From the Guidelines
Budd-Chiari syndrome typically presents with symptoms of liver congestion and portal hypertension, including abdominal pain, hepatomegaly, ascites, and edema, as a result of hepatic venous outflow obstruction 1. The clinical manifestations of Budd-Chiari syndrome can vary, ranging from an insidious form with progressive ascites to a hyperacute presentation with liver failure 1.
Common Symptoms
- Abdominal pain, particularly in the upper right quadrant
- Hepatomegaly (enlarged liver)
- Ascites (fluid accumulation in the abdomen)
- Edema (swelling in the legs)
- Nausea and vomiting
- Jaundice (yellowing of the skin and eyes)
- Visible dilated veins on the abdomen In severe cases, patients can develop bleeding from esophageal varices, mental confusion from hepatic encephalopathy, and signs of liver failure 1.
Pathophysiology
The pathophysiological consequences of Budd-Chiari syndrome include obstruction, which leads to sinusoidal congestion, ischemia, and finally hepatocellular necrosis, resulting in centrilobular fibrosis, nodular regenerative hyperplasia, and/or cirrhosis 1. The syndrome occurs when blood flow from the liver is obstructed, typically due to blood clots in the hepatic veins or inferior vena cava, causing blood to back up in the liver, leading to increased pressure, congestion, and eventual liver damage 1. Early diagnosis is crucial as untreated Budd-Chiari syndrome can progress to cirrhosis and liver failure, potentially requiring liver transplantation 1.
From the Research
Budd-Chiari Syndrome Symptoms
The symptoms of Budd-Chiari syndrome (BCS) can vary widely, but they are generally caused by the obstruction of the hepatic venous outflow 2. Some common symptoms include:
- Abdominal pain
- Ascites (fluid accumulation in the abdomen)
- Hepatomegaly (enlargement of the liver)
- Portal hypertension (high blood pressure in the portal vein)
Diagnosis and Treatment
BCS is typically diagnosed using imaging techniques such as contrast CT-angiography and/or Doppler ultrasound 3. Treatment options for BCS include:
- Anticoagulation therapy to prevent further blood clots
- Transjugular intrahepatic portosystemic shunt (TIPS) to improve blood flow through the liver
- Angioplasty and stenting to open up blocked blood vessels
- Liver transplantation in severe cases
Treatment Outcomes
Studies have shown that early radiological intervention and haematology screening can lead to excellent outcomes in BCS patients 4. Anticoagulation therapy, TIPS, and liver transplantation have all been shown to be effective in managing BCS, with overall survival rates of 96% at 1 year and 81% at 5 years 4. Direct oral anticoagulants (DOACs) have also been shown to be effective and safe for long-term anticoagulation in BCS patients, with a transplant-free survival rate of 91.6% at 5 years 5.
Risk Factors
BCS can be caused by a variety of underlying conditions, including: