Extrahepatic Portal Hypertension and Portal Vein Thrombosis (Review)

Extrahepatic portal vein thrombosis is the second most frequent cause of portal hypertension after liver cirrhosis. Risk factors of the thrombosis are divided into systemic: hereditary and acquired thrombophilia (60–70%); and local: abdominal inflammation, trauma, surgery, etc. (20–40%). Acute thrombosis episode is often olygo- or asymptomatic and remains undiagnosed until the development of portal hypertension and its complications: gastroesophageal variceal bleeding, portal biliopathy, etc. Early onset of anticoagulation is recommended and should be prescribed at least for 3 months. In patients with portal cavernoma a long-term (even lifelong) anticoagulation is directed to prevent recurrence and extension of thrombosis, especially in case of thrombophilia. Portocaval shunting is the most radical way to correct portal hypertension and to deliver a patient from variceal bleedings and other complications of the disease. Hepatic encephalopathy is a rare
condition and generally subclinical. Endoscopic band ligation and sclerotherapy are effective in acute variceal bleeding, primary and secondary prophylaxis of bleeding and should be supplied by non-selective β-adrenoblockers administration. Patients with extrahepatic portal vein thrombosis are well tolerated to variceal bleeding episodes and have high rates of survival during long-term follow-up due to normal liver function. Mortality in these patients is low (5-year mortality is less than 10%) and generally is associated with comorbidities rather complications of portal hypertension

1. Khanna R., Sarin S.K. Non-cirrhotic portal hypertension – diagnosis and management. J. Hepatol. 2014; 60 (2): 421–441. doi: 10.1016/j.jhep.2013.08.013.

2. De Franchis R., Baveno V Faculty: Bosch J., Burroughs A.K., D’Amico G., de Franchis R., Garcia-Tsao G., Grace N.D., Groszmann R.J., Lebrec D., Merkel C., Primignani M., Salerno F., Sarin S.K., Sorensen T.I. 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. doi: 10.1016/j.jhep.2010.06.004.

3. Ерамишанцев А.К. Первичная внепеченочная портальная гипертензия и ее хирургическое лечение: дис. … докт. мед. наук. М., 1983. 325 с. Eramishantsev A.K. Pervichnaya vnepechenochnaya portal’naya gipertenziya i ieyo khirurgicheskoe lechenie [Primary extrahepatic portal hypertension and its surgical treatment: dis. … doct. of med. sci.]. Moscow, 1983. 325 p. (In Russian)

4. Chawla Y., Duseja A., Dhiman R.K. Review article: the modern management of portal vein thrombosis. Aliment. Pharmacol. Ther. 2009; 30 (9): 881–894. doi: 10.1111/j.1365-2036.2009.04116.x.

5. De Leve L.D., Valla D.C., Garcia-Tsao G. Vascular disorders of the liver. Hepatology. 2009; 49 (5): 1729–1764. doi: 10.1002/hep.22772.

6. De Stefano V., Za T., Ciminello A., Betii S., Rossi E. Causes of splanchnic vein thrombosis in the Mediterranean area. Mediterr. J. Hematol. Infect. Dis. 2011; 3 (1): e2011063. doi: 10.4084/MJHID.2011.063. Published online: http://europepmc.org/articles/PMCID 3248340.

7. Garbuzenko D.V. Portal hypertension – causes and complications. Rijeca. Croatia: InTech., 2012. 146 p.

8. Фандеев Е.Е. Лечебная тактика при внепеченочной портальной гипертензии, развившейся вследствие тромбоза воротной вены: дис. … канд. мед. наук. М., 2014. 177 с. Fandeyev E.E. Lechebnaya taktika pri vnepechenochnoy portal’noy gipertenzii, razvivsheysya vsledstvie tromboza vorotnoy veni [Management of extrahepatic portal hypertension caused by portal vein thrombosis: dis. … cand. med. sci.]. Moscow, 2014. 177 p. (In Russian)

9. Ogren M., Bergqvist D., Bjorck M., Acosta S., Eriksson H., Strenby N.H. 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. doi: 10.3748/wjg.v12.i13.2115.

10. Шерцингер А.Г., Киценко Е.А., Любивый Е.Д., Фандеев Е.Е., Сысоева Е.П. Тромбоз воротной вены: этиология, диагностика и особенности лечения. Вестник экспериментальной и клинической хирургии. 2012; 1 (5): 83–91. Shertsinger A.G., Kitsenko E.A., Lyubiviy E.D., Fandeyev E.E., Sysoeva E.P. Portal vein thrombosis: etiology, diagnostics and treatment features. Vestnik eksperimental’noy i klinicheskoy khirurgii. 2012; 1 (5): 83–91. (In Russian)

11. Amitrano L., Guardascione M.A., Scaglione M., Pezzullo L., Sangiuliano N., Armellino M.F., Manguso F., Margaglione M., Ames P.R.J., Iannaccone L., Grandone E., Romano L., Balzano A. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am. J. Gastroenterol. 2007; 102 (11): 2464–2470. doi:10.1111/j.1572-0241.2007.01477.x.

12. Garcia-Pagan J.C., Hernandes-Guerra M., Bosch J. Extrahepatic portal vein thrombosis. Semin. Liver Dis. 2008; 28 (3): 282–292. doi: 10.1055/s-0028-1085096. Epub 2008 Sep 23.

13. Hoekstra J., Janssen H.L. Vascular liver disorders (II): portal vein thrombosis. Neth. J. Med. 2009; 67 (2): 46–53.

14. Poddar U., Borkar V. Management of extra hepatic portal venous obstruction (EHPVO): current strategies. Trop. Gastroenterol. 2011; 32 (2): 94–102.

15. Sarin S.K., Kumar A., Angus P.W., Baijal S.S., Baik S.K., Bayraktar Y., Chawla Y.K., Choudhuri G., Chung J.W., de Franchis R., de Silva H.J., Garg H., Garg P.K., Helmy A., Hou M.C., Jafri W., Jia J.D., Lau G.K., Li C.Z., Lui H.F., Maruyama H., Pandey C.M., Puri A.S., Rerknimitr R., Sahni P., Saraya A., Sharma B.C., Sharma P., Shiha G., Sollano J.D., Wu J., Xu R.Y., Yachha S.K., Zhang C. Diagnosis and management of acute variceal bleeding: Asian Pacific association for study of the liver recommendations. Hepatol. Int. 2011; 5 (2): 607–624. doi: 10.1007/s12072-010-9236-9.

16. Spaander M.C. Portal vein thrombosis in non cirrhotic patients. Rotterdam. Netherlands.: Optima Grafische Comm., 2010. 108 p.

17. Spaander M.C., van Buuren H.R., Janssen H.L. Non-cirrhotic non-malignant portal vein thrombosis: a review on management of non-cirrhotic non-malignant portal vein thrombosis and the concurrent portal hypertension complications in adults. Aliment. Pharmacol. Ther. 2007; 26 (2): 203–209. doi: 10.1111/j.1365-2036.2007.03488.x.

18. Wani H., Ahmed B., Wani G., Masoodi I., Mustafa S. Extrahepatic portal vein obstruction (EHVPO). Internet J. Gastroenterol. 2010; 9 (1): 695. Published online: http://ispub.com/IJGE/9/1/11432.

19. Ponziani F.R., Zocco M.A., Campanale C., Rinninella E., Tortora A., Di Maurizio L., Bombardieri G., De Cristofaro R., De Gaetano A.M., Landolfi R., Gasbarrini A. Portal vein thrombosis: insight into physiology, diagnosis, and treatment. World J. Gastroenterol. 2010; 16 (2): 143–155. doi: 10.3748/wjg.v16.i2.143.

20. Разумовский А.Ю., Рачков В.Е. Хирургическое лечение портальной гипертензии у детей. М.: Медицинское информационное агентство, 2012. 480 с. Razumovskiy A.Y., Rachkov V.E. Khirurgicheskoye lechenie portal’noy gipertenzii u detei [Surgical management of portal hypertension in children]. Moscow: Meditsinskoye informatsionnoye agentstvo, 2012. 480 p. (In Russian)

21. Morag I., Epelman M., Daneman A., Moineddin R., Parvez B., Shechter T., Hellmann J. Portal vein thrombosis in the neonate: risk factors, course, and outcome. J. Pediatr. 2006; 148 (6): 735–739. doi: 10.1016/j.jpeds.2006.01.051.

22. Weiss B., Shteyer E., Vivante A., Berkowitz D., Reif S., Weizman Z., Bujanover Y., Shapiro R. Etiology and long-term outcome of extrahepatic portal vein obstruction in children. World J. Gastroenterol. 2010; 16 (39): 4968–4972. doi: 10.3748/wjg.v16.i39.4968.

23. Janssen H.L., Wijnhoud A., Haagsma E.B., van Uum S.H., van Nieuwkerk C.M., Adang R.P., Chamuleau R.A., van Hattum J., Vlegaar F.P., Hansen B.E., Rosendaal F.R., van Hoek B. Extrahepatic portal vein thrombosis: aetiology and determinants of survival. Gut. 2001; 49 (5): 720–724. doi: 10.1136/gut.49.5.720.

24. Plessier A., Murad S.D., Hernandez-Guerra M., Consigny Y., Fabris F., Trebicka J., Heller J., Morard I., Lasser L., Langlet P., Denninger M.H., Vidaud D., Condat B., Hadengue A., Primignani M., Garcia-Pagan J.C., Janssen H.L., Valla D. Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. Hepatology. 2010; 51 (1): 210–218. doi: 10.1002/hep.23259.

25. Senzolo M., Riggio O., Primignani M., Italian Association for the Study of the Liver (AISF) ad hoc.: Marino L.B., Crema E.B., de Feo T., De Santis A., Fabris F.M., Guido M., Maggi U., Martinelli I., Merli M., Nicolini A., Rossi G., Sabba C., Sacerdoti D., Salerno F., Senzolo M., Vizzutti F. Vascular disorders of the liver: Recommendations from the Italian association for the study of the liver (AISF) ad hoc committee. Dig. Liver Dis. 2011; 43 (7): 503–514. doi: 10.1016/j.dld.2010.11.006.

26. Sogaard K.K., Astrup L.B., Vilstrup H., Gronbaek H. Portal vein thrombosis. Risk factors, clinical presentation and treatment. BMC Gastroenterol. 2007; 7: 34. doi: 10.1186/1471-230X-7-34.

27. Primignani M., Mannucci M. The role of thrombophilia in splanchnic vein thrombosis. Semin. Liver Dis. 2008; 28 (3): 293–301. doi: 10.1055/s-0028-1085097.

28. Dentali F., Galli M., Gianni M., Ageno W. Inherited thrombophilic abnormalities and risk of portal vein thrombosis. A meta-analysis. Thromb. Haemost. 2008; 99 (4): 675–682. doi: 10.1160/TH07-08-0526.

29. Sakha S.H., Rafeey M., Tarzamani M.K. Portal venous thrombosis after umbilical vein catheterization. Indian J. Gastroenterol. 2007; 26 (6): 283–284.

30. Ferri P.M., Rodrigues Ferreira A., Fagundes E.D., Xavier S.G., Dias R.D. Evaluation of the presence of hereditary and acquired thrombophilias in brazilian children and adolescents with diagnoses of portal vein thrombosis. J. Pediatr. Gastroenterol. Nutr. 2012; 55 (5): 599–604. doi: 10.1097/MPG.0b013e318261814d.

31. Qi X., De Stefano V., Wang J., Bai M., Yang Z., Han G., Fan D. Prevalence of inherited antithrombin, protein C, and protein S deficiencies in portal vein system thrombosis and Budd-Chiari syndrome: a systematic review and meta-analysis of observational studies. J. Gastroenterol. Hepatol. 2013; 28 (3): 432–442. doi: 10.1111/jgh.12085.

32. Sutkowska E., McBane R.D., Tafur A.J., Sutkowski K., Grill D.E., Slusser J.P., Wysokinski W.E. Thrombophilia differences in splanchnic vein thrombosis and lower extremity deep venous thrombosis in North America. J. Gastroenterol. 2013; 48 (10): 1111–1118. doi: 10.1007/s00535-012-0728-3.

33. Киценко Е.А., Любивый Е.Д., Лукина Е.А., Коршунов И.Б. Тромбофилия как причина внепеченочной портальной гипертензии. Анналы хирургической гепатологии. 2007; 12 (2): 26–30. Kitsenko E.A., Lyubiviy E.D., Lukina E.A., Korshunov I.B. Thrombophilia as a cause of extrahepatic portal hypertension. Annaly khirurgicheskoy gepatologii. 2007; 12 (2): 26–30. (In Russian)

34. Kobayashi S., Ng C.S., Kazama T., Madoff D.C., Faria S.C., Vauthey J.N., Charnsangavej C. Hemodynamic and morphologic changes after portal vein embolization: differential effects in central and peripheral zones in the liver on multiphasic computed tomography. J. Comput. Assist. Tomogr. 2004; 28 (6): 804–810.

35. Tublin M.E., Towbin A.J., Federle M.P., Nalesnik M.A. Altered morphology after portal vein thrombosis: not always cirrhosis. Dig. Dis. Sci. 2008; 53 (10): 2784–2788. doi: 10.1007/s10620-008-0201-6.

36. Mesa R.A., Nagorney D.S., Schwager S., Allred J., Tefferi A. Palliative goals, patient selection, and preoperative platelet mana gement. Outcomes and lessons from 3 decades of splenectomy for myelofibrosis with myeloid metaplasia at the Mayo clinic. Cancer. 2006; 107 (2): 361–370. doi: 10.1002/cncr.22021.

37. Kraemer D., Rudiger T., Reimer P., Muller-Hermelink H.K., Wilhelm M. Splenectomy in patients with mixed myelodysplastic / myeloproliferative disease. Ann. Hematol. 2002; 81 (6): 308–311.

38. Дмитренко Е.В. Лечение и профилактика кровотечений из варикозно расширенных вен пищевода и желудка у больных с внепеченочной портальной гипертензией и заболеваниями системы крови: дис. … канд. мед. наук. М., 2009. 158 с. Dmitrenko E.V. Lechenie i profilaktika krovotecheniy iz varikozno rasshirennikh ven pischevoda i geludka u bol’nikh s vnepechenochnoy portal’noy gipertenziyey i zabolevaniyami sistemi krovi [Management and prevention of bleedings from esophageal and gastric varices in patients with extrahepatic portal hypertension and myeloproliferative diseases: dis. … cand. med. sci.] Moscow, 2009. 158 p. (In Russian)

39. Ma J., Luo J., Liu Q., Wang J., Qui S. Rational classification of portal vein thrombosis and its clinical significance. PloS One. 2014; 9 (11): e112501. doi: 10.1371/journal.pone.0112501.

40. Bittencourt P.L., Farias A.Q., Strauss E., Mattos A.A., Pannel of the 1st Brazilian Concensus of Variceal Bleeding, Brazilian society of Hepatology: Ribeiro A.V., Brandao A.B., Lyra A., Cavalcanti A.R., Dominici A., Terra C., Marroni C.A., Tovo C.V., Crespo D., Parise E.R., Marinho F., Carrilho F.J., Souto F.J., Cotrim H., Coelho H.S., Paes I., Galizzi J., Soares J.S., Silva L., Lyra L.G., Costa M.A., Herman P., Mascarenhas R., Parana R. Variceal bleeding: Consensus meeting report from the Brazilian Society of Hepatology. Arq. Gastroenterol. 2010; 47 (2): 202–216. doi: 10.1590/S0004-28032010000200017.

41. Sheen C.L., Lamparelli H., Milne A., Green I., Ramage J.K. Clinical features, diagnosis and outcome of acute portal vein thrombosis. QJM. 2000; 93 (8): 531–534. doi: 10.1093/qjmed/93.8.531.

42. Harmanci O., Bayraktar Y. Portal hypertension due to portal venous thrombosis: etiology, clinical outcomes. World J. Gastroenterol. 2007; 13 (18): 2535–2540. doi: 10.3748/wjg.v13.i18.2535.

43. Khare R., Sikora S.S., Srikanth G., Choudhuri G., Saraswat V.A., Kumar A., Saxena R., Kapoor V.K. Extrahepatic portal venous obstruction and obstructive jaundice: approach to management. J. Gastroenterol. Hepatol. 2005; 20 (1): 56–61. doi: 10.1111/j.1440-1746.2004.03528.x.

44. Mutignani M., Shah S.K., Bruni A., Perri V., Costamagna G. Endoscopic treatment of extrahepatic bile duct strictures in patients with portal biliopathy carries a high risk of haemobilia: report of 3 cases. Dig. Liver Dis. 2002; 34 (8): 587–591.

45. Nagi B., Kochhar R., Bhasin D., Singh K. Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances. Acta Radiol. 2000; 41 (6): 612–615. doi: 10.1080/028418500127345992.

46. Dumortier J., Vaillant E., Boillot O., Poncet G., Henry L., Scoazec J.Y., Partensky C., Valette P.J., Paliard P., Ponchon T. Diagnosis and treatment of biliary obstruction caused by portal cavernoma. Endoscopy. 2003; 35 (5): 446–450. doi: 10.1055/s-2003-38779.

47. Sharma M., Pathak A. Intracholedochal varices in portal hypertensive biliopathy. Eur. J. Radiol. 2009; Extra 72: e119–e123.

48. Vibert E., Azoulay D., Aloia T., Pascal G., Veilhan L.A., Adam R., Samuel D., Castaing D. Therapeutic strategies in symptomatic portal biliopathy. Ann. Surg. 2007; 246 (1): 97–104. doi: 10.1097/SLA.0b013e318070cada.

49. Colle I., van Vlierberghe H., Pattyn P., Troisi R., Vogelaers D., De Hemptinne B., De Vos M. Cholestasis as presenting symptom of portal cavernoma. Hepatol. Res. 2003; 25 (1): 32–37. doi: 10.1016/S1386-6346(02)00172-9.

50. Dhiman R.K., Behera A., Chawla Y.K., Dilawari J.B., Suri S. Portal hypertensive biliopathy. Gut. 2007; 56 (7): 1001–1008. doi: 10.1136/gut.2006.103606.

51. Sezgin O., Oguz D., Attritas E., Saritas U., Sahin B. Endoscopic management of biliary obstruction carried by cavernous transformation of portal vein. Gastrointest. Endosc. 2003; 58 (4): 602–608. doi: 10.1067/S0016-5107(03)01975-8.

52. Nihal N., Bapat M.R., Rathi P., Shah N.S., Karvat A., Abraham P., Bhatia S.J. Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction. Hepatol. Int. 2009; 3 (1): 305–309. doi: 10.1007/s12072-008-9102-1.

53. Krebs-Schmitt D., Briem-Richter A., Grabhorn E., Burdelski M., Helmke K., Broering D.C., Ganschow R. Effectiveness of Rex shunt in children with portal hypertension following liver transplantation or with primary portal hypertension. Pediatr. Trans plant. 2009; 13 (5): 540–544. doi: 10.1111/j.1399-3046.2008.01109.x.

54. Камалов Ю.Р. Абдоминальное ультразвуковое исследование при синдроме портальной гипертензии. Методическое пособие. М., 2004. 48 с. Kamalov Y.R. Abdominal’noe ul’trazvukovoye issledovanie pri sindrome portal’noy gipertenzii. Metodicheskoye posobie [Abdominal ultrasonic in syndrome of portal hypertension. Methodical guideline]. Moscow, 2004. 48 p. (In Russian)

55. Камалов Ю.Р., Лебезев В.М., Ховрин В.В., Крюкова И.Е., Ким С.Ю., Гаврилов А.В., Архипов И.В. Возможности трехмерной реконструкции компьютерно-томографичеких изображений при дооперационной оценке состояния пациентов с синдромом портальной гипертензии. Медицинская визуализация. 2013; 3: 132–133. Kamalov Yu.R., Lebezev V.M., Khovrin V.V., Kryukova I.E., Kim S.Yu., Gavrilov A.V., Arkhipov I.V. The possibilities of 3D-reconstruction of computed-tomography images in preoperative evaluation of patients’ condition with portal hypertension syndrome. Meditsinskaya vizualizatsiya. 2013; 3: 132–133. (In Russian)

56. Basit S.A., Stone C.D., Gish R. Portal vein thrombosis. Clin. Liver Dis. 2015; 19 (1): 199–221. doi: 10.1016/j.cld.2014.09.012.

57. Lee H.K., Park S.J., Yi B.H., Yeon E.K., Kim J.H., Hong H.S. Portal vein thrombosis: CT features. Abdomin. Imaging. 2008; 33 (1): 72–79. doi: 10.1007/s00261-007-9200-x.

58. Spaander M.C., van Buuren H.R., Hansen B.E., Janssen H.L. Ascites in patients with non-cirrhotic non-malignant extrahepatic portal vein thrombosis. Aliment. Pharmacol. Ther. 2010; 32 (4): 529–534. doi: 10.1111/j.1365-2036.2007.03488.x.

59. Sharma P., Mishra S.R., Kumar M., Sharma B.C., Sarin S.K. Liver and spleen stiffness in patients with extrahepatic portal vein obstruction. Radiology. 2012; 263 (3): 893–899. doi: 10.1148/radiol.12111046.

60. Жигалова С.Б. Эндоскопические технологии в лечении и профилактике кровотечений из варикозно расширенных вен пищевода и желудка у больных портальной гипертензией: автореф. дис. … докт. мед. наук. М., 2011. 50 с. Zhigalova S.B. Endoskopicheskie tekhnologii v lechenii i profilaktike krovotecheniy iz varikozno rasshirennikh ven pischevoda i geludka u bol’nikh s portal’noy gipertenziyey [Endoscopic technologies for management and prevention of bleeding from esophageal and gastric varices in patients with portal hypertension: autoabstr. dis. … doc. med. sci.]. Moscow, 2011. 50 p. (In Russian)

61. Sato T., Akaike J., Toyota J., Karino Y., Ohmura T. Clinicopathological features and treatment of ectopic varices with portal hypertension. International J. Hepatol. 2011; 2011: 960720. doi: 10.4061/2011/960720.

62. Seeger M., Gunther R., Hinrichsen H., Both M., Helwig U., Arlt A., Stelck B., Brasen J.H., Sipos B., Schafmayer C., Braun F., Broring D.C., Schreiber S., Hampe J. Chronic portal vein thrombosis: transcapsular hepatic collateral vessels and communicating ectopic varices. Hampe Radiology. 2010; 257 (2): 568–578. doi: 10.1148/radiol.10100157.

63. De Franchis R., Dell’Era A., Primignani M. Diagnosis and monitoring of portal hypertension. Dig. Liver Dis. 2008; 40 (5): 312–317. doi: 10.1016/j.dld.2007.12.007.

64. Misra S.P., Dwivedi M., Misra V., Dharmani S., Kunwar B.K., Arora J.S. Colonic changes in patients with cirrhosis and in patients with extrahepatic portal vein obstruction. Endoscopy. 2005; 37 (5): 454–459. doi: 10.1055/s-2005-861252.

65. Ganguly S., Sarin S.K., Bhatia V., Lahoti D. The prevalence and spectrum of colonic lesions in patients with cirrhosis and noncirrhotic portal hypertension. Hepatology. 1995; 21 (5): 1226–1231.

66. Mustjoki S., Borze I., Lasho T.L., Alitalo R., Pardanani A., Knuutila S., Juvonen E. JAK2V617F mutation and spontaneous megakaryocytic or erythroid colony formation in patients with essential thrombocythaemia (ET) or polycythaemia vera (PV). Leuk. Res. 2009; 33 (1): 54–59. doi: 10.1016/j.leukres.2008.07.008.

67. Turnes J., Garcia-Pagan J.C., Gonzalez M., Aracil C., Calleja J.L., Ripoll C., Abraldes J.G., Banares R., Villanueva C., Albillos A., Ayuso J.R., Gilabert R., Bosch J. Portal hypertensive complications after acute portal vein thrombosis: impact of early anticoagulation. Clin. Gastroenterol. Hepatol. 2008; 6 (12): 1412–1417. doi: 10.1016/j.cgh.2008.07.031.

68. Tateishi A., Mitsui H., Oki T., Morishita J., Maekawa H., Yahagi N., Maruyama T., Ichinose M., Ohnishi S., Shiratori Y., Minami M., Koutetsu S., Hori N., Watanabe T., Nagawa H., Omata M. Extensive mesenteric vein and portal vein thrombosis successfully treated by thrombolysis and anticoagulation. J. Gastroenterol. Hepatol. 2001; 16 (12): 1429–1433. doi: 10.1046/j.1440-1746.2001.02557.x.

69. Condat B., Pessione F., Hillaire S., Denninger M.H., Guillin M.C., Poliquin M., Hadengue A., Erlinger S., Valla D. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology. 2001; 120 (2): 490–497. doi: 10.1053/gast.2001.21209.

70. Congly S.E., Lee S.S. Portal vein thrombosis: should anticoagulation be used? Curr. Gastroenterol. Rep. 2013; 15 (2): 306. doi: 10.1007/s11894-012-0306-0.

71. Franchini M., Mannicci P.M. A new era of anticoagulants. Eur. J. Intern. Med. 2009; 20 (6): 562–568. doi: 10.1016/j.ejim.2009.05.004.

72. Tripodi A., Mannucci P.M. The coagulopathy of chronic liver disease. N. Engl. J. Med. 2011; 365 (2): 147–156. doi: 10.1056/NEJMra1011170.

73. Adani G.L., Baccarani U., Risaliti A., Sponza M., Gasparini D., Bresadola F., de Anna D., Bresadola V. Percutaneous transhepatic portography for the treatment of early portal vein thrombosis after surgery. Cardiovasc. Intervent. Radiol. 2007; 30 (6): 1222–1226. doi: 10.1007/s00270-007-9056-z.

74. Aytekin C., Boyvat F., Kurt A., Yologlu Z., Coskun M. Catheter directed thrombolysis with transjugular access in portal vein thrombosis secondary to pancreatitis. Eur. J. Radiol. 2001; 39 (2): 80–82. doi: 10.1016/S0720-048X(01)00289-3.

75. Bilbao J.I., Elorz M., Vivas I., Martinez-Cuesta A., Bastarrika G., Benito A. Transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of venous symptomatic chronic portal thrombosis in non-cirrhotic patients. Cardiovasc. Intervent. Radiol. 2004; 27 (5): 474–480. doi: 10.1007/s00270-004-0241-z.

76. Ferro C., Rossi C.G., Bovio G., Dahamane M., Centenaro M. TIPS mechanical aspiration thrombectomy and direct thrombolysis in the treatment of acute portal and superior mesenteric vein thrombosis. Cardiovasc. Intervent. Radiol. 2007; 30 (5): 1070–1074. doi: 10.1007/s00270-007-9137-z.

77. Henao E.A., Bohannon W.T., Silva M.B. Jr. Treatment of portal venous thrombosis with selective superior mesenteric artery infusion of recombinant tissue plasminogen activator. J. Vasc. Surg. 2003; 38 (6): 1411–1415.

78. Hollingshead M., Burke C.T., Mauro M.A., Weeks S.M., Dixon R.G., Jaques P.F. Transcatheter thrombolytic therapy for acute mesenteric and portal vein thrombosis. J. Vasc. Intervent. Radiol. 2005; 16 (5): 651–661.

79. Kercher K.W., Sing R.F., Watson K.W., Matthews B.D., LeQuire M.H., Heniford B.T. Transhepatic thrombolysis in acute portal vein thrombosis after laparoscopic splenectomy. Surg. Laparosc. Endosc. Percutan. Tech. 2002; 12 (2): 131–136.

80. Malkowski P., Pawlak J., Michalowicz B., Szczerban J., Wroblewski T., Leowska E., Krawczyk M. Thrombolytic treatment of portal thrombosis. Hepatogastroenterology. 2003; 50 (54): 2098–2100.

81. Oguzkurt P., Tercan F., Ince E., Ezer S.S., Hicsonmez A. Percutaneous treatment of portal vein thrombosis in a child who has undergone splenectomy. J. Pediatr. Surg. 2008; 43 (8): 29–32.

82. Pfammatter T., Binoit C., Cathomas G., Blum U. Budd–Chiari synd rome with spleno-mesenteric-portal thrombosis: treatment with extended TIPS. J. Vasc. Interv. Radiol. 2000; 11 (6): 781–784.

83. Senzolo M., Tibbals J., Cholongitas E., Triantos C.K., Burroughs A.K., Patch D. Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation. Aliment. Pharmacol. Ther. 2006; 23 (6): 767–775. doi: 10.1111/j.1365-2036.2006.02820.x.

84. Smalberg J.H., Spaander M.C., Kon-Siong G.J., Pattynama P.M.T., van Buuren H.R., van den Berg B., Janssen H.L., Leebeek F.W.G. Risk and benefits of transcatheter thrombolytic therapy in patients with splanchnic venous thrombosis. Thromb. Haemost. 2008; 100 (6): 1084–1088. doi: 10.1160/TH08-01-0015.

85. Uflacker R. Applications of percutaneous mechanical thrombectomy in transjugular intrahepatic portosystemic shunt and portal vein thrombosis. Tech. Vasc. Interv. Radiol. 2003; 6 (1): 59–69. doi: 10.1053/tvir.2003.36433.

86. Boyer T.D., Henderson J.M., Heerey A.M., Arrigain S., Konig V., Connor J., Abu-Elmagd K., Galloway J., Rikkers L.F., Jeffers L. Cost of preventing variceal rebleeding with transjugular intrahepatic portal systemic shunt and distal splenorenal shunt. J. Hepatol. 2008; 48 (3): 407–414. doi: 10.1016/j.jhep.2007.08.014.

87. Лебезев В.М. Портокавальное шунтирование у больных с портальной гипертензией: дис. … докт. мед. наук. М., 1994. 213 с. Lebezev V.M. Portokaval’noe shuntirovanie u bol’nikh s portal’noy gipertenziyey [Portocaval shunting in patients with portal hypertension: dis. … doct. med. sci.]. Moscow, 1994. 213 p. (In Russian)

88. Pal S., Sahni P. Extrahepatic portal venous obstruction: Is the knife irrelevant? Indian J. Gastroenterol. 2009; 28 (6): 198–200. doi: 10.1007/s12664-009-0078-0.

89. Livingstone A.S., Koniaris L.G., Perez E.A., Alvarez N., Levi J.U., Hutson D.G. 507 Warren–Zeppa distal splenorenal shunts: a 34-year experience. Ann. Surg. 2006; 243 (6): 884–892. doi: 10.1097/01.sla.0000219681.08312.87.

90. Orloff M.J., Isenberg J.I., Wheeler H.O., Haynes K.S., Jinich-Brook H., Rapier R., Vaida F., Hye R.J. Direct costs of care in a randomized controlled trial of endoscopic sclerotherapy versus emergency portocaval shunt for bleeding esophageal varices in cirrhosis – Part 4. J. Gastrointest. Surg. 2010; 15 (1): 38–47. doi: 10.1007/s11605-010-1332-6.

91. Pedroso F.E., Koniaris L.G. Shunting: a better way to prevent variceal bleeding. J. Surg. Res. 2011; 167 (1): e1–e3. doi: 10.1016/j.jss.2010.12.037.

92. Wolff M., Hirner A. Current state of portosystemic shunt surgery. Langenbecks Arch. Surg. 2003; 388 (3): 141–149. doi: 10.1007/s00423-003-0367-5.

93. Wu J., Li Z., Wang Z., Han X., Ji F., Zhang W.W. Surgical and endovascular treatment of severe complications secondary to noncirrhotic portal hypertension: experience of 56 cases. Ann. Vasc. Surg. 2013; 27 (4): 441–446. doi: 10.1016/j.avsg.2012.05.027.

94. Di Francesco F., Grimaldi C., de Ville de Goyet J. Meso-Rex bypass – a procedure to cure prehepatic portal hypertension: the insight and the inside. J. Am. Coll. Surg. 2014; 218 (2): e23–36. doi: 10.1016/j.jamcollsurg.2013.10.02.

95. Query J.A., Sandler A.D., Sharp W.J. Use of autogenous saphenous vein as a conduit for mesenterico-left portal vein bypass. J. Pediatr. Surg. 2007; 42 (6): 1137–1140. doi: 10.1016/j.jpedsurg.2007.01.066.

96. Superina R., Shneider B., Emre S., Sarin S.K., de Ville de Goyet J. Surgical guidelines for the management of extra-hepatic portal vein obstruction. Pediatr. Transplant. 2006; 10 (8): 908–913. doi: 10.1111/j.1399-3046.2006.00598.x.

97. Srivastava A., Yadav S.K., Lal R., Yachha S.K., Thomas M.A., Saraswat V.A., Gupta R.K. Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry. J. Pediatr Gastroenterol. Nutr. 2010; 51 (6): 766–772. doi: 10.1097/MPG.0b013e3181ecaf62.

98. Sharma P., Sharma B.C. Lactulose for minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Saudi J. Gastroenterol. 2012; 18 (3): 168–172. doi: 10.4103/1319-3767.96448.

99. Goyal N., Singhal D., Gupta S., Soin A.S., Nundy S. Transabdo minal gastroesophageal devascularization without transection for bleeding varices: results and indicators of prognosis. J. Gastroenterol. Hepatol. 2007; 22 (1): 47–50. doi: 10.1111/j.1440-1746.2006.04330.x.

100. Johnson M., Rajendran S., Balachandar T.G., Kannan D., Jeswanth S., Ravichadran P., Surendran R. Transabdominal modified devascularization procedure with or without esophageal stapler transection – an operation adequate for effective control of a variceal bleed. Is esophageal stapler transection necessary? World J. Surg. 2006; 30 (8): 1507–1519. doi: 10.1007/s00268-005-0754-x.

101. Singhal D., Goyal N., Soin A.S., Gupta S., Nundy S. What are the essential components of a transabdominal devascularization procedure for bleeding esophageal varices? World J. Surg. 2007; 31 (4): 861–864. doi: 10.1007/s00268-007-0630-y.

102. Covarelli P., Badolato M., Boselli C., Noya G., Cristofani R., Mosca S., Tei F. Splenic vein thrombosis complicated by massive gastric bleeding: treatment with arterious embolization. Am. Surg. 2008; 74 (2): 184–186.

103. Saugel B., Gaa J., Phillip V., Schmid R.M., Huber W. Splenic artery embolization in a woman with bleeding gastric varices and splenic vein thrombosis: a case report. J. Med. Case Rep. 2010; 4: 247. doi: 10.1186/1752-1947-4-247.

104. Takahashi T., Yoshida H., Mamada Y., Taniani N., Tajiri T. Balloon-occluded retrograde transvenous obliteration for gastric varices in a child with extrahepatic portal vein obstruction. J. Nippon Med. Sch. 2009; 76 (3): 173–178. doi: 10.1272/jnms.76.173.

105. Spaander M.C., Murad S.D., van Buuren H.R., Hansen B.E., Kuipers E.J., Janssen H.L. Endoscopic treatment of esophagogastric variceal bleeding in patients with non-cirrhotic extrahepatic portal vein thrombosis: a long-term follow-up study. Gastrointest. Endosc. 2008; 67 (6): 821–827. doi: 10.1016/j.gie.2007.08.023.

106. Thomas V., Jose T., Kumar S. Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction. A 20-year follow-up. Indian J. Gastroenterol. 2009; 28 (6): 206–211. doi: 10.1007/s12664-009-0086-0.

107. Poddar U., Bhatnagar S., Yachha S.K. Endoscopic band ligation followed by sclerotherapy: is it superior to sclerotherapy in children with extrahepatic portal venous obstruction? J. Gastroenterol. Hepatol. 2011; 26 (2): 255–259. doi: 10.1111/j.1440-1746.2010.06397.x.

108. Sarin S.K., Gupta N., Jha S.K., Agrawal A., Mishra S.R., Sharma B.C., Kumar A. Equal efficacy of endoscopic variceal ligation and propranolol in preventing variceal bleeding in patients with noncirrhotic portal hypertension. Gastroenterology. 2010; 139 (4): 1238–1245. doi: 10.1053/j.gastro.2010.06.017.

109. Zargar S.A., Javid G., Khan B.A., Shah O.J., Yattoo G.N., Shah A.H., Gulzar G.M., Singh J., Shah N.A., Shafi H.M. Endoscopic ligation vs. sclerotherapy in adults with extrahepatic portal venous obstruction: a prospective randomized study. Gastrointest. Endosc. 2005; 61 (1): 58–66. doi: 10.1016/S0016-5107(04)02455-1.

110. Pavini М.Т., Puyana J.C. Gastroesophageal balloon tamponade for acute variceal hemorrhage. Irwin and Rippe’s Intensive Care Medicine. 6-th edition. Edited by: Rippe J.M. Philadelphia. USA.: Lippincot Williams & Wilkins, 2008. P. 138–145.

111. Bosch J., Garcia-Pagan J.C., Feu F., Luca A., Fernandez M., Pizcueta P., Rodes J. New approaches in the pharmacologic treatment of portal hypertension. J. Hepatol. 1993; 17 (2): 41–45.

112. Burroughs A.K., Panagou E. Pharmacological therapy for portal hypertension: rationale and results. Semin. Gastrointest. Dis. 1995; 6 (3): 23–27.

113. Burroughs A.K., McCormick P.A., Hughes M.D., Sprengers D., D’Heygere F., McIntyre N. Randomized double blind placebo controlled trial of somatostatin for variceal bleeding. Emergency control and prevention of early variceal bleeding. Gastroenterology. 1990; 99 (5): 1388–1395.

114. De La Pena J., Brullet E., Sanchez-Hernandez E., Rivero M., Vergara M., Martin-Lorente J.L., Garcia Suarez C. Variceal ligation plus nadolol compared with ligation for prophylaxis of variceal bleeding: a multicentre trial. Hepatology. 2005; 41 (3): 572–578. doi: 10.1002/hep.20584.

115. Schepke M., Kleber G., Nurnberg D., Willert J., Koch L., Veltzke-Schlieker W., Hellerbrand C., Kuth J., Schanz S., Kahl S., Fleig W.E., Sauerbruch T. Ligation versus propranolol for the primary prophylaxis of variceal bleeding in cirrhosis. Hepatology. 2004; 40 (1): 65–72. doi: 10.1002/hep.20284.

116. Banares R., Albillos A., Rincon D., Alonso S., Gonzalez M., Ruiz-del-Arbol L., Salcedo M., Molinero L.M. Endoscopic treatment versus endoscopic plus pharmacologic treatment for acute variceal bleeding: a meta-analysis. Hepatology. 2002; 35 (3): 609–615.

117. Kitchens C.S., Weidner M.H., Lottenberg R. Chronic oral anticoagulant therapy for extrahepatic visceral thrombosis is safe. J. Thromb. Thrombolysis. 2007; 23 (3): 223–228. doi: 10.1007/s11239-006-9017-4.