Portal Vein Obstruction Treatment & Management
- Author: Adnan Said, MD, MSPH; Chief Editor: Julian Katz, MD more...
Medical Care
- Acute bleeding: The primary goals are to alleviate acute bleeding and to prevent further bleeding.
- In the acute setting, these goals are best accomplished with variceal banding or sclerotherapy, often requiring several sessions to obliterate the bleeding. This has a success rate of 95% for the acute bleed.
- Octreotide infusion has also been used in acute bleeding, with control of the acute bleed in 85% of patients. The rate of recurrent bleeding with this approach is 16-28%.
- In the setting of portal vein obstruction, the role of propranolol to prevent rebleeding has not been studied, though it is used routinely.
- Treatment of underlying etiology: Anticoagulation in patients with acute/recent portal vein thrombosis, studied only retrospectively, has been shown to recanalize in more than 80% of cases. This is essential to prevent advancement of thrombosis or rethrombosis in patients with inherited coagulation disorders in which lifelong anticoagulation therapy is recommended once variceal control has been achieved. Anticoagulation therapy has also been recommended after shunt surgery to prevent rethrombosis. There was a study in which 84 of 136 nonmalignant, noncirrhotic patients with portal vein thrombosis were anticoagulated with similar bleeding risks but less risk for thrombotic propagation. Debate remains regarding the risk-to-benefit ratio of anticoagulation in chronic portal vein thrombosis and should be decided on a case-by-case approach at this time.
- Thrombolysis: This approach is recommended in acute portal vein thrombosis through the transhepatic route, which avoids the need for systemic thrombolysis.
- Tissue-type plasminogen activator (tPA) has been used for this purpose, followed by prolonged anticoagulation therapy with Coumadin for at least 3 months (indefinitely in patients with inherited coagulation disorders).
- In the setting of acute portal vein thrombosis with symptoms, shunt surgery with subsequent anticoagulation therapy is an alternative.
Surgical Care
- Shunt surgery
- In portal vein obstruction, the place for shunt surgery in the treatment of variceal bleeding is debated. Some authors recommend endoscopic treatment and propranolol as first-line treatment to prevent recurrent bleeding. Others recommend shunt surgery after the first variceal bleed to prevent further rebleeding.
- In general, only attempt shunt surgery when endoscopic treatment fails.
- A distal splenorenal shunt is usually the preferred surgical shunt. For patients in whom the splenic vein is also thrombosed and surgery is undertaken, splenectomy and other shunt procedures (eg, the Sugiura procedure) have been performed. A more recent salvage operation showing success is the right and left mesogonadal shunt. In patients who are critically ill, esophagogastrectomies have been used as a last resort.
- In the presence of cirrhosis, the operative mortality rate has been reported to be 18%. In the absence of cirrhosis, operative mortality is approximately 2%. The postoperative complication rate is approximately 30%.
- The presence of liver nodules has been reported following portal systemic shunt surgery in animal models or in humans with liver cirrhosis. In a small retrospective study of 45 children without liver disease, Guerin et al examined the incidence of liver nodules following surgical intervention for extrahepatic portal vein obstruction.[9] Using ultrasonography, the investigators noted 7 (15%) of the children had liver nodules (median 80 months' follow-up), all of which occurred following portal systemic shunt surgery and 5 of which demonstrated either liver cell adenomas (2 nodules) or focal nodular hyperplasias (3 nodules).[9] Guerin et al recommended keeping in mind the possible presence of liver nodules during follow-up of children post portal systemic shunt surgery for extrahepatic portal vein obstruction.[9]
- TIPS: Previously considered a relative contraindication in portal vein thrombosis, TIPS has been successfully used in this condition. Stent placement requires an aspiration thrombectomy through a sheath with subsequent angioplasty of the tract and stent placement. Some centers have obtained good results by performing an embolectomy and then using local thrombolytic therapy through the TIPS after deployment.
- In portal vein obstruction, TIPS is indicated in uncontrollable variceal bleeding in a patient with cirrhosis, usually as a bridge to transplant. The choice of TIPS over shunt surgery depends upon the expertise of the center in these techniques and the distance from skilled health care because TIPS is more likely to occlude and require revision. However, TIPS has the advantage of being less invasive than shunt surgery.
- In the setting of portal vein obstruction and cirrhosis, TIPS has a success rate of 69% in controlling variceal bleeding and a complication rate of 22%, including a mortality rate of 11% in one series.
- Liver transplantation
- In patients referred for orthotopic liver transplantation (OLT), portal vein thrombosis complicates 5-15% of cases.
- Although traditionally viewed as a relative contraindication to OLT, recent innovative surgical techniques (eg, thrombectomy, venous jump grafts, use of portal vein tributaries) have resulted in improved results post-OLT in end-stage liver disease with portal vein thrombosis.
- In patients with associated portal vein thrombosis, the 5- and 10-year survival rates after OLT are approximately 63% and 53%, respectively, whereas, in patients without thrombosis, the 5- and 10-year survival rates after OLT are 67% and 59%, respectively.
- In patients with associated portal vein thrombosis, a higher incidence (5%) of primary nonfunction, renal failure, and recurrent portal vein thrombosis exists.
- Young, otherwise healthy patients with extension of thrombus to the splenic and mesenteric venous systems, eliminating surgical shunt options, should be considered for multivisceral transplantation.
Consultations
- Gastroenterologists and hepatologists: Seek a consultation for management of acute variceal bleeding and to coordinate further management.
- Interventional radiologists: Seek a consultation for consideration of TIPS, especially if recurrent variceal bleeding exists in a transplant candidate.
- General surgeons and transplant surgeons: Seek a consultation for consideration of shunt surgery and to assess transplant suitability in patients with underlying cirrhosis.
Chawla Y, Dhiman RK. Intrahepatic portal venopathy and related disorders of the liver. Semin Liver Dis. Aug 2008;28(3):270-81. [Medline].
Abd El-Hamid N, Taylor RM, Marinello D, Mufti GJ, Patel R, Mieli-Vergani G, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience. J Pediatr Gastroenterol Nutr. Nov 2008;47(5):630-4. [Medline].
Sharma P, Sharma BC, Puri V, Sarin SK. Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol. Apr 2009;104(4):885-90. [Medline].
Facciuto ME, Rodriguez-Davalos MI, Singh MK, Rocca JP, Rochon C, Chen W, et al. Recanalized umbilical vein conduit for meso-Rex bypass in extrahepatic portal vein obstruction. Surgery. Apr 2009;145(4):406-10. [Medline].
Nihal L, Bapat MR, Rathi P, Shah NS, Karvat A, Abraham P, et al. 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. Mar 2009;3(1):305-9. [Medline].
Nakao A, Kanzaki A, Fujii T, Kodera Y, Yamada S, Sugimoto H, et al. Correlation Between Radiographic Classification and Pathological Grade of Portal Vein Wall Invasion in Pancreatic Head Cancer. Ann Surg. Dec 8 2011;[Medline].
Liu Q, Chen J, Li H, Liang B, Zhang L, Hu T. Hepatocellular carcinoma with bile duct tumor thrombi: Correlation of magnetic resonance imaging features to histopathologic manifestations. Eur J Radiol. Jun 5 2009;[Medline].
Tritou I, Megremis S, Stefanaki E, Goumenakis M, Sfakianaki E. Sonographic detection of transient gas in the portal vein in an infant following abdominal surgery: A possible sign of adhesive small bowel obstruction. J Clin Ultrasound. Sep 26 2011;[Medline].
Guérin F, Porras J, Fabre M, Guettier C, Pariente D, Bernard O, et al. Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg. Jul 2009;44(7):1337-43. [Medline].
Albertyn LE. Acute portal vein thrombosis. Clin Radiol. Nov 1987;38(6):645-8. [Medline].
Balfour GW, Stewart TG. Case of enlarged spleen complicated with ascites, both depending upon varicose dilatation and thrombosis of the portal vein. Edinburgh Med Journal. 1869;14:589-598.
Belli L, Romani F, Riolo F, et al. Thrombosis of portal vein in absence of hepatic disease. Surg Gynecol Obstet. Jul 1989;169(1):46-9. [Medline].
Bildozola M, Kravetz D, Argonz J. Efficacy of octreotide and sclerotherapy in the treatment of acute variceal bleeding in cirrhotic patients. A prospective, multicentric, and randomized clinical trial. Scand J Gastroenterol. Apr 2000;35(4):419-25. [Medline].
Bircher J, Benhamou JP, McIntyre N. Oxford Textbook of Clinical Hepatology. 2nd ed. 1999: 601, 1463-7, 1807-8, 1885-6, 2057-8.
Blum U, Haag K, Rossle M, et al. Noncavernomatous portal vein thrombosis in hepatic cirrhosis: treatment with transjugular intrahepatic portosystemic shunt and local thrombolysis. Radiology. Apr 1995;195(1):153-7. [Medline].
Charco R, Fuster J, Fondevila C. Portal vein thrombosis in liver transplantation. Transplant Proc. Nov 2005;37(9):3904-5. [Medline].
Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review. Am J Med. Feb 1992;92(2):173-82. [Medline].
Dubuisson C, Boyer-Neumann C, Wolf M. Protein C, protein S and antithrombin III in children with portal vein obstruction. J Hepatol. Jul 1997;27(1):132-5. [Medline].
Egesel T, Buyukasik Y, Dundar SV, et al. The role of natural anticoagulant deficiencies and factor V Leiden in the development of idiopathic portal vein thrombosis. J Clin Gastroenterol. Jan 2000;30(1):66-71. [Medline].
Ganger DR, Klapman JB, McDonald V, et al. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol. Mar 1999;94(3):603-8. [Medline].
Gimeno FA, Calvo J, Loinaz C. Comparative analysis of the results of orthotopic liver transplantation in patients with and without portal vein thrombosis. Transplant Proc. Nov 2005;37(9):3899-903. [Medline].
Hidajat N, Stobbe H, Griesshaber V. Imaging and radiological interventions of portal vein thrombosis. Acta Radiol. Jul 2005;46(4):336-43. [Medline].
Kaplan KL. Case 22-1991: portal-vein thrombosis. N Engl J Med. Nov 7 1991;325(19):1384. [Medline].
Kim HB, Pomposelli JJ, Lillehei CW. Mesogonadal shunts for extrahepatic portal vein thrombosis and variceal hemorrhage. Liver Transpl. Nov 2005;11(11):1389-94. [Medline].
Laishram H, Cramer B, Kennedy R. Idiopathic acute portal vein thrombosis: a case report. J Pediatr Surg. Sep 1993;28(9):1106-8. [Medline].
Macpherson AI. Portal hypertension due to extrahepatic portal venous obstruction. A review of 40 cases. J R Coll Surg Edinb. Jan 1984;29(1):4-10. [Medline].
Maddrey WC, Sen Gupta KP, Mallik KC, et al. Extrahepatic obstruction of the portal venous system. Surg Gynecol Obstet. Nov 1968;127(5):989-98. [Medline].
Mangia A, Villani MR, Cappucci G. Causes of portal venous thrombosis in cirrhotic patients: the role of genetic and acquired factors. Eur J Gastroenterol Hepatol. Jul 2005;17(7):745-51. [Medline].
Merkel C, Bolognesi M, Bellon S. Long-term follow-up study of adult patients with non-cirrhotic obstruction of the portal system: comparison with cirrhotic patients. J Hepatol. Jul 1992;15(3):299-303. [Medline].
Miyazaki Y, Shinomura Y, Kitamura S, et al. Portal vein thrombosis associated with active ulcerative colitis: percutaneous transhepatic recanalization. Am J Gastroenterol. Sep 1995;90(9):1533-4. [Medline].
Okuda K, Ohnishi K, Kimura K. Incidence of portal vein thrombosis in liver cirrhosis. An angiographic study in 708 patients. Gastroenterology. Aug 1985;89(2):279-86. [Medline].
Orozco H, Takahashi T, Garcia-Tsao G. A comparative clinical study of idiopathic portal hypertension, extrahepatic portal vein thrombosis, and cirrhosis. J Clin Gastroenterol. Oct 1994;19(3):217-21. [Medline].
Orozco H, Takahashi T, Mercado MA, et al. Surgical management of extrahepatic portal hypertension and variceal bleeding. World J Surg. Mar-Apr 1994;18(2):246-50. [Medline].
Parvey HR, Raval B, Sandler CM. Portal vein thrombosis: imaging findings. AJR Am J Roentgenol. Jan 1994;162(1):77-81. [Medline].
Pirisi M, Avellini C, Fabris C, et al. Portal vein thrombosis in hepatocellular carcinoma: age and sex distribution in an autopsy study. J Cancer Res Clin Oncol. 1998;124(7):397-400. [Medline].
Politoske D, Ralls P, Korula J. Portal vein thrombosis following endoscopic variceal sclerotherapy. Prospective controlled comparison in patients with cirrhosis. Dig Dis Sci. Jan 1996;41(1):185-90. [Medline].
Primignani M, Martinelli I, Bucciarelli P. Risk factors for thrombophilia in extrahepatic portal vein obstruction. Hepatology. Mar 2005;41(3):603-8. [Medline].
Robson SC, Kahn D, Kruskal J, et al. Disordered hemostasis in extrahepatic portal hypertension. Hepatology. Oct 1993;18(4):853-7. [Medline].
Sarin SK, Lamba GS, Kumar M. Comparison of endoscopic ligation and propranolol for the primary prevention of variceal bleeding. N Engl J Med. Apr 1 1999;340(13):988-93. [Medline].
Schiff ER, Sorrell MF, Maddrey WC. Schiff's Diseases of the Liver. 8th ed. 1999: 279-80, 509-10, 1479-80.
Scully RE, Mark EJ, McNelly WF. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 22-1991. A 15-year-old boy with fever of unknown origin, severe anemia, and portal-vein thrombosis. N Engl J Med. May 30 1991;324(22):1575-84. [Medline].
Seu P, Shackleton CR, Shaked A. Improved results of liver transplantation in patients with portal vein thrombosis. Arch Surg. Aug 1996;131(8):840-4; discussion 844-5. [Medline].
Sheen CL, Lamparelli H, Milne A, et al. Clinical features, diagnosis and outcome of acute portal vein thrombosis. QJM. Aug 2000;93(8):531-4. [Medline].
Sherlock S. Extrahepatic portal venous hypertension in adults. Clin Gastroenterol. Jan 1985;14(1):1-19. [Medline].
Tanaka K, Numata K, Okazaki H, et al. Diagnosis of portal vein thrombosis in patients with hepatocellular carcinoma: efficacy of color Doppler sonography compared with angiography. AJR Am J Roentgenol. Jun 1993;160(6):1279-83. [Medline].
Taylor CR. Computed tomography in the evaluation of the portal venous system. J Clin Gastroenterol. Mar 1992;14(2):167-72. [Medline].
Taylor CR, McCauley TR. Magnetic resonance imaging in the evaluation of the portal venous system. J Clin Gastroenterol. Apr 1992;14(3):268-73. [Medline].
Valla DC, Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol. May 2000;32(5):865-71. [Medline].
Vianna R, Giovanardi RO, Fridell JA. Multivisceral transplantation for diffuse portomesenteric thrombosis in a patient with life-threatening esophagogastroduodenal bleeding. Transplantation. Aug 27 2005;80(4):534-5. [Medline].
Wang LY, Lin ZY, Chang WY, et al. Duplex pulsed Doppler sonography of portal vein thrombosis in hepatocellular carcinoma. J Ultrasound Med. May 1991;10(5):265-9. [Medline].
Webb LJ, Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med. Oct 1979;48(192):627-39. [Medline].
Webster GJ, Burroughs AK, Riordan SM. Review article: portal vein thrombosis -- new insights into aetiology and management. Aliment Pharmacol Ther. Jan 1 2005;21(1):1-9. [Medline].
Yerdel MA, Gunson B, Mirza D, et al. Portal vein thrombosis in adults undergoing liver transplantation: risk factors, screening, management, and outcome. Transplantation. May 15 2000;69(9):1873-81. [Medline].

