Budd-Chiari syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]
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Overview
The Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein or inferior vena cava. Its presents with the classical triad of abdominal pain, ascites and hepatomegaly. Examples of occlusion include thrombosis of hepatic veins and membranous webs in the inferior vena cava. The syndrome can be fulminant, acute, chronic, or asymptomatic.
Historical Perspective
Budd-Chiari syndrome (BCS) was first discovered by George Budd in 1845. In 1899, Hans Chiari was the first to describe the histopathology of Budd-Chiari syndrome (BCS).
Classification
Budd-Chiari syndrome may be classified into several subtypes based on etiology, disease duration, severity and anatomical location of the occlusion.An obstruction below 300µm in diameter is not considered as BCS by some authors.Budd-Chiari syndrome may be classified according to etiology into two subtypes: primary and secondary.Budd-Chiari syndrome may be classified according to disease duration and severity into four subtypes: acute, subacute, chronic, fulminant liver failure.Budd-Chiari syndrome may be classified according to the anatomical location of obstruction into 3 subtypes: type I - truncal type, type II - radicular type, type III - venooclusive type.
Pathophysiology
Occlusion of at least two hepatic veins leads to Budd-Chiari syndrome. Single hepatic vein occlusion is usually silent. Obstruction in the venous drainage from liver results in venous congestion causing hepatomegaly.Patients develop postsinusoidal portal hypertension.Stasis of blood and congestion cause hypoxic damage of hepatocytes, affecting the liver function.This can result in centrilobular fibrosis, nodular regenerative hyperplasia and ultimately cirrhosis.Hepatocellular necrosis results from increased sinusoidal pressure.Budd-Chiari is commonly associated with atrophy of peripheral regions and enlargement of the caudate lobe because blood is directly shunted through it into the inferior vena cava.Genes associated with increased expression in the pathogenesis of Budd-Chiari syndrome include Matrix metalloproteinase 7, superior cervical ganglion 10 (SCG10), proliferating cell nuclear antigen gene,c-MYC oncogene,tumor protein p53 gene,thrombospondin-1 gene.
Causes
Budd-Chiari syndrome is associated with a wide range of etiologies.On the basis of underlying cause Budd- Chairi can be Primary(75%) caused bythrombosis of the hepatic vein and Secondary (25%) caused by invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts).Causes include myeloproliferative disorders, malignancy, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, factor V Leiden mutation, infections and benign liver lesions, oral contraceptives and pregnancy, hypercoagulable states, Behçet's syndrome, membranous webs.
Differentiating Budd-Chiari syndrome overview from Other Diseases
Epidemiology and Demographics
In 2009-2013, the prevalence of Budd-Chiari syndrome was estimated to be 424 patients cases per 5.29 per million (100,000) individuals in South Korea. Budd-Chiari syndrome is a rare disease. The incidence of Budd-Chiari syndrome was estimated to be about 1 case per million population per year 100,000 individuals in Sweden. The annual case fatality rate of Budd-Chiari syndrome is approximately 2.8%. Budd-Chiari syndrome commonly presents in the third or fourth decade of life. The median age at diagnosis is 51 years. Budd-Chiari syndrome is rare in children. Females are more commonly affected with Budd-Chiari syndrome than males. The female-to-male ratio is approximately 1.8 to 1. Budd-Chiari syndrome in the United States is predominantly seen in women and is commonly associated with hematologic disorders.Congenital membranous forms is the most common cause of Budd-Chiari syndrome worldwide and particularly in Asia.
Risk Factors
Approximately 80 percent of patients with the Budd-Chiari syndrome have an underlying disorder.Many patients may have more than one risk factor. Same patient may have multiple causes that predispose to the development of Budd-Chiari Syndrome. Associated risk factors include hematologic disorders, coagulopathies, chronic infections, chronic inflammatory diseases, tumors, congenital membranous obstructions.
Screening
There is insufficient evidence to recommend routine screening for Budd-Chiari syndrome.
Natural History, Complications, and Prognosis
Natural History
The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain.Without treatment, the patient will develop complications like hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, Complications secondary to hepatic decompensation, which will/ may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.
Complications
Complications that can develop as a result of Budd-Chiari syndrome are portal hypertension, hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, hepatic decompensation, bacterial peritonitis, especially the following paracentesis.
Prognosis
If left untreated, patients with Budd-Chiari syndrome have a high mortality rate.About 90% die within 3 years as the disease may progress to develop intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.Prognosis is generally good with treatment, and the 5-year survival rate of patients with treatment of Budd-Chiari syndrome is approximately 74 %.Poor prognostic factors include older age at diagnosis, chronic disease, severe liver failure and associated refractory ascites.
Diagnosis
CT
Computed tomography (CT) or magnetic resonance imaging (MRI) is sometimes employed although these methods are generally not as sensitive.
Diagnostic Criteria
History and Symptoms
The hallmark of Budd-Chiari syndrome is a triad of right upper quadrant abdominal pain, ascites, and hepatomegaly. Symptoms of Budd-Chiari syndrome include fever, abdominal pain, abdominal distension from ascites, lower extremity edema, jaundice, gastrointestinal bleeding from varices, portal hypertensive gastropathy, hepatic encephalopathy. Patients with subacute or chronic Budd-Chiari syndrome may be asymptomatic.
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
A minority of patients can be treated medically with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The transjugular intrahepatic portosystemic shunt (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from angioplasty. [1] Limited studies on thrombolysis with direct infusion of urokinase and tissue plasminogen activator (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.
Surgery
Prevention
References
- ↑ Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.