Budd-Chiari Syndrome – A review of the diagnosis and management

2011 ◽  
Vol 10 (1) ◽  
pp. 5-9
Author(s):  
MA Fox ◽  
◽  
JA Fox ◽  
MH Davies ◽  
◽  
...  
Keyword(s):  
Liver Disease ◽  
Abdominal Discomfort ◽  
Budd Chiari Syndrome ◽  
Outflow Obstruction ◽  
Venous Outflow ◽  
Hepatic Venous Outflow Obstruction ◽  
Key Points ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow ◽  
Venous Outflow Obstruction

Budd-Chiari syndrome (BCS) is the liver disease resulting from hepatic venous outflow obstruction comprising a triad of abdominal discomfort, hepatomegaly and ascites. Advances in the management of this disorder over the last three decades have dramatically improved survival. We present a review of the management of BCS followed by a case which illustrates some key points in the diagnosis and treatment of this condition.

scholarly journals The Spectrum of Histologic Findings in Hepatic Outflow Obstruction

2016 ◽  
Vol 141 (1) ◽  
pp. 98-103 ◽  
Author(s):  
Raul S. Gonzalez ◽  
Michael A. Gilger ◽  
Won Jae Huh ◽  
Mary Kay Washington
Keyword(s):  
Portal Tract ◽  
Central Vein ◽  
Budd Chiari Syndrome ◽  
Outflow Obstruction ◽  
Venous Outflow ◽  
Ductular Reaction ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow ◽  
Venous Outflow Obstruction ◽  
Hematoxylin And Eosin

Context.—Cardiac hepatopathy and Budd-Chiari syndrome are 2 forms of hepatic venous outflow obstruction with different pathophysiology but overlapping histologic findings, including sinusoidal dilation and centrilobular necrosis. Objective.—To determine whether a constellation of morphologic findings could help distinguish between the 2 and could suggest the diagnoses in previously undiagnosed patients. Design.—We identified 26 specimens with a diagnosis of cardiac hepatopathy and 23 with a diagnosis of Budd-Chiari syndrome. Slides stained with hematoxylin and eosin and with trichrome were evaluated for several distinctive histologic findings. Results.—Features common to both forms of hepatic outflow obstruction included sinusoidal dilation and portal tract changes of fibrosis, chronic inflammation, and bile ductular reaction. Histologic findings significantly more common in cardiac hepatopathy included pericellular/sinusoidal fibrosis and fibrosis around the central vein. Only centrilobular hepatocyte dropout/necrosis was significantly more common in Budd-Chiari, regardless of duration. Conclusions.—The finding of pericellular/sinusoidal fibrosis in cardiac hepatopathy compared with Budd-Chiari is not unexpected, given the chronic nature of most cardiac hepatopathy. Portal tract changes are common in both forms of hepatic outflow obstruction and should not deter one from making the diagnosis of hepatic outflow obstruction. Fibrosis along sinusoids and around the central vein may be suggestive of cardiac hepatopathy in biopsies from patients without a prior diagnosis.

scholarly journals Hepatic Venous Outflow Obstruction in Polycystic Liver Disease

HPB ◽  
2021 ◽  
Vol 23 ◽  
pp. S156
Author(s):  
E. Buckarma ◽  
A. Glasgow ◽  
E. Habermann ◽  
S. Venkatesh ◽  
J. Fidler ◽  
...  
Keyword(s):  
Liver Disease ◽  
Polycystic Liver Disease ◽  
Outflow Obstruction ◽  
Venous Outflow ◽  
Hepatic Venous Outflow Obstruction ◽  
Polycystic Liver ◽  
Hepatic Venous Outflow ◽  
Venous Outflow Obstruction

scholarly journals Hepatic Venous Outflow Obstruction: Suggestion of a New Classification

2017 ◽  
Vol 1 (2) ◽  
pp. 41-51
Author(s):  
Santosh Man Shrestha
Keyword(s):  
Vena Cava ◽  
Sinusoidal Obstruction Syndrome ◽  
New Classification ◽  
Outflow Obstruction ◽  
Venous Outflow ◽  
Hepatic Venous Outflow Obstruction ◽  
Chiari Syndrome ◽  
Hepatic Venous Outflow ◽  
Venous Outflow Obstruction

Hepatic venous outflow obstruction (HVOO) is common in developing countries. It is a serious condition that clinically manifests with ascites from sinusoidal hypertension, and carries the risk of high mortality or development of liver cirrhosis. In the past, the eponym Budd–Chiari Syndrome (BCS) was used synonymously for HVOO. In the West, hepatic vein (HV) thrombosis caused by prothrombotic disorders is the main cause of HVOO. In the East, obliterative disease of hepatic portion of inferior vena cava induced by bacterial infection, now renamed hepatic vena cava syndrome, is the common cause of HVOO. These two diseases with different etiology, epidemiology, and natural history are at present grouped together under BCS causing much confusion. Sinusoidal obstruction syndrome, another important cause of HVOO at the level of the sinusoid and terminal HV, was left out in the classification of HVOO. In this article, the pathophysiology of sinusoidal hypertension is described, the term BCS is redefined, and a new classification of HVOO is suggested.

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