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.
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
- right upper quadrant abdominal pain
Other diagnostic factors
- gastrointestinal bleeding
- leg edema
- truncal venous collaterals
- hepatic encephalopathy
- female sex
- myeloproliferative disorders (MPD)
- thrombophilic disorders
- oral contraceptive pill use
- pregnancy and immediate postpartum period
1st investigations to order
- thrombophilia screening
- color and pulsed Doppler ultrasound
- BUN 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
- Sinusoidal obstruction syndrome (SOS) (veno-occlusive disease)
- Fulminant hepatic failure due to other etiologies
- Congestive hepatopathy
- Vascular diseases of the liver
- AASLD Practice Guidelines: vascular disorders of the liver
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