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 per cent of patients have at least one underlying hypercoagulable state.
Colour 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 radiological 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.
International expert panels have agreed that 'Budd-Chiari syndrome' (BCS) should be used as an eponym for 'hepatic venous outflow tract obstruction', independent of the level or mechanism of obstruction. Cardiac and pericardial diseases, and sinusoidal obstruction syndrome are excluded from this definition.
History and exam
Key diagnostic factors
- presence of risk factors
- right upper quadrant abdominal pain
Other diagnostic factors
- gastrointestinal bleeding
- leg oedema
- truncal venous collaterals
- hepatic encephalopathy
- female sex
- myeloproliferative disorders (MPD)
- thrombophilic disorders
- oral contraceptive pill use
- pregnancy and immediate post-partum period
1st investigations to order
- thrombophilia screening
- colour and pulsed Doppler ultrasound
- urea and creatinine
- coagulation profile
- Janus kinase 2 (JAK2) mutation
- flow cytometry for CD55- and CD59-deficient blood cells
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
Soha Saoud Abdel Moneim, MD, PhD
Lecturer of Gastroenterology and Hepatology
SSAM declares that she has no competing interests.
Vijay H. Shah, MD
Professor of Medicine
Physiology and Cancer Cell Biology
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.
Howard J. Worman, MD
Professor of Medicine and Cell Biology
Columbia University College of Physicians and Surgeons
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
PN declares that he has no competing interests.
Muhammad Dawwas, MRCP
- Sinusoidal obstruction syndrome (SOS) (veno-occlusive disease)
- Fulminant hepatic failure due to other aetiologies
- Congestive hepatopathy
- Asia-Pacific working group consensus on non-variceal upper gastrointestinal bleeding: an update 2018
- Vascular diseases of the liver
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