Logo of gutGutVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Gut. Nov 2001; 49(5): 720–724.
PMCID: PMC1728504

Extrahepatic portal vein thrombosis: aetiology and determinants of survival


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 (127K).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Webb LJ, Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med. 1979 Oct;48(192):627–639. [PubMed]
  • Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review. Am J Med. 1992 Feb;92(2):173–182. [PubMed]
  • Valla D, Casadevall N, Huisse MG, Tulliez M, Grange JD, Muller O, Binda T, Varet B, Rueff B, Benhamou JP. Etiology of portal vein thrombosis in adults. A prospective evaluation of primary myeloproliferative disorders. Gastroenterology. 1988 Apr;94(4):1063–1069. [PubMed]
  • Valla DC, Condat B. Portal vein thrombosis in adults: pathophysiology, pathogenesis and management. J Hepatol. 2000 May;32(5):865–871. [PubMed]
  • Cardin F, Graffeo M, McCormick PA, 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. [PubMed]
  • Belli L, Romani F, Sansalone CV, Aseni P, Rondinara G. Portal thrombosis in cirrhotics. A retrospective analysis. Ann Surg. 1986 Mar;203(3):286–291. [PMC free article] [PubMed]
  • Janssen HL, Meinardi JR, Vleggaar FP, van Uum SH, Haagsma EB, van Der Meer FJ, van Hattum J, Chamuleau RA, Adang RP, Vandenbroucke JP, 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 Oct 1;96(7):2364–2368. [PubMed]
  • Pabinger I, Allaart CF, Hermans J, Briët E, Bertina RM. 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]
  • Fonkalsrud EW, Myers NA, Robinson MJ. Management of extrahepatic portal hypertension in children. Ann Surg. 1974 Oct;180(4):487–493. [PMC free article] [PubMed]
  • Warren WD, Henderson JM, Millikan WJ, Galambos JT, Bryan FC. Management of variceal bleeding in patients with noncirrhotic portal vein thrombosis. Ann Surg. 1988 May;207(5):623–634. [PMC free article] [PubMed]
  • Vandenbroucke JP, Koster T, Briët E, Reitsma PH, Bertina RM, Rosendaal FR. 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. [PubMed]
  • Rosendaal FR. Venous thrombosis: a multicausal disease. Lancet. 1999 Apr 3;353(9159):1167–1173. [PubMed]
  • Denninger MH, Chaït Y, Casadevall N, Hillaire S, Guillin MC, 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. [PubMed]
  • Chawla YK, Dilawari JB, Ramesh GN, Kaur U, Mitra SK, Walia BN. Sclerotherapy in extrahepatic portal venous obstruction. Gut. 1990 Feb;31(2):213–216. [PMC free article] [PubMed]
  • 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. [PubMed]
  • Grauer SE, Schwartz SI. Extrahepatic portal hypertension: a retrospective analysis. Ann Surg. 1979 May;189(5):566–574. [PMC free article] [PubMed]
  • Voorhees AB, Jr, Price JB., Jr Extrahepatic portal hypertension. A retrospective analysis of 127 cases and associated clinical implications. Arch Surg. 1974 Mar;108(3):338–341. [PubMed]
  • Lagasse JP, Bahallah ML, Salem N, Debillon G, Labarrière D, Serve MP, Advenier V, Causse X, Legoux JL. 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]

Figures and Tables

Figure 1
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
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 ...

Articles from Gut are provided here courtesy of BMJ Group


Related citations in PubMed

See reviews...See all...

Cited by other articles in PMC

See all...


  • MedGen
    Related information in MedGen
  • PubMed
    PubMed citations for these articles
  • Substance
    PubChem Substance links

Recent Activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...