eMedicine Specialties > Gastroenterology > Liver

Budd-Chiari Syndrome

Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
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

Updated: Sep 9, 2008

Introduction

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.

Clinical

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

Differential Diagnoses

Pericarditis, Constrictive

Other Problems to Be Considered

Right-sided heart failure
Metastatic liver disease
Alcoholic liver disease
Granulomatous liver disease

Workup

Laboratory 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

• Liver biopsy

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.

Treatment

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.

Medication

Anticoagulation is needed in some patients, especially those with underlying hematological disorders as the cause of the syndrome.

Anticoagulants

Prevent recurrent or ongoing thromboembolic occlusion.

Warfarin (Coumadin)

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.

Dosing

Adult

5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR

Pediatric

0.05-0.34 mg/kg/d PO; adjust dose according to desired INR

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

Contraindications

Documented hypersensitivity; severe liver or kidney disease; open wounds; GI ulcers

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

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

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.

Streptokinase (Kabikinase, Streptase)

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.

Dosing

Adult

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

Administer as in adults

Interactions

Antifibrinolytic agents may decrease effects; heparin, warfarin, and aspirin may increase risk of bleeding

Contraindications

Documented hypersensitivity; active internal bleeding, intracranial neoplasm, aneurysm, diathesis, or severe uncontrolled arterial hypertension

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

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

Urokinase (Abbokinase)

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.

Dosing

Adult

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

Pediatric

Administer as in adults

Interactions

Thrombolytic enzymes, alone or in combination with anticoagulants and antiplatelets, may increase risk of bleeding complications

Contraindications

Documented hypersensitivity; internal bleeding, recent trauma, history of intracranial or intraspinal surgery or trauma, cerebrovascular accident, and intracranial neoplasm

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

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

Alteplase (Activase)

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.

Dosing

Adult

0.25-0.50 mg/kg IV over 60 min

Pediatric

Administer as in adults

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

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

Precautions

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

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

Follow-up

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

• Long-term anticoagulation is often needed, and it may also be needed after liver transplantation.

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%.

Miscellaneous

Medicolegal 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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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

Contributor Information and Disclosures

Author

Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Praveen K Roy, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and Canadian Association of Gastroenterology
Disclosure: Nothing to disclose.

Coauthor(s)

Abhishek Choudhary, MD, Resident, Department of Internal Medicine, University Hospital of Missouri
Abhishek Choudhary, MD is a member of the following medical societies: American College of Physicians
Disclosure: Nothing to disclose.

Homayoun Shojamanesh, MD, Former Fellow, Digestive Diseases Branch, National Institutes of Health
Homayoun Shojamanesh, MD is a member of the following medical societies: American Gastroenterological Association, American Medical Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Jack Bragg, DO, FACOI, Assistant Professor, Department of Clinical Medicine, University of Missouri School of Medicine
Jack Bragg, DO, FACOI is a member of the following medical societies: American College of Osteopathic Internists and American Osteopathic Association
Disclosure: Nothing to disclose.

Gautam Dehadrai, MD, Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital
Gautam Dehadrai, MD is a member of the following medical societies: American College of Radiology, Medical Council of India, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

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
Robert J Fingerote, MD, MSc, BSc, FRCPC is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

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Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
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Managing Editor

James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine
James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

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
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
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