Budd-Chiari Syndrome
Although most clinicians may not recognize BCS, understanding the trio of symptoms — abdominal pain, hepatomegaly, and ascites — can improve the likelihood of an accurate diagnosis and facilitate optimal treatment - Mary-Kate Hunnicutt PAC, Alicia Elam PharmD & Michael Felz MD
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Budd-Chiari Syndrome: A Case of Hepatomegaly, Abdominal Pain, and Ascites
George Budd first described the clinical picture of BCS in 1845 and Hans Chiari described the underlying histopathology half a century later.1 A multifactorial disease process characterized by hepatic venous outflow obstruction, BCS occurs in approximately 1.4 million people in the United States.2,3 An underlying disorder can be identified in more than 80% of these patients.
Causes of BCS include myeloproliferative syndromes (MPS), malignancy, infection, Behҫet syndrome, as well as miscellaneous other conditions and idiopathic causes. BCS often is the initial presentation of an MPS, and approximately 50% of BCS cases may be due to an underlying MPS, such as essential thrombocytopenia,…
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Budd-Chiari Syndrome (Including Hepatic Vein Thrombosis)
Budd-Chiari syndrome (BCS) refers to a partial or complete obstruction of the hepatic venous outflow tract that is not due to heart failure, pericardial disease, or sinusoidal obstruction syndrome.
State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review
The clinical presentation of BCS varies widely from fulminant to asymptomatic presentations. The principal hallmark features of BCS are hepatomegaly, ascites, abdominal pain, and the presence of dilated superficial abdominal wall veins. Manifestation of clinical signs and features mainly depends on the degree of involvement of HVs and IVC. Fulminant presentation occurs when all three HVs are obstructed, and its incidence rate is very low
Articles of Interest
Budd Chiari Syndrome: ED Presentation, Evaluation, and Management
The liver is one of the most metabolically active organs in our body, and as such it is particularly susceptible to vascular insults. In Budd-Chiari syndrome (BCS), obstruction of the liver circulation usually occurs at the level of the hepatic veins and inferior vena cava. Presentation of the syndrome may be fulminant, acute, subacute, or chronic.
Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis
Budd-Chiari syndrome (BCS) is a rare hepatic venous disease. It presents with thrombosis, located anywhere from the hepatic veins (HV) to the suprahepatic of the inferior vena cava (IVC). The result is an outflow obstruction of hepatic veins1,2. The obstruction of BCS is classified as primary or secondary depending on the site of hepatic vein obstruction. The obstruction site can be a thrombus inside the vein or outside the vein due to compression with tumors3.
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Life in the Fastlane
Hepatic venous outflow obstruction causing portal hypertension.
StatPearls
Hepatic venous outflow obstruction due to any cause except the cardiac ones regardless of the level of obstruction is known as Budd-Chiari syndrome. To improve outcomes associated with this condition, prompt diagnosis and treatment are essential.
UMEM Educational Pearls
Conditions to consider in the critically ill pregnant patient who presents with acute RUQ pain include acute fatty liver of pregnancy (AFLP), liver infarction, liver hematoma, and Budd-Chiari Syndrome.
