Extrahepatic portal vein thrombosis: aetiology and determinants of survival

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. (Gut 2001;49:720-724)

Natural history and management of esophagogastric varices in chronic noncirrhotic, nontumoral portal vein thrombosis

“…Although gastrointestinal bleed at diagnosis of PVT, (13) anticoagulant therapy, (25) extension of thrombosis to the splenic vein, presence of gastric fundal varices, (32) and thrombosis of the superior mesenteric vein (34) have all been described as predictive factors associated with rebleeding episodes during follow-up, we could not confirm these findings in our study. Finally, in our cohort of patients with PVT the mortality was very low, only occurring in nine patients (5%) with an actuarial probability of survival of 99% and 96% at 1 and 5 years, respectively, confirming the good prognosis of these patients.…”

“…Finally, in our cohort of patients with PVT the mortality was very low, only occurring in nine patients (5%) with an actuarial probability of survival of 99% and 96% at 1 and 5 years, respectively, confirming the good prognosis of these patients. (13,25,33,35) In our study, by univariate Cox regression analysis, age, altered liver enzymes, and ascites were significantly associated with mortality, as observed by Janssen et al (25,34) and Orr et al (33) In this study, selection bias was minimized by including all patients with noncirrhotic, nontumoral PVT registered and followed at the two centers. The long study period of over 20 years could be associated with a change in the management of patients seen early in the study compared to those diagnosed more recently, with the latter patients being more likely to receive anticoagulation and consequently a greater possibility of portal vein recanalization.…”

Portal Vein Thrombosis

“…17 A multivariate analysis done on determinants of survival in extra hepatic portal vein thrombosis showed that advanced age, malignancy, cirrhosis, mesenteric vein thrombosis, absence of abdominal inflammation, serum levels of aminotransferase and albumin are associated with reduced survival and not to complications of portal hypertension. 15 Chronic PVT did not show any extension of the thrombus as shown by repeat imaging after as long as 10 years. 114 Bleeding after initial variceal eradication occurs usually within first 4-10 years and the risk of bleeding reduces as age progresses especially after 10 years.…”

“…[8][9][10][11][12][13] In some cases multiple prothrombotic factors may be associated in the development of PVT. [14][15][16] In one study one or more risk factors namely prothrombotic state or abdominal inflammation was present in 87% of patients. 17 Amongst the thrombophilic states, primary myeloproliferative disorders (MPD) are common in 30.5%.…”

“…Once the diagnosis of PVT is made, extensive investigation of prothrombotic disorders and local factors is recommended 8,15,109 especially for patients with a life expectancy of more than 3-6 months and where anticoagulation is considered relevant. A list of procoagulant work up is given in Table 2.…”