blood 2007;110:1092–7.CrossRefPubMedGoogle Scholar
  25. 25.
    Poultsides GA, Lewis WC, Feld R, Walters DL, Cherry DA, Ruby ST. Portal vein thrombosis after laparoscopic colectomy: Thrombolytic therapy via the superior mesenteric vein. Am Surg 2005;71:856–60.PubMedGoogle Scholar
  26. 26.
    de Bruijne EL, Darwish Murad S, de Maat MP, Tanck MW, Haagsma EB, van Hoek B, et al. Genetic variation in thrombin-activatable fibrinolysis inhibitor (TAFI) is associated with the risk of splanchnic vein thrombosis. Thromb Haemost 2007;97:181–5.PubMedGoogle Scholar
  27. 27.
    Martinelli I, Primignani M, Aghemo A, Reati R, Bucciarelli P, Fabris F, et al. High levels of factor VIII and risk of extra-hepatic portal vein obstruction. J Hepatol 2009;50:916–22.CrossRefPubMedGoogle Scholar
  28. 28.
    Mohanty D, Shetty S, Ghosh K, Pawar A, Abraham P. Hereditary thrombophilia as a cause of Budd-Chiari syndrome: A study from Western India. Hepatology 2001;34(4 Pt 1):666–70.CrossRefPubMedGoogle Scholar
  29. 29.
    Sharma S, Kumar SI, Poddar U, Yachha SK, Aggarwal R. Factor V Leiden and prothrombin gene G20210A mutations are uncommon in portal vein thrombosis in India. Indian J Gastroenterol 2006;25:236–9.Google Scholar
  30. 30.
    Koshy A, Jeyakumari M. Factor V Leiden is not commonly associated with idiopathic portal vein thrombosis in southern India. Indian J Gastroenterol 2006;25:1–2.Google Scholar
  31. 31.
    Koshy A, Jeyakumari M. High FVIII level is associated with idiopathic portal vein thrombosis in South India. Am J Med 2007;120:552.e9–11.Google Scholar
  32. 32.
    Shah SR, DasGupta A, Sharma A, Joshi A, Desai D, Abraham P, et al. Thrombophilic conditions in non-cirrhotic portal vein thrombosis. Indian J Gastroenterol 2005;24:205–10.PubMedGoogle Scholar
  33. 33.
    Liappis AP, Roberts AD, Schwartz AM, Simon GL. Thrombosis and infection: A case of transient anti-cardiolipin antibody associated with pylephlebitis. Am J Med Sci 2003;325:365–8.CrossRefPubMedGoogle Scholar
  34. 34.
    Amitrano L, Guardascione MA, Scaglione M, Menchise A, Romano L, Balzano A. Acute portal and mesenteric thrombosis: Unusual presentation of cytomegalovirus infection. Eur J Gastroenterol Hepatol 2006;18:443–5.CrossRefPubMedGoogle Scholar
  35. 35.
    Habu D, Nishiguchi S, Shiomi S, Tamori A, Sakaguchi H, Takeda T, et al. Portal vein thrombosis following percutaneous ethanol injection therapy for hepatocellular carcinoma. Indian J Gastroenterol 2002;21:162–3.PubMedGoogle Scholar
  36. 36.
    Zheng RQ, Kudo M, Inui K, Suetomi Y, Minami Y, Chung H, et al. Transient portal vein thrombosis caused by radiofrequency ablation for hepatocellular carcinoma. J Gastroenterol 2003;38:101–3.CrossRefPubMedGoogle Scholar
  37. 37.
    Matsumoto K, Yamao K, Ohashi K, Watanabe Y, Sawaki A, Nakamura T, et al. Acute portal vein thrombosis after EUS-guided FNA of pancreatic cancer: Case report. Gastrointest Endosc 2003;57:269–71.CrossRefPubMedGoogle Scholar
  38. 38.
    Zocco MA, Di Stasio E, De Cristofaro R, Novi M, Ainora ME, Ponziani F, et al. Thrombotic risk factors in patients with liver cirrhosis: Correlation with MELD scoring system and portal vein thrombosis development. J Hepatol 2009;51:682–9.CrossRefPubMedGoogle Scholar
  39. 39.
    Spaander VM, van Buuren HR, Janssen HL. Review article: The management of non-cirrhotic non-malignant portal vein thrombosis and concurrent portal hypertension in adults. Aliment Pharmacol Ther 2007;26 (Suppl 2):203–9. Erratum in: Aliment Pharmacol Ther 2008;27:528–9.Google Scholar
  40. 40.
    Kumar S, Sarr MG, Kamath PS. Mesenteric venous thrombosis. N Engl J Med 2001;345:1683–8.CrossRefPubMedGoogle Scholar
  41. 41.
    Gertsch P, Matthews J, Lerut J, Luder P, Blumgart LH. Acute thrombosis of the splanchnic veins. Arch Surg 1993;128:341–5.CrossRefPubMedGoogle Scholar
  42. 42.
    Thomas RM, Ahmad SA. Management of acute post-operative portal venous thrombosis. J Gastrointest Surg 2010;14:570–7.CrossRefPubMedGoogle Scholar
  43. 43.
    Pleassier A, Murad SD, Hernandez-Guerra M, Consigny Y, Fabris F, Heller J, et al. A prospective multicentric follow-up study on 105 patients with acute portal vein thrombosis (PVT): Results from the European network for vascular disorders of the liver (en-vie). Hepatology 2007;46 (Suppl 1):310A.Google Scholar
  44. 44.
    Klempnauer J, Grothues F, Bektas H, Pichlmayr R. Results of portal thrombectomy and splanchnic thrombolysis for the surgical management of acute mesentericoportal thrombosis. Br J Surg 1997;84:129–32.CrossRefPubMedGoogle Scholar
  45. 45.
    Plessier A, Darwish-Murad S, Hernandez-Guerra M, Consigny Y, Fabris F, Trebicka J, 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;51:210–18.CrossRefPubMedGoogle Scholar
  46. 46.
    Parikh S, Shah R, Kapoor P. Portal vein thrombosis. Am J Med 2010;123:111–19.CrossRefPubMedGoogle Scholar
  47. 47.
    Tessler FN, Gehring BJ, Gomes AS, Perrella RR, Ragavendra N, Busuttil RW, et al. Diagnosis of portal vein thrombosis: Value of color Doppler imaging. AJR Am J Roentgenol 1991;157:293–6.CrossRefPubMedGoogle Scholar
  48. 48.
    Bach AM, Hann LE, Brown KT, Getrajdman GI, Herman SK, Fong Y, et al. Portal vein evaluation with US: Comparison to angiography combined with CT arterial portography. Radiology 1996;201:149–54.CrossRefPubMedGoogle Scholar
  49. 49.
    Rossi S, Rosa L, Ravetta V, Cascina A, Quaretti P, Azzaretti A, et al. Contrast-enhanced versus conventional and color Doppler sonography for the detection of thrombosis of the portal and hepatic venous systems. AJR Am J Roentgenol 2006;186:763–73.CrossRefPubMedGoogle Scholar
  50. 50.
    Bayraktar Y, Harmanci O. Etiology and consequences of thrombosis in abdominal vessels. World J Gastroenterol 2006;12:1165–74.CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Cakmak O, Elmas N, Tamsel S, Demirpolat G, Sever A, Altunel E, et al. Role of contrast-enhanced 3D magnetic resonance portography in evaluating portal venous system compared with color Doppler ultrasonography. Abdom Imaging 2008;33:65–71.CrossRefPubMedGoogle Scholar
  52. 52.
    Smith CS, Sheehy N, McEniff N, Keogan MT. Magnetic resonance portal venography: Use of fast-acquisition true FISP imaging in the detection of portal vein thrombosis. Clin Radiol 2007;62:1180–8.CrossRefPubMedGoogle Scholar
  53. 53.
    Vlachogiannakos J, Patch D, Watkinson A, Tibballs J, Burroughs AK. Carbon-dioxide portography: An expanding role? Lancet 2000;355:987–8.CrossRefPubMedGoogle Scholar
  54. 54.
    Kreft B, Strunk H, Flacke S, Wolff M, Conrad R, Gieseke J, et al. Detection of thrombosis in the portal venous system: Comparison of contrast-enhanced MR angiography with intraarterial digital subtraction angiography. Radiology 2000;216:86–92.CrossRefPubMedGoogle Scholar
  55. 55.
    Dilawari JB, Chawla YK, Raju GS, Kaur U, Bambery P. Splenoportovenography in portal hypertension: A safe outpatient procedure. Can Assoc Radiol J 1990;41:146–8.PubMedGoogle Scholar
  56. 56.
    Kapoor S, Pal S, Sahni P, Chattopadhyay TK. Thromboelastographic evaluation of coagulation in patients with extrahepatic portal vein thrombosis and non-cirrhotic portal fibrosis: A pilot study. J Gastroenterol Hepatol 2009;24:992–7.CrossRefPubMedGoogle Scholar
  57. 57.
    Müller MC, Meijers JC, Vroom MB, Juffermans NP. Utility of thromboelastography and/or thromboelastometry in adults with sepsis: A systematic review. Crit Care 2014;18:R30.CrossRefPubMedPubMedCentralGoogle Scholar
  58. 58.
    Rossetto V, Spiezia L, Senzolo M, Rodriguez-Castro KI, Maggiolo S, Simioni P. Whole blood rotation thromboelastometry (ROTEM®) profiles in subjects with non-neoplastic portal vein thrombosis. Thromb Res 2013;132:e131–4.CrossRefPubMedGoogle Scholar
  59. 59.
    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;35:2510–20.CrossRefPubMedGoogle Scholar
  60. 60.
    de Franchis R. Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol 2005;43:167–76. Erratum in: J Hepatol 2005;43:547.Google Scholar
  61. 61.
    Chawla YK, Bodh V. Portal vein thrombosis. J Clin Exp Hepatol 2015;5:22–40.CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Manzano-Robleda Mdel C, Barranco-Fragoso B, Uribe M, Méndez-Sánchez N. Portal vein thrombosis: What is new? Ann Hepatol 2015;14:20–7.PubMedGoogle Scholar
  63. 63.
    Louvet A, Texier F, Dharancy S, Pruvot FR, Sergent G, Deltenre P, et al. Anticoagulation therapy may reverse biliary abnormalities due to acute portal thrombosis. Dig Dis Sci 2006;51:11–17.CrossRefPubMedGoogle Scholar
  64. 64.
    Hollingshead M, Burke CT, Mauro MA, Weeks SM, Dixon RG, Jaques PF. Transcatheter thrombolytic therapy for acute mesenteric and portal vein thrombosis. J Vasc Interv Radiol 2005;16:651–61.CrossRefPubMedGoogle Scholar
  65. 65.
    Uflacker R. Applications of percutaneous mechanical thrombectomy in transjugular intrahepatic portosystemic shunt and portal vein thrombosis. Teeh Vasc Interv Radiol 2003;6:59–69.CrossRefGoogle Scholar
  66. 66.
    Manzanet G, Sanjuán F, Orbis P, López R, Moya A, Juan M, et al. Liver transplantation in patients with portal vein thrombosis. Liver Transpl 2001;7:125–31.CrossRefPubMedGoogle Scholar
  67. 67.
    Lerut JP, Mazza D, van Leeuw V, Laterre PF, Donataccio M, de Ville de Goyet J, et al. Adult liver transplantation and abnormalities of splanchnic veins: Experience in 53 patients. Transpl Int 1997;10:125–32.CrossRefPubMedGoogle Scholar
  68. 68.
    Davidson BR, Gibson M, Dick R, Burroughs A, Rolles K. Incidence, risk factors, management, and outcome of portal vein abnormalities at orthotopic liver transplantation. Transplantation 1994;57:1174–7.CrossRefPubMedGoogle Scholar
  69. 69.
    Stieber AC, Zetti G, Todo S, Tzakis AG, Fung JJ, Marino I, et al. The spectrum of portal vein thrombosis in liver transplantation. Ann Surg 1991;213:199–206.CrossRefPubMedPubMedCentralGoogle Scholar
  70. 70.
    Zyromski NJ, Howard TJ. Acute superior mesenteric-portal vein thrombosis after pancreaticoduodenectomy: Treatment by operative thrombectomy. Surgery 2008;143:566–7.CrossRefPubMedGoogle Scholar
  71. 71.
    Adani GL, Baccarani U, Risaliti A, Sponza M, Gasparini D, Bresadola F, et al. Percutaneous transhepatic portography for the treatment of early portal vein thrombosis after surgery. Cardiovase Intervent Radiol 2007;30:1222–6.CrossRefGoogle Scholar

Copyright information

© T.K. Chattopadhyay 2015

Authors and Affiliations

  • Sahaj Rathi
    • 1
  • Radha K. Dhiman
    • 1
  1. 1.Department of HepatologyPostgraduate Institute of Medical Education and ResearchChandigarhIndia

Personalised recommendations