Portal vein thrombosis

Background

Drawing of portal vein thrombosis.

Portal vein thrombosis (PVT) is a vascular disease of the liver that occurs when a blood clot occurs in the hepatic portal vein, leading to increased pressure in the portal vein system and reduced blood supply to the liver.

Inciting Causes

• Abdominal sepsis
• Abdominal surgery
• Behçet's syndrome
• Cholangiocarcinoma
• Cirrhosis
• Collagen vascular diseases (eg, lupus)
• Compression or invasion of the portal vein by tumor (eg, pancreatic cancer)
• Diverticulitis
• Endoscopic sclerotherapy
• Hepatocellular carcinoma
• Inflammatory bowel disease
• Inherited thrombophilias (e.g. factor V Leiden deficiency, protein C or S deficiency, antiphospholipid syndrome)
• Myeloproliferative disorders (e.g. polycythemia vera or essential thrombocytosis)
• Omphalitis
• Oral contraceptives
• Pancreatic islet cell transplantation
• Pancreatitis
• Paroxysmal nocturnal hemoglobinuria
• Pregnancy
• Retroperitoneal fibrosis
• Splenectomy complication
• Transjugular intrahepatic portosystemic shunt (TIPS)
• Trauma

Clinical Features

Acute

• Upper abdominal pain developing suddenly or progressing over a few days
• Possibly accompanied by nausea, hepatomegaly, splenomegaly; may be accompanied by persistent non-spiking fever in the setting of systemic inflammation
• Other signs/symptoms based on underlying cause (e.g. bleeding disorders, hepatic stigmata if due to cirrhosis)
• May be clinically silent in a portion of patients and diagnosed incidentally during a CT exam for other reasons (e.g. acute pancreatitis)

Chronic

• Often asymptomatic; incidental finding on imaging
• Associated with portal hypertension

Differential Diagnosis

• Mesenteric vein thrombosis
• Budd-Chiari syndrome
• Portal vein thrombosis
• Venous thromboembolism

Evaluation

Phase contrast CT image. Contrast is perfusing the right liver but not the left due to a left portal vein thrombus.

• Typically diagnosed on abdominal CT with contrast
• Filling defect on Doppler ultrasound
• EGD to assess for gastric/esophageal varices if chronic PVT

Management

Acute

• In non-cirrhotic, LMWH -> warfarin x 6 months

Chronic

• In non-cirrhotic or hypercoagulable, anticoagulation after screening for varices
• Anticoagulate patients with cirrhosis AND 1) thrombophilia, 2) clot extension in mesenteric veins, or 3) bowel ischemia
• If severe, may require shunt, liver transplant, or bowel resection

See Also

• Budd-Chiari syndrome (hepatic vein thrombosis)
• Pylephlebitis
• Venous thromboembolism

References

1. DeLeve LD, Valla DC, Garcia-Tsao G (2009). "Vascular disorders of the liver". Hepatology. 49:1729-64.

2. O'Mara SR, Wiesner L. "Hepatic Disorders". In Tintinalli JE, Ma O, Yealy DM, Meckler GD, Stapczynski J, Cline DM, Thomas SH (eds.). Tintinalli's Emergency Medicine: A Comprehensive Study Guide (9 ed.). New York, NY: McGraw-Hill.

3. Simonetto DA, Singal AK, Garcia-Tsao G, Caldwell SH, Ahn J, Kamath PS (January 3, 2020). "ACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation". Am J Gastroenterol. 115: 18-40.

4. Nery F, Chevret S, Condat B, de Raucourt E, Boudaoud L, Rautou P, Plessier A, Roulot D, Chaffaut C, Bourcier V, Trinchet J, Valla D (February 2015). "Causes and Consequences of Portal Vein Thrombosis in 1,243 Patients with Cirrhosis: Results of a Longitudinal Study". Hepatology. 61: 660-667. doi:10.1002/hep.27546

5. Friedman LS (2020). "Noncirrhotic Portal Hypertension". In Papadakis MA, McPhee SJ, Rabow MW (eds.). Current Medical Diagnosis and Treatment 2020. McGraw-Hill.