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2015 ; 3
(6
): 489-500
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Budd-Chiari syndrome
#MMPMID26668741
Martens P
; Nevens F
United European Gastroenterol J
2015[Dec]; 3
(6
): 489-500
PMID26668741
show ga
Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder
characterized by obstruction of the hepatic outflow tract at any level between
the junction of the inferior vena cava with the right atrium and the small
hepatic veins. 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-invasive treatment regimen. The
application of these treatment strategies allows for a five-year 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-transplant lymphoma in transplant patients.