Portal Vein Obstruction 

  • Author: Adnan Said, MD, MSPH; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 5, 2012
 

Background

In the English literature, portal vein obstruction was first reported in 1868 by Balfour and Stewart, who described a patient presenting with an enlarged spleen, ascites, and variceal dilatation.

The vast majority of cases are due to primary thrombosis of the portal vein; most of the remaining cases are caused by malignant obstruction. See the images below.

Portal vein thrombosis with cavernous transformatiPortal vein thrombosis with cavernous transformation. The long arrow indicates the splenic vein at the junction with the superior mesenteric vein just below the site of thrombosis. The short arrow points to a serpiginous mass consistent with periportal collaterals, the so-called cavernous transformation of the portal vein. Hepatocellular carcinoma with portal vein thrombosHepatocellular carcinoma with portal vein thrombosis. The short arrow indicates the tumor thrombus with an abrupt cut off of the portal vein. The long arrow points to a compensatory, prominent left hepatic arterial branch.
Next

Pathophysiology

The portal vein forms at the junction of the splenic vein and the superior mesenteric vein behind the pancreatic head, and it can become thrombosed or obstructed at any point along its course. In cirrhosis and hepatic malignancies, the thromboses usually begin intrahepatically and spread to the extrahepatic portal vein. In most other etiologies, the thromboses usually start at the site of origin of the portal vein. Occasionally, thrombosis of the splenic vein propagates to the portal vein, most often resulting from an adjacent inflammatory process such as chronic pancreatitis.

Inherited and acquired disorders of the coagulation pathway are frequent causes of portal vein thrombosis. Inherited disorders include mutations in the prothrombin gene G20210A as well as deficiencies of various intrinsic anticoagulation factors, such as protein C and protein S, and activated protein C resistance. Acquired disorders include antithrombin III deficiency resulting from malnutrition, sepsis, disseminated intravascular coagulation, inflammatory bowel disease, liver disease, or estrogen use. See the image below.

Coagulation disorders in portal vein thrombosis. Coagulation disorders in portal vein thrombosis.

Stasis can be another major category for portal vein thrombosis. The global resistance to hepatic blood flow produced by cirrhosis is a common cause. Sclerotherapy for esophageal varices has been postulated as a possible mechanism though not proven thus far. The portal vein or its tributaries can be obstructed by adjacent tumor compression or invasion. Infectious and inflammatory processes may also lead to venous thrombosis.

Portal vein obstruction does not affect liver function unless the patient has an underlying liver disease such as cirrhosis.[1] This is partially due to a rapid arterial buffer response, with compensatory increased flow of the hepatic artery maintaining the total hepatic blood flow. Formation of collaterals occurs rather rapidly as well, and they have been described as early as 12 days after acute thrombosis, though the average time to formation is approximately 5 weeks.

The development of a collateral circulation, with its attendant risk of variceal hemorrhage, is responsible for most of the complications and is the most common manifestation of portal vein obstruction. Other sequelae of the subsequent portal hypertension, such as ascites, are less frequent. Rarely, the thrombosis extends from the portal vein to the mesenteric arcades, leading to bowel ischemia and infarction.

Previous
Next

Epidemiology

Frequency

United States

Portal vein obstruction is a relatively rare condition with an overall incidence of 0.05-0.5% in autopsy studies. Incidence varies, depending on the group of patients studied (eg, general population vs patients with cirrhosis) and the method used to diagnose portal vein obstruction (eg, autopsy studies, angiography studies, noninvasive radiological screening).

Incidence of portal vein obstruction in people with cirrhosis has been reported to vary from 5-18%. However, these were patients referred for transplantation and were at an advanced stage of liver disease. No large autopsy studies are available. Extrahepatic portal vein obstruction is estimated to be responsible for 5-10% of all cases of portal hypertension.

International

In Japan, the frequency of portal vein obstruction in autopsy studies was reported to be 0.05%. In an angiography surveillance study of patients with cirrhosis, the incidence was 0.5%, which is much lower than the reported incidence in the Western literature.

In India, extrahepatic portal vein obstruction is reported more frequently; in one study, the incidence even exceeded reported cases of cirrhosis. Of all cases of portal hypertension in developing countries, 40% are attributed to portal vein obstruction, presumably secondary to an increased incidence of pylephlebitis associated with abdominal infections.

Mortality/Morbidity

  • In the absence of cirrhosis, the 2 year bleeding risk from esophageal varices is reported to be 0.25% and of those that bleed the mortality rate is approximately 5%. Those with cirrhosis and varices have a 20-30% 2 year bleeding risk with a mortality rate of 30-70%. This difference is primarily a consequence of the normal hepatic function in the noncirrhotic patient. Variceal size is the major predictive factor for bleeding.
  • In adults with portal vein thrombosis, the 10-year survival rate has been reported to be 38-60%, with most of the deaths occurring secondary to the underlying disease (eg, cirrhosis, malignancy).
  • In children with portal vein thrombosis, the prognosis is much better overall, with a 10-year survival rate greater than 70%, which is attributable to the low incidence of underlying malignancy and cirrhosis.

Race

No racial differences have been reported.

Sex

No sex differences have been reported overall, except for a slight male predominance in patients whose obstruction is secondary to cirrhosis.

Age

  • The distribution of the age of presentation of portal vein thrombosis reflects the demographics of the underlying disease process. Primary portal vein thrombosis from coagulopathies occurs with equal frequency in adults and children.[2] The frequency of portal vein obstruction from tumor compression or invasion is greater in adults.
Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Adnan Said, MD, MSPH  Assistant Professor, Department of Medicine, Section of Gastroenterology and Hepatology, University of Wisconsin School of Medicine and Public Health at Madison; Consulting Staff, Department of Medicine, William S Middleton Memorial Veterans Hospital

Adnan Said, MD, MSPH is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and American Society of Transplantation

Disclosure: Nothing to disclose.

Coauthor(s)

Jennifer T Wells, MD  Fellow, Department of Gastroenterology and Hepatology, University of Wisconsin Hospitals and Clinics, Madison

Jennifer T Wells, MD is a member of the following medical societies: American Medical Association and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Ann Ouyang, MBBS  Professor, Department of Internal Medicine, Pennsylvania State University College of Medicine; Attending Physician, Division of Gastroenterology and Hepatology, Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Noel Williams, MD  Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors, Mark Reichelderfer, MD, and Andrew Taylor, MD, to the development and writing of this article.

References

  1. Chawla Y, Dhiman RK. Intrahepatic portal venopathy and related disorders of the liver. Semin Liver Dis. Aug 2008;28(3):270-81. [Medline].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. Albertyn LE. Acute portal vein thrombosis. Clin Radiol. Nov 1987;38(6):645-8. [Medline].

  11. 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.

  12. 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].

  13. 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].

  14. Bircher J, Benhamou JP, McIntyre N. Oxford Textbook of Clinical Hepatology. 2nd ed. 1999: 601, 1463-7, 1807-8, 1885-6, 2057-8.

  15. 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].

  16. Charco R, Fuster J, Fondevila C. Portal vein thrombosis in liver transplantation. Transplant Proc. Nov 2005;37(9):3904-5. [Medline].

  17. Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review. Am J Med. Feb 1992;92(2):173-82. [Medline].

  18. 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].

  19. 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].

  20. 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].

  21. 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].

  22. Hidajat N, Stobbe H, Griesshaber V. Imaging and radiological interventions of portal vein thrombosis. Acta Radiol. Jul 2005;46(4):336-43. [Medline].

  23. Kaplan KL. Case 22-1991: portal-vein thrombosis. N Engl J Med. Nov 7 1991;325(19):1384. [Medline].

  24. 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].

  25. Laishram H, Cramer B, Kennedy R. Idiopathic acute portal vein thrombosis: a case report. J Pediatr Surg. Sep 1993;28(9):1106-8. [Medline].

  26. 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].

  27. 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].

  28. 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].

  29. 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].

  30. 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].

  31. 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].

  32. 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].

  33. 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].

  34. Parvey HR, Raval B, Sandler CM. Portal vein thrombosis: imaging findings. AJR Am J Roentgenol. Jan 1994;162(1):77-81. [Medline].

  35. 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].

  36. 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].

  37. Primignani M, Martinelli I, Bucciarelli P. Risk factors for thrombophilia in extrahepatic portal vein obstruction. Hepatology. Mar 2005;41(3):603-8. [Medline].

  38. Robson SC, Kahn D, Kruskal J, et al. Disordered hemostasis in extrahepatic portal hypertension. Hepatology. Oct 1993;18(4):853-7. [Medline].

  39. 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].

  40. Schiff ER, Sorrell MF, Maddrey WC. Schiff's Diseases of the Liver. 8th ed. 1999: 279-80, 509-10, 1479-80.

  41. 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].

  42. 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].

  43. 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].

  44. Sherlock S. Extrahepatic portal venous hypertension in adults. Clin Gastroenterol. Jan 1985;14(1):1-19. [Medline].

  45. 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].

  46. Taylor CR. Computed tomography in the evaluation of the portal venous system. J Clin Gastroenterol. Mar 1992;14(2):167-72. [Medline].

  47. 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].

  48. Valla DC, Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol. May 2000;32(5):865-71. [Medline].

  49. 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].

  50. 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].

  51. 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].

  52. 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].

  53. 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].

 
Previous
Next
 
The etiology of portal vein obstruction.
Coagulation disorders in portal vein thrombosis.
Portal vein thrombosis with cavernous transformation. The long arrow indicates the splenic vein at the junction with the superior mesenteric vein just below the site of thrombosis. The short arrow points to a serpiginous mass consistent with periportal collaterals, the so-called cavernous transformation of the portal vein.
Hepatocellular carcinoma with portal vein thrombosis. The short arrow indicates the tumor thrombus with an abrupt cut off of the portal vein. The long arrow points to a compensatory, prominent left hepatic arterial branch.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.