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In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less\u2010invasive treatment regimen. The application of these treatment strategies allows for a five\u2010year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post\u2010transplant lymphoma in transplant patients.<\/jats:p>","DOI":"10.1177\/2050640615582293","type":"journal-article","created":{"date-parts":[[2015,4,29]],"date-time":"2015-04-29T01:22:30Z","timestamp":1430270550000},"page":"489-500","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":86,"title":["Budd\u2010Chiari syndrome"],"prefix":"10.1002","volume":"3","author":[{"given":"Pieter","family":"Martens","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederik","family":"Nevens","sequence":"additional","affiliation":[{"name":"Department of Liver and Biliopancreatic Disorders University Hospital Gasthuisberg KU Leuven Leuven 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