eMedicine Specialties > Gastroenterology > Liver

Budd-Chiari Syndrome

Author: Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
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
Contributor Information and Disclosures

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

More on Budd-Chiari Syndrome

Overview: Budd-Chiari Syndrome
Differential Diagnoses & Workup: Budd-Chiari Syndrome
Treatment & Medication: Budd-Chiari Syndrome
Follow-up: Budd-Chiari Syndrome
References
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Further Reading

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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

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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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

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
Disclosure: Nothing to disclose.

 
 
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