Budd–Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging

Faraoun, S.A.; Boudjella, Mohamed El Amine; Debzi, N.; Afredj, Nawel; Guerrache, Youcef; Benidir, Naima; Bouzid, C.; Bentabak, K.; Soyer, Philippe; Bendib, S.E.

doi:10.1007/s00261-015-0380-5

Cited by 32 publications

(14 citation statements)

References 24 publications

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“… 11 This technique allows the evaluation of the hepatic veins and the inferior vena cava blood flows that may be decreased, reversed, or absent in the BCS. 5 , 11 – 13 Stenosis or dilation of these veins and the caudate lobe hypertrophy are other signs 12 (the latter, although non-specific, is present in 75% of cases). 1 In chronic cases, collateral veins are observed in the hepatic veins and/or inferior vena cava.…”

Section: Discussionmentioning

confidence: 99%

“…Magnetic resonance imaging is usually helpful in the differentiation of acute and chronic cases, and it helps to better evaluate the involvement of the inferior vena cava. 13 The gold standard examination, for the diagnosis of BCS, is the hepatic venography that should be performed when the suspicion is high and any other imaging examination has failed to elucidate the problem. 2 , 13 , 14 …”

Section: Discussionmentioning

confidence: 99%

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Budd Chiari Syndrome: an unnoticed diagnosis

Falcão¹,

Fagundes²,

Lamos³

et al. 2015

ACR

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Budd–Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Budd Chiari Syndrome: an unnoticed diagnosis

Falcão¹,

Fagundes²,

Lamos³

et al. 2015

ACR

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“…Sonography is well correlated with the pathologic findings or results of invasive imaging . Characteristic findings of BCS include obstruction and stenosis recognized at the main trunk of the hepatic vein and hepatic segment of the IVC …”

Section: Clinical Features Diagnosis and Prognosis Of Bcsmentioning

confidence: 99%

Budd–Chiari syndrome: Focus on surgical treatment

Hidaka

Eguchi

2016

Hepatology Research

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Budd-Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC. Liver transplantation (LT) is usually indicated as a treatment for liver failure despite various treatments. The outcomes of LT are good, with a 5-year survival after LT of nearly 70%.

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“…The number of patients receiving treatment (the number of patients with confirmed disease) in 2004 was estimated to be between 190 [108][109][110][111][112][113][114][115][116][117][118] Ultrasound Doppler examination occasionally showed regurgitation or turbulence at the main trunk of the hepatic vein and the hepatic segment of the inferior vena cava, and blood flow waves of the hepatic vein may become flat or defective. 108-113 (b) Swelling of the spleen is recognized.…”

Section: Epidemiologymentioning

confidence: 99%

Diagnosis and treatment guidelines for aberrant portal hemodynamics

Furuichi

Tanaka

Takikawa

et al. 2017

Hepatology Research

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Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.

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Budd–Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging

Abstract: Our results indicate that BCS is a chronic and insidious disease, more often discovered at an advanced stage. These results should warrant further evaluation of screening strategies in patients with risk factors for BCS to identify the disease at an early stage.

Cited by 32 publications

References 24 publications

Budd Chiari Syndrome: an unnoticed diagnosis

Budd Chiari Syndrome: an unnoticed diagnosis

Budd–Chiari syndrome: Focus on surgical treatment

Diagnosis and treatment guidelines for aberrant portal hemodynamics

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