Clinics and Research in Hepatology and Gastroenterology
Practice guidelinesRecent (non-cirrhotic) extrahepatic portal vein obstruction
Introduction
Recent non-cirrhotic extrahepatic portal vein obstruction (REHPVO) is defined as the recent occurrence of a thrombus in the portal vein and/or in the right or left portal branches. The thrombosis can extend to the mesenteric or splenic veins. Occlusion may be total or partial, and may occur in patients with a past history of obstruction of part of the portal venous system. The estimated incidence of recent or chronic portal vein thrombosis in adults is 3/100,000 inhabitants in Europe [1], [2]. The diagnosis of recent extrahepatic portal vein obstruction is rare in children and it is most often made at the chronic stage of portal cavernoma. This chapter describes REHPVO in the absence of cirrhosis and/or malignancy.
Section snippets
What are the causes of REHPVO?
Recent extrahepatic portal vein obstruction is often caused by a combination of local and general risk factors. A general prothrombotic disorder or a local factor is detected in approximately 60% and 30% of patients, respectively. Although several factors may be identified in the same patient, no cause is identified in one third of cases. Ideally, testing for thrombophilia should be performed before anticoagulants are started, but this should not delay the initiation of treatment. Local causes
What is the treatment of REHPVO?
The goal of treatment aims is to limit the extension of thrombosis, mesenteric infarction, and later to achieve recanalization to avoid the complications of portal hypertension. In adults, anticoagulants and treatment of the identified cause are begun as soon as thrombosis has been confirmed, in the absence of bleeding.
Recommendations
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consider a diagnosis of REHPVO in any patients with abdominal pain (B1);
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screen for REHPVO in the high risk new-born population: sepsis on umbilical vein catheterization, omphalitis, or antenatal diagnosis of hepatic vascular malformation (B1);
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perform first-line Doppler-US to detect REHPVO. Rapidly perform CT-scan to confirm the diagnosis, to assess for thrombus extension, signs of bowel infarction, and local causes (A1);
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rule out cirrhosis and malignant disease (B1);
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assess associated
Disclosure of interest
The authors declare that they have no competing interest.
Acknowledgements
We gratefully acknowledge the contributions of Dale Roche-Lebrec, Emilie Le Beux and Livia Etzol who reviewed the manuscript before the submission of the final version of the manuscript.
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