Imaging of Budd-Chiari syndrome

Buckley, Orla; Brien, Joanna; Snow, Aisling; Stunell, Helen; Lyburn, Iain; Munk, Peter L.; Torreggiani, W C

doi:10.1007/s00330-006-0537-2

Cited by 43 publications

(27 citation statements)

References 29 publications

(29 reference statements)

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“…MRI is not as effective as Doppler ultrasonography in documenting intrahepatic collaterals, 41 but MRI and CT are able to better visualize the hepatic parenchyma demonstrating areas of reduced perfusion or necrosis. 42 MRI and CT offer high spatial resolution, clearly documenting the surrounding anatomy, thereby providing the multidisciplinary team with appropriate images to use for therapeutic planning, such as scheduling a transjugular intrahepatic portosystemic shunt (TIPS). 1 There are no data available regarding the diagnostic superiority of MRI over CT; however, MRI seems more useful in patients with renal impairment and younger patients given the avoidance of radiation.…”

Section: Diagnostic Workupmentioning

confidence: 99%

Budd‐Chiari syndrome

Martens

Nevens

2015

UEG Journal

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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.

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Section: Diagnostic Workupmentioning

confidence: 99%

Budd‐Chiari syndrome

Martens

Nevens

2015

UEG Journal

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“…Though the results of Doppler-ultrasound are operator-dependent, its estimated diagnostic yield is 90%, according to recent studies [12][13][14] , or even greater with advanced technical devices such as contrast ultrasound [15] . The diagnostic yield was estimated to be 87% in our series.…”

Section: Discussionmentioning

confidence: 99%

Aetiological factors of Budd-Chiari syndrome in Algeria

Afredj¹

2015

WJH

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AIM:To study the clinical presentation of Budd-Chiari syndrome (BCS) and identify the aetiologies of this disease in Algeria. METHODS:Patients with BCS, hospitalised in our unit from January 2004 until June 2010 were included and the aetiological factors were assessed. Patients presenting a BCS in the setting of advanced-stage cirrhosis or a liver transplantation were excluded from the study. The diagnosis was established when an obstruction of hepatic venous outflow (thrombosis, stenosis or compression) was demonstrated. We diagnosed myeloproliferative disease (MPD) by bone marrow biopsy and V617F JAK2 mutation. Anti-phospholipid syndrome (APLS) was detected by the presence of anticardiolipin antibodies, anti-β2 glycoprotein antibodies and Lupus anticoagulant. We also detected paroxysmal nocturnal haemoglobinuria (PNH) by flow cytometry. Celiac disease and Behçet disease were systematically investigated in our patients. Hereditary anticoagulant protein deficiencies were also assessed. We tested our patients for the G20210A mutation at Beaujon Hospital. Imaging procedures were performed to determine a local cause of BCS, such as a hydatid cyst or a liver tumour. RESULTS: Aetiological factors of Budd-Chiari syndrome in AlgeriaProspective Study ORIGINAL ARTICLE63.5%. The revealing symptoms for the BCS were ascites (74.8%) and abdominal pain (42.6%). The most common site of thrombosis was the hepatic veins (72.2%). Involvement of the inferior vena cava alone was observed in 3 patients. According to the radiological investigations, BCS was primary in 94.7% of the cases (n = 109) and secondary in 5.2% (n = 6). An aetiology was identified in 77.4% of the patients (n = 89); it was multifactorial in 27% (n = 31). The predominant aetiology of BCS in our patients was a myeloproliferative disease, observed in 34.6% of cases. APLS was found in 21.7% and celiac disease in 11.4%. Other acquired conditions were: PNH (n = 4), systemic disease (n = 6) and inflammatory bowel disease (n = 5). Anticoagulant protein deficiency was diagnosed in 28% of the patients (n = 18), dominated by protein C deficiency (n = 13). Secondary BCS was caused by a compressing hydatic cyst (n = 5) and hepatocellular carcinoma (n = 1). CONCLUSION:The main aetiologic factor of BCS in Algeria is MPD. The frequency of celiac disease justifies its consideration when BCS is diagnosed in our region.

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“…Ascites, right upper quadrant pain, and hepatosplenomegaly are the classic findings of this disorder. 2 The primary pathogenesis of the disease is increased intrasinusoidal pressure due to hepatic outflow obstruction and a delay or reversal of the portal venous flow. 3 The degree of the hepatic cellular injury depends primarily on the acuteness of the obstruction of the flow.…”

Section: Discussionmentioning

confidence: 99%