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Figure 1. Computed tomography showing ascites, splenomegaly and typical patchy reduced perfusion of the liver. The portal vein is patent.
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Figure 2. Computed tomography with a typical small enhancing regenerative BCS nodule in the left lobe.
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Figure 3. Management of nodules in Budd-Chiari syndrome. AFP: Alpha-fetoprotein; BCS: Budd-Chiari syndrome. Data taken from [31].
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Figure 4. Treatment algorithm for Budd-Chiari syndrome. HCC: Hepatocellular carcinoma; TIPSS: Transjugular intrahepatic portosystemic stent-shunt.
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Figure 5. Transhepatic needle puncture of left hepatic vein, recanalization and stent insertion. (A) Venogram after transhepatic needle puncture of obstructed left hepatic vein. (B) Venogram of same patient after recanalization and stent insertion.
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The Budd-Chiari syndrome (BCS) was first described by a British physician, William Budd in 1845 in his seminal work 'Diseases of the Liver'. He reported the case of a man who died in King's College Hospital, London, in February 1844. All the hepatic veins seemed thicker and more opaque than natural and on examining them closely found a thin false membrane on their inner surface which in the large veins could be readily stripped off [1]. Budd did not associate any clinical features with this finding. The description of the clinical features of hepatic vein outflow obstruction is generally attributed to a pathologist, Hans Chiari (although he was not the first). In 1899, Chiari described an "obliterating endophlebitis of the hepatic veins"and its association with hepatomegaly, ascites and abdominal pain [2].
The BCS initially referred to symptomatic occlusion of the hepatic veins, but later also to obstructive changes in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices. The term 'obliterative hepatocavopathy'has been used to describe obstruction primarily affecting the hepatic portion of the IVC. More recently, the nomenclature has been clarified following the meeting of an expert study group. BCS is defined as hepatic venous outflow obstruction at any level from the small hepatic veins (HV) to the junction of the IVC and the right atrium, regardless of the cause of obstruction. Outflow obstruction caused by the sinusoidal obstruction syndrome (formerly 'veno-occlusive disease') and cardiac disorders is excluded [3].
The focus of...