Budd-Chiari syndrome

Last reviewed: 23 Aug 2023
Last updated: 07 Jan 2020

Summary

Definition

History and exam

Key diagnostic factors

  • right upper quadrant abdominal pain
  • hepatomegaly
  • ascites
  • jaundice
More key diagnostic factors

Other diagnostic factors

  • splenomegaly
  • gastrointestinal bleeding
  • leg edema
  • truncal venous collaterals
  • hepatic encephalopathy
Other diagnostic factors

Risk factors

  • female sex
  • myeloproliferative disorders (MPD)
  • thrombophilic disorders
  • oral contraceptive pill use
  • pregnancy and immediate postpartum period
More risk factors

Diagnostic investigations

1st investigations to order

  • thrombophilia screening
  • color and pulsed Doppler ultrasound
  • LFTs
  • BUN and creatinine
  • coagulation profile
  • CBC
  • Janus kinase 2 (JAK2) mutation
  • flow cytometry for CD55- and CD59-deficient blood cells
More 1st investigations to order

Investigations to consider

  • serum-ascites albumin gradient (SAAG)
  • total ascitic protein
  • CT abdomen
  • MRI abdomen
  • hepatic venography
  • contrast-enhanced 3-dimensional magnetic resonance angiography
  • transjugular liver biopsy
  • bone marrow biopsy
More investigations to consider

Treatment algorithm

ACUTE

primary: nonfulminant

primary: fulminant

secondary BCS

Contributors

Authors

Soha Saoud Abdel Moneim, MD, PhD
Soha Saoud Abdel Moneim

Lecturer of Gastroenterology and Hepatology

Assiut University

Assiut

Egypt

Disclosures

SSAM declares that she has no competing interests.

Vijay H. Shah, MD
Vijay H. Shah

Professor of Medicine

Physiology and Cancer Cell Biology

Mayo Clinic

Rochester

MN

Disclosures

VHS is the author of studies referenced in this topic. VHS is on the advisory boards of the following companies: Novartis Pharmaceuticals, Merck and Company, Enterome Corporation, Durect Corporation, Afimmune Ltd., Vital Therapies.

Peer reviewers

Howard J. Worman, MD

Professor of Medicine and Cell Biology

Columbia University College of Physicians and Surgeons

New York

NY

Disclosures

HJW declares that he has no competing interests.

Philip Newsome, PhD, FRCPE

Senior Lecturer in Hepatology & Honorary Consultant Physician

Liver Research Group

Institute of Biomedical Research

The Medical School

University of Birmingham

Birmingham

UK

Disclosures

PN declares that he has no competing interests.

Muhammad Dawwas, MRCP

Specialist Registrar

Liver Unit

Addenbrooke's Hospital

Cambridge

UK

Disclosures

Not disclosed.

  • Budd-Chiari syndrome images
  • Differentials

    • Sinusoidal obstruction syndrome (SOS) (veno-occlusive disease)
    • Fulminant hepatic failure due to other etiologies
    • Congestive hepatopathy
    More Differentials
  • Guidelines

    • Vascular diseases of the liver
    • AASLD Practice Guidelines: vascular disorders of the liver
    More Guidelines
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