Primary Budd-Chiari syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assessment of V617F Janus tyrosine kinase 2 (JAK2) mutation in peripheral granulocytes. A diagnosis of Budd-Chiari syndrome should be considered in any patient presenting with acute or chronic liver disease, as clinical manifestations are extremely diverse. Spontaneous outcome in symptomatic patients is poor. Diagnosis can be made in most patients noninvasively when imaging shows venous obstruction and/or collaterals. A treatment strategy is recommended where anticoagulation is given first, followed by angioplasty when appropriate, then TIPS in patients not responding to previous measure, and finally liver transplantation. This strategy has achieved 5-year survival rates close to 90%.
[Show abstract][Hide abstract] ABSTRACT: To evaluate the type of venous involvement in Chinese Budd-Chiari syndrome (BCS) patients and the relative diagnostic accuracy of the different imaging modalities.
Using digital subtraction angiography (DSA) as a reference standard, color Doppler ultrasound (CDUS), computed tomography angiography (CTA), and magnetic resonance angiography (MRA) were performed on 338 patients with BCS. We analyzed the course of the main and any accessory hepatic veins (HVs) and the inferior vena cava (IVC) to assess the etiology of obstructed segments and diagnostic accuracy of CDUS, CTA and MRA.
Among the 338 cases, there were 8 cases (2.4%) of isolated IVC membranous obstruction, 45 cases (13.3%) of isolated HV occlusion, and 285 cases (84.3%) with both IVC membranous obstruction and HV occlusion. Comparing with DSA, CDUS, CTA had a diagnostic accuracy of 89.3% and 80.2% in detecting BCS, and 83.4% of cases correctly correlated by MRA.
In Henan Province, most patients with BCS have complex lesions combining IVC and HV involvement. The combination of CDUS and CTA or MRI is useful for diagnosis of BCS and guiding therapy.
PLoS ONE 01/2014; 9(1):e85135. DOI:10.1371/journal.pone.0085135 · 3.23 Impact Factor
"Therapeutic options include the positioning of shunts or IVC bypasses, the radical membrane resection with thrombus extraction, thrombolysis, angioplasty, stenting, and anticoagulation . Progressed BCS may be associated with the development of liver cirrhosis and different focal liver nodes. "
[Show abstract][Hide abstract] ABSTRACT: A-26-year old female patient with chronic Budd-Chiari syndrome due to different underlying blood disorders applied for a two-year followup of the liver with Gadolinium-ethoxybenzyl-diethylenetriaminepentaacetic-acid-(Gd-EOB-DTPA-) enhanced MRI. The liver function tests were raised. Besides showing a progressive hepatosplenomegaly and a cirrhotic liver alteration, the MRI revealed multiple new nodular lesions in all liver segments. These lesions showed typical patterns in the precontrast images, while there was an arterial and a persistent portal venous enhancement. In the hepatobiliary liver-specific late phase, a central "washout" and a persistent rim enhancement were observed (target sign). The additionally performed contrast-enhanced ultrasonography showed a strong zentrifugal arterial enhancement of the lesions followed by an isoechoic enhancement in the portal venous and delayed liver phase. Histologically these lesions turned out as focal nodular hyperplasias (FNH) or FNH-like lesions, also known as large regenerative nodules (LRNs). Differentiation between regenerative nodules like LRN and hepatocellular carcinoma (HCC) in cirrhotic livers is crucial, and the target sign in the hepatobiliary phase of Gd-EOB-DTPA as well as the centrifugal arterial enhancement followed by an isoenhancement during a CEUS might be useful for establishing the correct diagnosis of such hypervascular lesions with proliferated and likely aberrant bile ducts.
"Hepatic or portal vein thrombosis is a rare disease, usually caused by multiple concurrent factors, including acquired and inherited thrombophilias . Long term anticoagulation therapy with vitamin K antagonists (VKA) is currently recommended in this clinical setting with a target international normalized ratio (INR) comprised between 2 and 3 . "
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