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Gastroenterology > Liver
Budd-Chiari Syndrome
Article Last Updated: Sep 9, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Praveen K Roy is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and Canadian Association of Gastroenterology
Coauthor(s):
Abhishek Choudhary, MD, Resident, Department of Internal Medicine, University Hospital of Missouri;
Homayoun Shojamanesh, MD, Former Fellow, Digestive Diseases Branch, National Institutes of Health;
Jack Bragg, DO, FACOI, Assistant Professor, Department of Clinical Medicine, University of Missouri School of Medicine;
Gautam Dehadrai, MD, Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital
Editors: Robert J Fingerote, MD, MSc, BSc, FRCPC, Consultant, Clinical Evaluation Division, Biologic and Gene Therapies, Directorate Health Canada; Consulting Staff, Department of Medicine, Division of Gastroenterology, York Central Hospital, Richmond Hill, Ontario; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine; Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine; Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Author and Editor Disclosure
Synonyms and related keywords:
Budd-Chiari syndrome, hepatic vein occlusion, hepatic vein obstruction, hepatic obstruction, liver obstruction, liver disease, hepatic disease, thrombotic hepatic vein obstruction, nonthrombotic hepatic vein obstruction, non-thrombotic hepatic vein obstruction, obliterating hepatic vein endophlebitis, hepatomegaly, ascites, thrombotic diathesis, congestive hepatopathy, hepatic congestion, liver congestion, membranous webs, acute liver disease, subacute liver disease, fulminant liver disease, liver failure
Background
Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction to hepatic venous outflow. Budd described it in 1845, and Chiari added the first pathologic description of a liver with "obliterating endophlebitis of the hepatic veins" in 1899. Hepatomegaly, ascites, and abdominal pain characterize Budd-Chiari syndrome. The syndrome most often occurs in patients with underlying thrombotic diathesis, including myeloproliferative disorders, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria, pregnancy, tumors, chronic inflammatory diseases, clotting disorders, and infections. See related CME at The Management of Paroxysmal Nocturnal Hemoglobinuria: Recent Advances in Diagnosis and Treatment and New Hope for Patients.
Pathophysiology
Obstruction of intrahepatic veins leads to congestive hepatopathy. This results from obstruction of large- or small-caliber veins, which leads to hepatic congestion as blood flows into, but not out of, the liver. Hepatocellular injury results from microvascular ischemia due to congestion. Portal hypertension and liver insufficiency result.
Frequency
United States
Budd-Chiari syndrome is rare, but the exact frequency is unknown.
International
Internationally, Budd-Chiari syndrome is also rare, but the exact frequency is unknown. Membranous webs are a common cause of Budd-Chiari syndrome in Asian countries.
Mortality/Morbidity
Budd-Chiari syndrome is a potentially fatal disorder, if untreated.
Race
The syndrome occurs in persons of all races.
Sex
The syndrome is equally present in both sexes. Emergent presentation is more common in women than in men.
Age
Age at presentation is usually the third or fourth decade of life, although the condition may also occur in children or elderly persons.
History
The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients but is nonspecific. A high index of suspicion is needed to make the diagnosis. Four main clinical variants have been described: acute liver disease, subacute liver disease, fulminant liver disease, and liver failure. The most common presentation is subacute liver disease complicated by portal hypertension and varying degrees of liver decompensation. - Acute and subacute form: These forms are characterized by rapid development of abdominal pain, ascites, hepatomegaly, jaundice, and renal failure.
- Chronic form: This form of presentation is the most common. Patients present with progressive ascites. Jaundice is absent, and approximately 50% of patients also have renal impairment.
- Fulminant form: This form of presentation is uncommon. Fulminant or subfulminant hepatic failure is present along with ascites, tender hepatomegaly, jaundice, and renal failure.
Physical
Physical examination may reveal the following findings:
- Icterus
- Ascites
- Hepatomegaly
- Splenomegaly
- Ankle edema
- Stasis ulcerations
- Prominence of collateral veins
Causes
Most patients have an underlying thrombotic diathesis. In approximately one third of patients, an underlying cause is not evident. The causes of Budd-Chiari syndrome are as follows: - Hematological disorders
- Polycythemia rubra vera
- Paroxysmal nocturnal hemoglobinuria
- Unspecified myeloproliferative disorder
- Antiphospholipid antibody syndrome
- Essential thrombocytosis
- Inherited thrombotic diathesis
- Protein C deficiency
- Protein S deficiency
- Antithrombin III deficiency
- Factor V Leiden deficiency
- Pregnancy and postpartum
- Membranous webs
- Oral contraceptives
- Chronic infections
- Hydatid cysts
- Aspergillosis
- Amebic abscess
- Syphilis
- Tuberculosis
- Chronic inflammatory diseases
- Behçet disease
- Inflammatory bowel disease
- Sarcoidosis
- Systemic lupus erythematosus
- Sjögren syndrome
- Mixed connective-tissue disease
- Tumors
- Hepatocellular carcinoma
- Renal cell carcinoma
- Leiomyosarcoma
- Adrenal carcinoma
- Wilms tumor
- Right atrial myxoma
- Miscellaneous
- Alpha1-antitrypsin deficiency
- Trauma
- Dacarbazine
- Urethane
- Idiopathic
Pericarditis, Constrictive
Other Problems to be Considered
Right-sided heart failure
Metastatic liver disease
Alcoholic liver disease
Granulomatous liver disease
Lab Studies
- Examination of ascitic fluid provides useful clues to the diagnosis.
- Patients usually have high protein concentrations (>2 g/dL). This may not be present in persons with the acute form of Budd-Chiari syndrome.
- The WBC count is usually less than 500/µL.
- The serum ascites–albumin gradient is usually less than 1.1 (except in the acute forms of the disease).
- Routine biochemical test results are usually nonspecific. Mild elevations in serum aminotransferase and alkaline phosphatase levels are present in 25-50% of patients.
- Hematological studies should be performed to evaluate for a hypercoagulable state.
- See Polycythemia Vera; Thrombocytosis, Essential; Protein C Deficiency; Protein S Deficiency; and Myeloproliferative Disease for more information.
Imaging Studies
- Ultrasound
- Thrombi can be visualized.
- Color-flow Doppler ultrasound is the preferred mode. Sensitivity and specificity are 85-90%.
- Magnetic resonance imaging (MRI) scanning with pulsed sequencing
- MRI images help in the assessment of hepatic venous blood flow.
- Sensitivity and specificity are 90%.
- Hepatic venography
- Thrombi are observed in hepatic veins.
- With venography, hepatic vein orifices cannot be cannulated.
Procedures
Histologic Findings
Pathological findings after liver biopsy are (1) high-grade venous congestion and centrilobular liver cell atrophy, and, possibly, (2) thrombi within the terminal hepatic venules. The extent of fibrosis can be determined based on biopsy findings.
Medical Care
Medical therapy can be instituted for short-term, symptomatic benefit. Medical therapy alone is associated with a high 2-year mortality rate (80-85%). - Management of ascites: See Ascites for more information.
- Anticoagulation
- Antithrombolytic therapy: This therapy has been used in a few cases. Agents include streptokinase, urokinase, recombinant tissue plasminogen activator, and other modalities.
- Angioplasty: This can help relieve obstruction caused by membranous webs.
Surgical Care
Decompression of the hepatic vasculature should be offered if portal hypertension is the cause of the symptoms. Either surgery or a transjugular intrahepatic portosystemic shunt procedure can be performed. Liver transplantation should be offered if decompensated liver cirrhosis is present (see Liver Transplantation for more information).
Consultations
- Gastroenterologist
- Hematologist
- Surgeon
- Radiologist
Diet
A low-sodium diet is recommended for the control of ascites.
Anticoagulation is needed in some patients, especially those with underlying hematological disorders as the cause of the syndrome.
Drug Category: Anticoagulants
Prevent recurrent or ongoing thromboembolic occlusion.
| Drug Name | Warfarin (Coumadin) |
|---|
| Description | Interferes with hepatic synthesis of vitamin K–dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders. Tailor dose to maintain an INR in the range of 2-3. |
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| Adult Dose | 5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR |
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| Pediatric Dose | 0.05-0.34 mg/kg/d PO; adjust dose according to desired INR |
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| Contraindications | Documented hypersensitivity; severe liver or kidney disease; open wounds; GI ulcers |
|---|
| Interactions | Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate
Medications that may increase anticoagulant effects include oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac |
|---|
| Pregnancy | X - Contraindicated; benefit does not outweigh risk
|
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| Precautions | Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients who have protein C or S deficiency are at risk of developing skin necrosis |
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Drug Category: Fibrinolytic agents
Used to dissolve a pathologic intraluminal thrombus or embolus that has not been dissolved by the endogenous fibrinolytic system. Also used for the prevention of recurrent thrombus formation and for the rapid restoration of hemodynamic disturbances.
| Drug Name | Streptokinase (Kabikinase, Streptase) |
|---|
| Description | Acts with plasminogen to convert plasminogen to plasmin. Plasmin degrades fibrin clots, fibrinogen, and other plasma proteins. Increase in fibrinolytic activity that degrades fibrinogen levels for 24-36 h occurs with intravenous infusion. |
|---|
| Adult Dose | Not established; can be administered locally via catheter or IV
Local: 7500 U/h
IV: 100,000 U/h after loading dose of 250,000 U bolus |
|---|
| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; active internal bleeding, intracranial neoplasm, aneurysm, diathesis, or severe uncontrolled arterial hypertension |
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| Interactions | Antifibrinolytic agents may decrease effects; heparin, warfarin, and aspirin may increase risk of bleeding |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
|
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| Precautions | Caution in severe hypertension, IM administration of medications, or trauma or surgery in the previous 10 d; measure hematocrit, platelet count, aPTT, TT, PT, or fibrinogen levels before therapy is implemented; either TT or aPTT should be less than twice the normal control value following infusion of streptokinase and before instituting or reinstituting heparin; do not take blood pressure in lower extremities because may dislodge possible deep vein thrombi; PT, aPTT, TT, or fibrinogen should be monitored 4 h after initiation of therapy |
|---|
| Drug Name | Urokinase (Abbokinase) |
|---|
| Description | Direct plasminogen activator that acts on the endogenous fibrinolytic system and converts plasminogen to the enzyme plasmin, which, in turn, degrades fibrin clots, fibrinogen, and other plasma proteins. Most often used for local fibrinolysis of thrombosed catheters and superficial vessels. Advantage is that agent is nonantigenic. However, more expensive than streptokinase and thus limits use. When used for local fibrinolysis, urokinase is given as local infusion directly into area of thrombus and with no bolus given. Dose should be adjusted to achieve clot lysis or patency of affected vessel. |
|---|
| Adult Dose | Can be given locally or systemically
Loading dose: 4400 U/kg IV over 10 min and increase to 6000 U/kg/h
Maintenance dose: 4400-6000 U/kg/h IV |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; internal bleeding, recent trauma, history of intracranial or intraspinal surgery or trauma, cerebrovascular accident, and intracranial neoplasm |
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| Interactions | Thrombolytic enzymes, alone or in combination with anticoagulants and antiplatelets, may increase risk of bleeding complications |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
|
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| Precautions | Caution in patients receiving IM administration of medications, severe hypertension, and trauma or surgery in previous 10 d; to avoid dislodging possible deep vein thrombi, do not measure blood pressure in lower extremities; monitor therapy by measuring PT, aPTT, TT, or fibrinogen level approximately 4 h after initiation of therapy |
|---|
| Drug Name | Alteplase (Activase) |
|---|
| Description | Tissue plasminogen activator used in management of acute myocardial infarction, acute ischemic stroke, and pulmonary embolism. Safety and efficacy with concomitant administration of heparin or aspirin during first 24 h after symptom onset have not been investigated. |
|---|
| Adult Dose | 0.25-0.50 mg/kg IV over 60 min |
|---|
| Pediatric Dose | Administer as in adults |
|---|
| Contraindications | Documented hypersensitivity; active internal bleeding, cerebrovascular accident or stroke within last 2 mo, intracranial or intraspinal surgery or trauma, intracranial hemorrhage upon pretreatment evaluation, possible subarachnoid hemorrhage, intracranial neoplasm, arteriovenous malformation or aneurysm, bleeding diathesis, or severe uncontrolled hypertension |
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| Interactions | Anticoagulants and antiplatelets may increase risk of bleeding; may give heparin with and after alteplase infusions to reduce risk of rethrombosis; either heparin or alteplase may cause bleeding complications |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Monitor for bleeding, especially at arterial puncture sites, with coadministration of vitamin K antagonists; control and monitor blood pressure frequently during and following administration (when managing acute ischemic stroke); do not use >0.9 mg/kg to manage acute ischemic stroke; doses >0.9 mg/kg may cause ICH |
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Further Inpatient Care
- Gastroscopy should be performed to help rule out the presence of esophageal and gastric varices. If present, they may be obliterated with banding or sclerotherapy. Nonselective beta-blockers (eg, propranolol, nadolol) can be administered for primary prophylaxis against variceal bleeding.
- Electrolyte levels should be monitored closely because diuretics and other factors can cause electrolyte imbalances.
- Prothrombin time and activated partial thromboplastin time should be monitored once anticoagulation is started. They should be maintained within the therapeutic range.
Further Outpatient Care
Transfer
- Once a patient is determined to be a transplant candidate, transfer to a liver transplant unit.
Complications
- Complications secondary to hepatic decompensation
- Hepatic encephalopathy
- Variceal hemorrhage
- Hepatorenal syndrome
- Portal hypertension
- Complications secondary to hypercoagulable state
Prognosis
- The natural history is not well known. The following factors have been associated with a good prognosis:
- Younger age at diagnosis
- Low Child-Pugh score
- Absence of ascites or easily controlled ascites
- Low serum creatinine level
- A formula to calculate the prognostic index has been proposed. A score of less than 5.4 is associated with a good prognosis. The formula to calculate the prognostic index is as follows:
Prognostic index = (ascites score X 0.75) + (Pugh score X 0.28) + (age X 0.037) + (creatinine level X 0.0036) - The prognosis is poor in patients with Budd-Chiari syndrome who remain untreated. Death results from progressive liver failure in 3 months to 3 years from the time of diagnosis.
- The 5-year survival rate is 38-87% following portosystemic shunting. The actuarial 5-year survival rate following liver transplantation is 70%.
Medical/Legal Pitfalls
- Failure to perform hematological studies to evaluate for a hypercoagulable state
- Failure to offer long-term anticoagulation if indicated
- Failure to offer liver transplantation if indicated
- Failure to decompress the hepatic vasculature if portal hypertension is the cause of symptoms
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Budd-Chiari Syndrome excerpt Article Last Updated: Sep 9, 2008
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