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Budd Chiari Syndrome & VOD

Key Differences in Budd-Chiari Syndrome (BCS) Between West and East:

Feature West East
Membranous obstruction of the IVC Rare Frequent
Hepatic vein occlusion predominates Yes (+) No (-)
IVC occlusion predominates No (-) Yes (+)
Acute or subacute BCS predominates Yes (+) No (-)
Chronic BCS predominates No (-) Yes (+)
Pregnancy/postpartum Uncommon Frequent
Infection Rare Common
Oral contraceptives Frequent Uncommon
Myeloproliferative disease Common Rare

Summary of Patterns:

  • In the West, hepatic vein occlusion and acute/subacute forms of Budd-Chiari Syndrome predominate. Oral contraceptives and myeloproliferative diseases are common predisposing factors.
  • In the East, IVC (inferior vena cava) occlusion and chronic BCS are more common. Membranous obstruction of the IVC, infection [ Hepatic Amebiasis], and pregnancy/postpartum states are frequent causes.

Concise CT Features of Budd-Chiari Syndrome (BCS)

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  1. Hepatomegaly (especially caudate lobe hypertrophy).
  2. Ascites due to portal hypertension.
  3. Heterogeneous liver enhancement (patchy hypoattenuation).
  4. Thrombus in hepatic veins or IVC.
  5. Collateral vessel formation in chronic cases.
  6. Splenomegaly secondary to portal hypertension.

Clinical Presentation of Budd-Chiari Syndrome:

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  1. Fulminant:
    • Rare.
    • Associated with high fatality.
  2. Acute:
    • Short history (less than 6 months).
    • Presents with tender hepatomegaly and ascites.
  3. Subacute:
    • Symptoms persist for 6 months to 1 year.
    • Development of collateral circulation.
    • Minimal ascites.
  4. Chronic:
    • Associated with variceal hemorrhage, intractable ascites, and hepatic encephalopathy (HE).

Treatment Options for Budd-Chiari Syndrome (BCS):

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  1. Thrombolytic Therapy:
    • Used to dissolve thrombi that are obstructing hepatic veins or the inferior vena cava (IVC).
  2. Interventional Radiology:
    • TIPS (Transjugular Intrahepatic Portosystemic Shunt): Creates a channel between the portal and systemic circulations to reduce portal hypertension.
    • IVC Stenting: Used when the inferior vena cava is obstructed or narrowed, to improve blood flow.
  3. Portosystemic Shunts:
    • Portocaval Shunt: Connects the portal vein to the IVC to bypass the hepatic venous system.
    • Mesocaval Shunt: Connects the superior mesenteric vein to the IVC.
    • Mesoatrial Shunt: Connects the superior mesenteric vein to the right atrium.
    • Portocaval + Cavoatrial Shunt: A combination of shunts for severe cases.

  4. Transplant:

    • Liver transplantation is considered in cases of severe liver dysfunction or failure when other treatments are not effective.

    Indications for Liver Transplantation (LTP) in Budd-Chiari Syndrome (BCS):

    1. Cirrhosis with progressive liver failure:
      • Life expectancy (LE) less than 1 year due to advanced liver disease.
    2. Failure of a portosystemic shunt or TIPS:
      • When previous attempts at decompression (shunt or TIPS) do not adequately control portal hypertension or prevent disease progression.
    3. BCS with unshuntable portal hypertension:
      • In cases where the portal venous system is not suitable for shunting procedures due to extensive thrombosis or anatomical limitations.
    4. Acute fulminant hepatic failure:
      • In patients presenting with acute liver failure due to BCS, where rapid deterioration requires urgent liver transplantation.

Objectives of Nonoperative Therapy in Budd-Chiari Syndrome (BCS):

  1. Remove the cause of venous thrombosis:
    • Address underlying conditions such as hypercoagulable states, infections, or other factors contributing to thrombosis.
  2. Relieve high pressure and congestion within the liver:
    • Reduce portal hypertension and venous congestion, often achieved with TIPS or other interventional procedures.
  3. Prevent extension of the venous thrombosis:
    • Use anticoagulation or thrombolytic therapy to stop the clot from growing and obstructing more of the venous system.
  4. Reverse the massive ascites:
    • Manage ascites through diuretics, paracentesis, or shunting procedures to improve fluid balance and relieve symptoms.

ORLOFF Experience:

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Veno-Occlusive Disease (VOD) - Key Points

Pathophysiology:

  • Subendothelial sclerosis of the hepatic veins and sinusoids.
  • Occurs secondary to endothelial injury caused by:
    • Toxic agents: Pyrrolizidine alkaloids.
    • Antineoplastic drugs (chemotherapy).
    • Radiation.
    • Stem cell transplantation (common post-transplant complication).

Pathogenesis:

  • Plasminogen Activator Inhibitor-1 (PAI-1) plays a key role in the pathogenesis by promoting fibrosis and thrombosis.

Treatment:

  • Defibrotide: The most promising therapy, works by protecting the endothelial cells and promoting fibrinolysis.

Prophylaxis:

  • Heparin and Prostaglandin E1 (PGE1).
  • Defibrotide can also be used as prophylaxis to prevent VOD, especially in high-risk patients undergoing stem cell transplantation.

These key points summarize the causes, pathogenesis, treatment, and prophylaxis of Veno-Occlusive Disease.

MCQ Discussion - Budd-Chiari Syndrome (BCS)

Question 1:

Budd-Chiari Syndrome:

a) Causes presinusoidal portal hypertension

b) Can be associated with a lupus anticoagulant

c) Is more common in men

d) Does not involve hepatic veins

Answer: B

  • Budd-Chiari syndrome can be associated with a lupus anticoagulant, which is one of the prothrombotic conditions contributing to venous thrombosis in BCS.

Question 2:

The triad of Budd-Chiari syndrome (BCS) is:

a) Abdominal pain, Ascites, Hepatomegaly

b) Abdominal pain, Jaundice, Hepatomegaly

c) Abdominal pain, Ascites, Jaundice

d) Jaundice, Ascites, Hepatomegaly

Answer: A

  • The classic triad of Budd-Chiari Syndrome includes abdominal pain, ascites, and hepatomegaly.

Question 3:

Which does not cause secondary BCS?

a) Syphilitic gumma

b) Aspergillosis

c) Filariasis

d) None of the above

Answer: D

  • All of the options (syphilitic gumma, aspergillosis, filariasis) can potentially cause secondary Budd-Chiari syndrome through various mechanisms, such as infection and fibrosis.

Question 4:

Predictor of survival following TIPS in BCS (Specific):

a) MELD

b) СТР

c) Apache II

d) Garcia-Pagan score

Answer: D

  • The Garcia-Pagan score is a specific predictor of survival following TIPS (Transjugular Intrahepatic Portosystemic Shunt) in Budd-Chiari Syndrome.

Question 5:

Not a cause of venoocclusive disease:

a) Consumption of bush tea

b) Cytosine arabinoside

c) Mushroom poisoning

d) BM transplant

Answer: C

  • Mushroom poisoning is not typically associated with veno-occlusive disease. Causes include bush tea consumption, cytosine arabinoside (chemotherapy), and bone marrow transplant.