Acute portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management
- 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 and 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). Occlusion of the portal vein by thrombus (portal vein thrombosis [PVT]) typically occurs in patients with cirrhosis and/or prothrombotic disorders (table 1). Patients with acute PVT have the sudden onset of portal venous occlusion due to thrombus. The occlusion may be complete or partial. In addition to involving the portal vein, the clot may also involve the mesenteric veins or the splenic vein. Patients with acute PVT have not yet developed features of chronic PVT, such as collateral circulation (eg, cavernous portal transformation) or portal hypertension. If it is not known when the clot developed, but the patient does not have features of chronic PVT, the PVT can be referred to as being "recent" . Patients with recent PVT are managed the same as those with acute PVT.
This topic will review the clinical manifestations, diagnosis, and management of acute PVT. The epidemiology and pathogenesis of PVT, as well as the approach to patients with chronic PVT are discussed elsewhere. (See "Epidemiology and pathogenesis of portal vein thrombosis in adults" and "Chronic portal vein thrombosis in adults: Clinical manifestations, diagnosis, and management".)
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.
The clinical manifestations of acute portal vein thrombosis (PVT) depend on the extent of the obstruction and the speed of its development.
Symptoms — Acute PVT may be clinically silent and diagnosed during a radiologic examination for other reasons (such as acute pancreatitis) (image 1). Other patients may have abdominal pain that develops suddenly or progresses over a few days . Patients may also report fever and dyspeptic symptoms. Patients with cirrhosis may present with variceal bleeding. The presence of spiking fevers, chills, and a painful liver is suggestive of septic PVT (acute pylephlebitis). In addition to symptoms related to the PVT, patients may also have symptoms related to conditions that predispose to the development of PVT, such as acute pancreatitis. (See "Pylephlebitis", section on 'Clinical manifestations'.)
- Condat B, Pessione F, Helene Denninger M, et al. Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy. Hepatology 2000; 32:466.
- DeLeve LD, Valla DC, Garcia-Tsao G, American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009; 49:1729.
- Primignani M. Portal vein thrombosis, revisited. Dig Liver Dis 2010; 42:163.
- Subramanyam BR, Balthazar EJ, Lefleur RS, et al. Portal venous thrombosis: correlative analysis of sonography, CT and angiography. Am J Gastroenterol 1984; 79:773.
- Hidajat N, Stobbe H, Griesshaber V, et al. Imaging and radiological interventions of portal vein thrombosis. Acta Radiol 2005; 46:336.
- Marn CS, Francis IR. CT of portal venous occlusion. AJR Am J Roentgenol 1992; 159:717.
- Bach AM, Hann LE, Brown KT, et al. Portal vein evaluation with US: comparison to angiography combined with CT arterial portography. Radiology 1996; 201:149.
- Mathieu D, Vasile N, Grenier P. Portal thrombosis: dynamic CT features and course. Radiology 1985; 154:737.
- 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.
- Van Gansbeke D, Avni EF, Delcour C, et al. Sonographic features of portal vein thrombosis. AJR Am J Roentgenol 1985; 144:749.
- Tessler FN, Gehring BJ, Gomes AS, et al. Diagnosis of portal vein thrombosis: value of color Doppler imaging. AJR Am J Roentgenol 1991; 157:293.
- Marshall MM, Beese RC, Muiesan P, et al. Assessment of portal venous system patency in the liver transplant candidate: a prospective study comparing ultrasound, microbubble-enhanced colour Doppler ultrasound, with arteriography and surgery. Clin Radiol 2002; 57:377.
- 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.
- Tublin ME, Dodd GD 3rd, Baron RL. Benign and malignant portal vein thrombosis: differentiation by CT characteristics. AJR Am J Roentgenol 1997; 168:719.
- 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.
- Pannach S, Babatz J, Beyer-Westendorf J. Successful treatment of acute portal vein thrombosis with rivaroxaban. Thromb Haemost 2013; 110:626.
- Lenz K, Dieplinger B, Buder R, et al. Successful treatment of partial portal vein thrombosis (PVT) with low dose rivaroxaban. Z Gastroenterol 2014; 52:1175.
- Potze W, Adelmeijer J, Lisman T. Decreased in vitro anticoagulant potency of Rivaroxaban and Apixaban in plasma from patients with cirrhosis. Hepatology 2015; 61:1435.
- Intagliata NM, Henry ZH, Maitland H, et al. Direct Oral Anticoagulants in Cirrhosis Patients Pose Similar Risks of Bleeding When Compared to Traditional Anticoagulation. Dig Dis Sci 2016; 61:1721.
- Plessier A, Darwish-Murad S, Hernandez-Guerra M, et al. Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study. Hepatology 2010; 51:210.
- Turnes J, García-Pagán JC, González M, et al. Portal hypertension-related complications after acute portal vein thrombosis: impact of early anticoagulation. Clin Gastroenterol Hepatol 2008; 6:1412.
- Amitrano L, Guardascione MA, Scaglione M, et al. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses. Am J Gastroenterol 2007; 102:2464.
- Sogaard KK, Astrup LB, Vilstrup H, Gronbaek H. Portal vein thrombosis; risk factors, clinical presentation and treatment. BMC Gastroenterol 2007; 7:34.
- Delgado MG, Seijo S, Yepes I, et al. Efficacy and safety of anticoagulation on patients with cirrhosis and portal vein thrombosis. Clin Gastroenterol Hepatol 2012; 10:776.
- Martinelli I, Primignani M, Aghemo A, et al. High levels of factor VIII and risk of extra-hepatic portal vein obstruction. J Hepatol 2009; 50:916.
- Blum U, Haag K, Rössle M, et al. Noncavernomatous portal vein thrombosis in hepatic cirrhosis: treatment with transjugular intrahepatic portosystemic shunt and local thrombolysis. Radiology 1995; 195:153.
- Cherukuri R, Haskal ZJ, Naji A, Shaked A. Percutaneous thrombolysis and stent placement for the treatment of portal vein thrombosis after liver transplantation: long-term follow-up. Transplantation 1998; 65:1124.
- Hänig V, Stenzel G, Rössle M. [Acute portal vein thrombosis in liver cirrhosis: successful recanalization with the use of a portosystemic shunt (TIPS)]. Rofo 1996; 165:403.
- Mann O, Haag K, Hauenstein KH, et al. [Septic portal vein thrombosis. Its successful therapy by local fibrinolysis and a transjugular portasystemic stent-shunt (TIPS)]. Dtsch Med Wochenschr 1995; 120:1201.
- Hall TC, Garcea G, Metcalfe M, et al. Management of acute non-cirrhotic and non-malignant portal vein thrombosis: a systematic review. World J Surg 2011; 35:2510.
- Hollingshead M, Burke CT, Mauro MA, et al. Transcatheter thrombolytic therapy for acute mesenteric and portal vein thrombosis. J Vasc Interv Radiol 2005; 16:651.
- Baril N, Wren S, Radin R, et al. The role of anticoagulation in pylephlebitis. Am J Surg 1996; 172:449.
- Sheen CL, Lamparelli H, Milne A, et al. Clinical features, diagnosis and outcome of acute portal vein thrombosis. QJM 2000; 93:531.
- Lagasse JP, Bahallah ML, Salem N, et al. [Acute thrombosis of the portal system. Treatment with alteplase and heparin or with heparin alone in 10 patients]. Gastroenterol Clin Biol 1997; 21:919.
- Grisham A, Lohr J, Guenther JM, Engel AM. Deciphering mesenteric venous thrombosis: imaging and treatment. Vasc Endovascular Surg 2005; 39:473.
- Joh JH, Kim DI. Mesenteric and portal vein thrombosis: treated with early initiation of anticoagulation. Eur J Vasc Endovasc Surg 2005; 29:204.
- Plessier A, Murad SD, Hernandez-Guerra M, et al. A prospective multicentric follow-up study on 105 patients with acute portal vein thrombosis (PVT): results from the European Network for Vascular Disorders of the LIver (EN-Vie). Hepatology 2007; 46:310A.
- Kumar S, Sarr MG, Kamath PS. Mesenteric venous thrombosis. N Engl J Med 2001; 345:1683.
- CLINICAL MANIFESTATIONS
- Physical examination
- Laboratory testing
- Abdominal imaging
- Abdominal CT or MRI
- Abdominal ultrasound with Doppler imaging
- Identification of predisposing conditions
- DIFFERENTIAL DIAGNOSIS
- Efficacy of anticoagulation
- Duration of anticoagulation
- Complications of anticoagulation
- Alternatives to anticoagulation
- SUMMARY AND RECOMMENDATIONS