Chronic portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management
- Arun J Sanyal, MD
Arun J Sanyal, MD
- Charles Caravati 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
- Section Editor — 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). Occlusion of the portal vein by thrombus (portal vein thrombosis [PVT]) typically occurs in patients with cirrhosis and/or prothrombotic disorders (table 1). Chronic PVT develops in patients with acute PVT that does not resolve (with or without treatment). Patients with chronic PVT develop collateral blood vessels that bring blood in a hepatopetal manner around the area of obstruction, known as cavernous transformation of the portal vein or portal cavernoma. When seen in a transverse section, as on a computed tomographic (CT) scan, cavernous transformation gives the appearance of multiple caveolar orifices (image 1). Complications of chronic PVT include portal hypertension and portal cholangiopathy.
This topic will review the clinical manifestations, diagnosis, and management of chronic PVT. The epidemiology and pathogenesis of PVT, the approach to patients with acute PVT, and other causes of noncirrhotic portal hypertension are discussed elsewhere. (See "Epidemiology and pathogenesis of portal vein thrombosis in adults" and "Acute portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management" and "Noncirrhotic portal hypertension".)
The approach to patients with PVT has also been reviewed in 2009 guidelines from the American Association for the Study of Liver Diseases . The discussion that follows is generally consistent with those guidelines.
Chronic portal vein thrombosis (PVT) may be asymptomatic and discovered incidentally when abdominal imaging is obtained for other reasons, or patients may present with symptoms related to portal hypertension or portal cholangiopathy, two of the complications of chronic PVT. Patients with chronic PVT may also develop intestinal ischemia and infarction if there is extension of the clot into the superior mesenteric vein, though the risk is small . Patients with chronic PVT may also have clinical manifestations related underlying conditions that predisposed to PVT, such as cirrhosis (table 1). (See "Epidemiology and pathogenesis of portal vein thrombosis in adults", section on 'Portal hypertension' and "Epidemiology and pathogenesis of portal vein thrombosis in adults", section on 'Portal cholangiopathy' and "Cirrhosis in adults: Etiologies, clinical manifestations, and diagnosis", section on 'Clinical manifestations'.)
Patients with chronic PVT, even if asymptomatic, frequently have esophageal or gastric varices, and the most common clinical presentation is gastrointestinal bleeding [3-6]. In a retrospective series that included 40 patients with chronic PVT, at the time of diagnosis, esophageal varices were present in 35 patients (88 percent), gastric varices in 20 patients (50 percent), portal hypertensive gastropathy in 19 patients (48 percent), and gastrointestinal bleeding in 19 patients (48 percent) .
- DeLeve LD, Valla DC, Garcia-Tsao G, American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009; 49:1729.
- Hoekstra J, Janssen HL. Vascular liver disorders (II): portal vein thrombosis. Neth J Med 2009; 67:46.
- Sogaard KK, Astrup LB, Vilstrup H, Gronbaek H. Portal vein thrombosis; risk factors, clinical presentation and treatment. BMC Gastroenterol 2007; 7:34.
- Amitrano L, Guardascione MA, Brancaccio V, et al. Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis. J Hepatol 2004; 40:736.
- Webb LJ, Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med 1979; 48:627.
- Kameda H, Yamazaki K, Imai F, et al. Obliterative portal venopathy: A comparative study of 184 cases of extrahepatic portal obstruction and 469 cases of idiopathic portal hypertension. J Gastroenterol Hepatol 1986; 1:139.
- Condat B, Vilgrain V, Asselah T, et al. Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology 2003; 37:1302.
- Llop E, de Juan C, Seijo S, et al. Portal cholangiopathy: radiological classification and natural history. Gut 2011; 60:853.
- Khuroo MS, Yattoo GN, Zargar SA, et al. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology 1993; 17:807.
- Chattopadhyay S, Nundy S. Portal biliopathy. World J Gastroenterol 2012; 18:6177.
- Malkan GH, Bhatia SJ, Bashir K, et al. Cholangiopathy associated with portal hypertension: diagnostic evaluation and clinical implications. Gastrointest Endosc 1999; 49:344.
- Dilawari JB, Chawla YK. Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. Gut 1992; 33:272.
- Dhiman RK, Puri P, Chawla Y, et al. Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic? Gastrointest Endosc 1999; 50:646.
- Sarin SK, Bhatia V, Markwane U. Portal biliopathy in extra hepatic portal vein obstruction. Indian J Gastroenterol 1992; 2:A82.
- Handa P, Crowther M, Douketis JD. Portal Vein Thrombosis: A Clinician-Oriented and Practical Review. Clin Appl Thromb Hemost 2013; 20:498.
- Cohen J, Edelman RR, Chopra S. Portal vein thrombosis: a review. Am J Med 1992; 92:173.
- Blendis LM, Banks DC, Ramboer C, Williams R. Spleen blood flow and splanchnic haemodynamics in blood dyscrasia and other splenomegalies. Clin Sci 1970; 38:73.
- Condat B, Pessione F, Hillaire S, et al. Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy. Gastroenterology 2001; 120:490.
- Mínguez B, García-Pagán JC, Bosch J, et al. Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. Hepatology 2006; 43:707.
- Van Gansbeke D, Avni EF, Delcour C, et al. Sonographic features of portal vein thrombosis. AJR Am J Roentgenol 1985; 144:749.
- Hidajat N, Stobbe H, Griesshaber V, et al. Imaging and radiological interventions of portal vein thrombosis. Acta Radiol 2005; 46:336.
- De Gaetano AM, Lafortune M, Patriquin H, et al. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR Am J Roentgenol 1995; 165:1151.
- Mori H, Hayashi K, Uetani M, et al. High-attenuation recent thrombus of the portal vein: CT demonstration and clinical significance. Radiology 1987; 163:353.
- Gabata T, Matsui O, Kadoya M, et al. Gallbladder varices: demonstration of direct communication to intrahepatic portal veins by color doppler sonography and CT during arterial portography. Abdom Imaging 1997; 22:82.
- Catalano OA, Choy G, Zhu A, et al. Differentiation of malignant thrombus from bland thrombus of the portal vein in patients with hepatocellular carcinoma: application of diffusion-weighted MR imaging. Radiology 2010; 254:154.
- Kreft B, Strunk H, Flacke S, et al. Detection of thrombosis in the portal venous system: comparison of contrast-enhanced MR angiography with intraarterial digital subtraction angiography. Radiology 2000; 216:86.
- Squillaci E, Crecco M, Lo Presti G, et al. [Angiography with magnetic resonance in the study of portal hypertension. Clinical applications]. Radiol Med 1993; 85:756.
- Primignani M. Portal vein thrombosis, revisited. Dig Liver Dis 2010; 42:163.
- Lai L, Brugge WR. Endoscopic ultrasound is a sensitive and specific test to diagnose portal venous system thrombosis (PVST). Am J Gastroenterol 2004; 99:40.
- Piscaglia F, Gianstefani A, Ravaioli M, et al. Criteria for diagnosing benign portal vein thrombosis in the assessment of patients with cirrhosis and hepatocellular carcinoma for liver transplantation. Liver Transpl 2010; 16:658.
- Cedrone A, Rapaccini GL, Pompili M, et al. Portal vein thrombosis complicating hepatocellular carcinoma. Value of ultrasound-guided fine-needle biopsy of the thrombus in the therapeutic management. Liver 1996; 16:94.
- Dodd GD 3rd, Carr BI. Percutaneous biopsy of portal vein thrombus: a new staging technique for hepatocellular carcinoma. AJR Am J Roentgenol 1993; 161:229.
- Francoz C, Belghiti J, Vilgrain V, et al. Splanchnic vein thrombosis in candidates for liver transplantation: usefulness of screening and anticoagulation. Gut 2005; 54:691.
- Amitrano L, Guardascione MA, Menchise A, et al. Safety and efficacy of anticoagulation therapy with low molecular weight heparin for portal vein thrombosis in patients with liver cirrhosis. J Clin Gastroenterol 2010; 44:448.
- Senzolo M, M Sartori T, Rossetto V, et al. Prospective evaluation of anticoagulation and transjugular intrahepatic portosystemic shunt for the management of portal vein thrombosis in cirrhosis. Liver Int 2012; 32:919.
- Dumortier J, Vaillant E, Boillot O, et al. Diagnosis and treatment of biliary obstruction caused by portal cavernoma. Endoscopy 2003; 35:446.
- Simpson WG, Schwartz RW, Strodel WE. Splenic vein thrombosis. South Med J 1990; 83:417.
- Gouge TH, Ranson JH. Esophageal transection and paraesophagogastric devascularization for bleeding esophageal varices. Am J Surg 1986; 151:47.
- Borgonovo G, Costantini M, Grange D, et al. Comparison of a modified Sugiura procedure with portal systemic shunt for prevention of recurrent variceal bleeding in cirrhosis. Surgery 1996; 119:214.
- Ganger DR, Klapman JB, McDonald V, et al. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol 1999; 94:603.
- Senzolo M, Tibbals J, Cholongitas E, et al. Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation. Aliment Pharmacol Ther 2006; 23:767.
- Luca A, Miraglia R, Caruso S, et al. Short- and long-term effects of the transjugular intrahepatic portosystemic shunt on portal vein thrombosis in patients with cirrhosis. Gut 2011; 60:846.
- Radosevich PM, Ring EJ, LaBerge JM, et al. Transjugular intrahepatic portosystemic shunts in patients with portal vein occlusion. Radiology 1993; 186:523.
- Villa E, Cammà C, Marietta M, et al. Enoxaparin prevents portal vein thrombosis and liver decompensation in patients with advanced cirrhosis. Gastroenterology 2012; 143:1253.
- Orr DW, Harrisson PM, Karani J, et al. Chronic portomesenteric and portosplenomesenteric venous thrombosis: evaluation of long term follow-up and determinants of survival. Hepatology 2005; 42:212A.
- CLINICAL MANIFESTATIONS
- Physical examination
- Laboratory testing
- Abdominal imaging
- Abdominal ultrasound
- Abdominal CT
- Abdominal MRI
- Identification of predisposing conditions
- DIFFERENTIAL DIAGNOSIS
- Screening for varices
- - Efficacy
- Portal cholangiopathy
- Management of complications
- PREVENTION IN PATIENTS WITH CIRRHOSIS
- SUMMARY AND RECOMMENDATIONS