Epidemiology and pathogenesis of portal vein thrombosis in adults
- Arun J Sanyal, MD
Arun J Sanyal, MD
- Professor of Medicine
- Virginia Commonwealth University School of Medicine
- Section Editor
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology/Hepatology
- Section Editor — General Hepatology; Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
The portal vein is formed by the confluence of the splenic and superior mesenteric veins, which drain the spleen and small intestine, respectively (figure 1). Portal hypertension develops as a result of obstruction to flow within the portal venous system. It can be categorized as prehepatic, intrahepatic, or posthepatic based upon the site of obstruction to flow (table 1). The intrahepatic causes of portal hypertension have been further subdivided as presinusoidal, sinusoidal, or postsinusoidal based on the location of the obstruction to portal blood flow within the liver. Causes of extrahepatic portal vein obstruction include thrombosis and invasion or constriction by a malignant tumor.
This topic will review portal vein thrombosis (PVT), the most common cause of extrahepatic portal vein obstruction. Other important causes of noncirrhotic portal hypertension (including noncirrhotic portal fibrosis and schistosomiasis) and the clinical manifestations, diagnosis, and treatment of acute and chronic PVT are discussed separately. (See "Noncirrhotic portal hypertension" and "Acute portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management" and "Chronic portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management".)
The incidence of PVT among patients without cirrhosis is unclear. It is thought to account for 5 to 10 percent of patients with portal hypertension in developed countries and up to a third of patients in developing countries (because of an increased frequency of infectious complications that predispose to PVT) [1,2]. Among patients with cirrhosis, PVT is common and is associated with the severity of the patient's liver disease [3,4]. Autopsy studies have reported prevalences of 6 to 64 percent, whereas studies that used ultrasonography to diagnose PVT reported prevalences of 5 to 24 percent . The prevalence of PVT is estimated to be less than 1 percent in patients with compensated cirrhosis, but is 8 to 25 percent in patients who are candidates for liver transplantation [6,7].
In a study of almost 24,000 autopsies in Sweden performed between 1970 and 1982, the prevalence of PVT was 1 percent . The most common predisposing conditions for PVT were cirrhosis (28 percent), primary or secondary hepatobiliary malignancy (23 and 44 percent, respectively), major infectious or inflammatory abdominal disease (10 percent), or a myeloproliferative disorder (3 percent). However, no predisposing factors were identified in 14 percent.
Portal vein thrombosis (PVT) in patients with a previously healthy liver is thought to be due to inherited or acquired prothrombotic states (table 2). However, no apparent cause for PVT is identified in more than 25 percent of patients [9-11]. Among patients with cirrhosis, the pathogenesis is likely related to unbalanced hemostasis and slowing of portal flow. (See "Overview of the causes of venous thrombosis" and "Evaluating patients with established venous thromboembolism for acquired and inherited risk factors".)
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