Skip to main content
PMC home page
Gut logoLink to Gut
. 2001 Nov;49(5):720–724. doi: 10.1136/gut.49.5.720

Extrahepatic portal vein thrombosis: aetiology and determinants of survival

H Janssen 1, A Wijnhoud 1, E Haagsma 1, S H M van Uum 1, C M J van Nieuwkerk 1, R Adang 1, R Chamuleau 1, J van Hattum 1, F Vleggaar 1, B Hansen 1, F Rosendaal 1, B van Hoek 1
PMCID: PMC1728504  PMID: 11600478

Abstract

BACKGROUND—Malignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis (EPVT). Mortality of patients with EPVT may be associated with these concurrent medical conditions or with manifestations of portal hypertension, such as variceal haemorrhage.
PATIENTS AND METHODS—To determine which variables have prognostic significance with respect to survival, we performed a retrospective study of 172 adult EPVT patients who were followed over the period 1984-1997 in eight university hospitals.
RESULTS—Mean follow up was 3.9 years (range 0.1-13.1). Overall survival was 70% (95% confidence interval (CI) 62-76%) at one year, 61% (95% CI, 52-67%) at five years, and 54% (95% CI, 45-62%) at 10 years. The one, five, and 10 year survival rates in the absence of cancer, cirrhosis, and mesenteric vein thrombosis were 95% (95% CI 87-98%), 89% (95% CI 78-94%), and 81% (95% CI 67-89%), respectively (n=83). Variables at diagnosis associated with reduced survival according to multivariate analysis were advanced age, malignancy, cirrhosis, mesenteric vein thrombosis, absence of abdominal inflammation, and serum levels of aminotransferase and albumin. The presence of variceal haemorrhage and myeloproliferative disorders did not influence survival. Only four patients died due to variceal haemorrhage and one due to complications of a portosystemic shunt procedure.
CONCLUSION—We conclude that mortality among patients with EPVT is related primarily to concurrent disorders leading to EPVT and not to complications of portal hypertension.


Keywords: extrahepatic portal vein thrombosis; portal vein obstruction; survival; mortality

Full Text

The Full Text of this article is available as a PDF (126.7 KB).

Figure 1  .

Figure 1  

Open in a new tab

Actuarial survival of 172 patients with extrahepatic portal vein thrombosis. Number of patients at risk during follow up are shown along the x axis.

Figure 2  .

Figure 2  

Open in a new tab

Actuarial survival rates for patients with extrahepatic vein thrombosis without cirrhosis and cancer (patients at risk n=98, n=47, and n=17 after 0, 5, and 10 years of follow up, respectively), for patients with cirrhosis but no cancer (patients at risk n=33, n=9, and n=3 after 0, 5 and 10 years of follow up, respectively), and for patients with cancer (patients at risk n=41 and n=3 after 0 and 5 years of follow up, respectively).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Belli L., Romani F., Sansalone C. V., Aseni P., Rondinara G. Portal thrombosis in cirrhotics. A retrospective analysis. Ann Surg. 1986 Mar;203(3):286–291. doi: 10.1097/00000658-198603000-00012. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Cardin F., Graffeo M., McCormick P. A., McIntyre N., Burroughs A. Adult "idiopathic" extrahepatic venous thrombosis. Importance of putative "latent" myeloproliferative disorders and comparison with cases with known etiology. Dig Dis Sci. 1992 Mar;37(3):335–339. doi: 10.1007/BF01307724. [DOI] [PubMed] [Google Scholar]
  3. Chawla Y. K., Dilawari J. B., Ramesh G. N., Kaur U., Mitra S. K., Walia B. N. Sclerotherapy in extrahepatic portal venous obstruction. Gut. 1990 Feb;31(2):213–216. doi: 10.1136/gut.31.2.213. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cohen J., Edelman R. R., Chopra S. Portal vein thrombosis: a review. Am J Med. 1992 Feb;92(2):173–182. doi: 10.1016/0002-9343(92)90109-o. [DOI] [PubMed] [Google Scholar]
  5. Denninger M. H., Chaït Y., Casadevall N., Hillaire S., Guillin M. C., Bezeaud A., Erlinger S., Briere J., Valla D. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology. 2000 Mar;31(3):587–591. doi: 10.1002/hep.510310307. [DOI] [PubMed] [Google Scholar]
  6. Fonkalsrud E. W., Myers N. A., Robinson M. J. Management of extrahepatic portal hypertension in children. Ann Surg. 1974 Oct;180(4):487–493. doi: 10.1097/00000658-197410000-00014. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Grauer S. E., Schwartz S. I. Extrahepatic portal hypertension: a retrospective analysis. Ann Surg. 1979 May;189(5):566–574. doi: 10.1097/00000658-197905000-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Janssen H. L., Meinardi J. R., Vleggaar F. P., van Uum S. H., Haagsma E. B., van Der Meer F. J., van Hattum J., Chamuleau R. A., Adang R. P., Vandenbroucke J. P. 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 Oct 1;96(7):2364–2368. [PubMed] [Google Scholar]
  9. Lagasse J. P., Bahallah M. L., Salem N., Debillon G., Labarrière D., Serve M. P., Advenier V., Causse X., Legoux J. L. Thrombose aiguë du système porte. Traitement par alteplase et héparine ou héparine seule chez 10 malades. Gastroenterol Clin Biol. 1997;21(12):919–923. [PubMed] [Google Scholar]
  10. Merkel C., Bolognesi M., Bellon S., Sacerdoti D., Bianco S., Amodio P., Gatta A. Long-term follow-up study of adult patients with non-cirrhotic obstruction of the portal system: comparison with cirrhotic patients. J Hepatol. 1992 Jul;15(3):299–303. doi: 10.1016/0168-8278(92)90059-x. [DOI] [PubMed] [Google Scholar]
  11. Pabinger I., Allaart C. F., Hermans J., Briët E., Bertina R. M. Hereditary protein C-deficiency: laboratory values in transmitters and guidelines for the diagnostic procedure. Report on a study of the SSC Subcommittee on Protein C and Protein S. Protein C Transmitter Study Group. Thromb Haemost. 1992 Oct 5;68(4):470–474. [PubMed] [Google Scholar]
  12. Rosendaal F. R. Venous thrombosis: a multicausal disease. Lancet. 1999 Apr 3;353(9159):1167–1173. doi: 10.1016/s0140-6736(98)10266-0. [DOI] [PubMed] [Google Scholar]
  13. Valla D. C., Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol. 2000 May;32(5):865–871. doi: 10.1016/s0168-8278(00)80259-7. [DOI] [PubMed] [Google Scholar]
  14. Valla D., Casadevall N., Huisse M. G., Tulliez M., Grange J. D., Muller O., Binda T., Varet B., Rueff B., Benhamou J. P. Etiology of portal vein thrombosis in adults. A prospective evaluation of primary myeloproliferative disorders. Gastroenterology. 1988 Apr;94(4):1063–1069. doi: 10.1016/0016-5085(88)90567-7. [DOI] [PubMed] [Google Scholar]
  15. Vandenbroucke J. P., Koster T., Briët E., Reitsma P. H., Bertina R. M., Rosendaal F. R. Increased risk of venous thrombosis in oral-contraceptive users who are carriers of factor V Leiden mutation. Lancet. 1994 Nov 26;344(8935):1453–1457. doi: 10.1016/s0140-6736(94)90286-0. [DOI] [PubMed] [Google Scholar]
  16. Voorhees A. B., Jr, Price J. B., Jr Extrahepatic portal hypertension. A retrospective analysis of 127 cases and associated clinical implications. Arch Surg. 1974 Mar;108(3):338–341. doi: 10.1001/archsurg.1974.01350270068012. [DOI] [PubMed] [Google Scholar]
  17. Warren W. D., Henderson J. M., Millikan W. J., Galambos J. T., Bryan F. C. Management of variceal bleeding in patients with noncirrhotic portal vein thrombosis. Ann Surg. 1988 May;207(5):623–634. doi: 10.1097/00000658-198805000-00017. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Webb L. J., Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med. 1979 Oct;48(192):627–639. [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES