Budd-Chiari syndrome

Last reviewed: September 2018

Last updated: December 2017

Summary

Includes hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium, regardless of the cause of the obstruction.

Classic triad of symptoms is abdominal pain, ascites, and hepatomegaly.

Seventy-five percent of patients have at least one underlying hypercoagulable state.

Color and pulsed Doppler ultrasonography is the first-line investigation for diagnosis. Patients should also undergo testing for hypercoagulable states to identify any predisposition to venous thrombosis.

The aim of therapy and interventional management is to relieve hepatic congestion and prevent necrosis, fibrosis, and ultimately liver failure, cirrhosis, and/or portal hypertension.

The main treatment options are radiologic interventional procedures including angioplasty of hepatic vein and/or inferior vena cava, or transjugular intrahepatic portosystemic shunts (TIPS). Further to this, surgical procedures may be used, such as surgical shunts and liver transplantation.

Medical treatment in the form of anticoagulation therapy, diuretics, and treatment of underlying thrombophilic condition is given parallel to interventional therapy.

Untreated, the syndrome has a reported mortality of 80%. In the past few decades, prognosis has dramatically improved due to advances and new modalities in diagnosis and treatment of the disease.

Definition

International expert panels have agreed that “Budd-Chiari syndrome” should be used as an eponym for “hepatic venous outflow tract obstruction”, independent of the level or mechanism of obstruction. [1] Cardiac and pericardial diseases, and sinusoidal obstruction syndrome are excluded from this definition.

History and exam

Key diagnostic factors

RUQ abdominal pain
hepatomegaly
ascites
jaundice

Full details

Other diagnostic factors

splenomegaly
GI bleeding
leg edema
truncal venous collaterals
hepatic encephalopathy

Full details

Risk factors

female gender
myeloproliferative disorders (MPD)
thrombophilic disorders
oral contraceptive pill use
pregnancy and immediate postpartum period

Full details

Diagnostic investigations

1st investigations to order

thrombophilia screening
color and pulsed Doppler ultrasound
LFT
BUN and creatinine
coagulation profile
CBC
Janus kinase 2 (JAK2) mutation
flow cytometry for CD55- and CD59-deficient blood cells

Full details

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

Full details

Treatment algorithm

ACUTE

Contributors

Authors VIEW ALL 

Soha Saoud Abdel Moneim, MD, PhD

Lecturer of Gastroenterology and Hepatology

Assiut University

Assiut

Egypt

Disclosures

SSAM declares that she has no competing interests.

Vijay H. Shah, MD

Professor of Medicine

Physiology and Cancer Cell Biology

Mayo Clinic

Rochester

Disclosures

VHS is the author of studies referenced in this monograph.

Peer reviewers VIEW ALL 

Howard J. Worman, MD

Professor of Medicine and Cell Biology

Columbia University College of Physicians and Surgeons

New York

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

Disclosures

PN declares that he has no competing interests.

Muhammad Dawwas, MRCP

Specialist Registrar

Liver Unit

Addenbrooke's Hospital

Cambridge

Disclosures

Not disclosed.

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Budd-Chiari syndrome

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

primary: nonfulminant

primary: fulminant

secondary BCS

Contributors

Authors VIEW ALL 

Soha Saoud Abdel Moneim, MD, PhD

Disclosures

Vijay H. Shah, MD

Disclosures

Peer reviewers VIEW ALL 

Howard J. Worman, MD

Disclosures

Philip Newsome, PhD, FRCPE

Disclosures

Muhammad Dawwas, MRCP

Disclosures

Differentials

Guidelines

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