Budd-Chiari Syndrome

Aka: Budd-Chiari Syndrome, Budd Chiari Syndrome, Hepatic Venous Outflow Tract Obstruction

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II. Definitions

  1. Budd-Chiari Syndrome (BCS)
    1. Hepatic Venous Outflow Tract Obstruction within IVC or hepatic veins, and due to non-cardiac cause
    2. Primary BCS are conditions affecting the venous lumen (e.g. thrombosis)
    3. Secondary BCS are external conditions that invade or compress venous outflow (hepatic veins, IVC)

III. Epidemiology

  1. Incidence: 0.1 to 10 per Million (rare)
  2. Age: Peaks age 20 to 40 years
  3. Gender Predominance
    1. Non-asian countries: Women (hepatic vein obstruction)
    2. Asian countries: Men (Inferior vena cava or hepatic vein obstruction)

IV. Pathophysiology

  1. Venous congestion occurs when more than one hepatic vein is obstructed
  2. Lymphatics return a part of the excessive fluid
  3. Hepatic sinusoids dilate with congestion
    1. Hepatomegaly
    2. Right upper quadrant pain (liver capsule stretch)
    3. Ascites (leakage of fluid from liver capsule into peritoneum)
  4. Portal venous Hypertension
    1. Centrilobular hepatocyte Hypoxia
  5. Fibrosis and Hepatic failure with longterm obstruction
    1. Caudate liver lobe is most commonly affected by Budd-Chiari (due to shunting to IVC)

V. Causes

  1. Idiopathic in 20% of cases
  2. Hypercoagulable State
    1. Contributes to most of the other Budd-Chiari Syndrome causes
    2. Malignancy
    3. Pregnancy
    4. Oral Contraceptives
    5. Inherited conditions
      1. Factor V (Leiden) mutation
      2. Antiphospholipid Antibody Syndrome
      3. Antithrombin III Deficiency
      4. Protein C Deficiency
      5. Paroxysmal Nocturnal Hemoglobinuria
  3. Myeloproliferative Disorders
    1. Polycythemia Vera
    2. Essential Thrombocythemia
  4. Malignancy (10% of cases)
    1. Mass compresses or invades venous outflow
    2. Hepatocellular Carcinoma
    3. Renal Cell Carcinoma
    4. Adrenal Cancer
    5. Leiomyosarcoma
    6. Right atrial Myxoma
    7. Wilms tumor
  5. Other Compressive Masses
    1. Hepatic Cysts
    2. Abscess
    3. Aortic aneurysm

VI. Findings: Presentations

  1. Acute
    1. Rapid developoment over weeks (too rapidly to have developed collateral venous drainage)
    2. Significant acute hepatic necrosis
    3. Presents with Abdominal Pain, Jaundice, severe Ascites
    4. Hepatic Encephalopathy and fulminant liver failure may occur is some cases
  2. Subacute
    1. Gradual onset over months with more mild symptoms
    2. Collateral venous drainage develops and spares hepatocyte necrosis and minimal Ascites
  3. Chronic
    1. Cirrhosis may be present
    2. Collateral venous drainage is well developed
    3. Progressive Ascites
    4. Jaundice is typically absent
    5. Renal Impairment in 50% of cases

VII. Findings: General

  1. BCS Triad is commonly present (esp. Acute BCS)
    1. Abdominal Pain
    2. Ascites
    3. Hepatomegaly
  2. Other findings
    1. Jaundice
    2. Splenomegaly
    3. Leg Edema
    4. Venous Stasis Ulcers

VIII. Labs

  1. Liver Function Tests
    1. Serum transaminase increased (esp. acute BCS)
    2. Alkaline Phosphatase increased
    3. Serum Direct Bilirubin increased (acute BCS)
    4. Prothrombin (INR) increased (esp. acute and chronic BCS)
  2. Paracentesis
    1. Fluid Protein increased (>2 g) in chronic BCS
    2. White Blood Cell Count low (<500) in chronic BCS
    3. Serum Ascites-albumin gradient low (<1.1 g/dl) in chronic BCS
  3. Liver Biopsy
    1. Centrilobular hepatocyte necrosis
    2. Sinusoidal dilation

IX. Imaging

  1. Right Upper Quadrant Color-Flow Doppler Ultrasound
    1. First-line study in Budd-Chiari Syndrome
    2. High Test Sensitivity and Test Specificity for thrombosis (>85%) within IVC or hepatic veins
    3. Also identifies collateral venous drainage, Ascites and caudate lobe size
  2. Other imaging
    1. Consider CT Abdomen or MRI Abdomen

X. Differential Diagnosis

  1. See Cirrhosis
  2. Alcoholic Liver Disease
  3. Alpha-1 Antitrypsin Deficiency
  4. Biliary Atresia
  5. Congestive Heart Failure (including right-sided failure)
  6. Cystic Fibrosis
  7. Drug Induced Liver Injury
  8. Fitz-Hugh Curtis syndrome
  9. Hemochromatosis
  10. Inborn Errors of Metabolism
  11. Niemann-Pick Disease (type C)
  12. Viral Hepatitis

XI. Management

  1. Consult Hepatology
  2. Anticoagulation
    1. Key management in nearly all patients
    2. Warfarin is most commonly used (after initial low-molecular-weight Heparin)
  3. Relieving obstruction
    1. Thrombolysis with stenting
    2. Transjugular intrahepatic portosystemic shunt (TIPS)
    3. Surgical decompression in refractory acute cases
  4. Liver Transplantation
    1. Indicated in decompensated Cirrhosis
    2. Ten year survival >=70% after transplant

XII. Prognosis: Better Prognostic Factors

  1. Younger age
  2. Low Child-Pugh Score
  3. No Ascites
  4. Normal Serum Creatinine

XIII. Complications

  1. See Cirrhosis
  2. Esophageal Varices
  3. Hepatic Encephalopathy
  4. Hepatocellular Carcinoma
  5. Hepatorenal Syndrome
  6. Spontaneous Bacterial Peritonitis

XIV. References

  1. Hitawala (2023) Budd-Chiari Syndrome, StatPearls, Treasure Island, FL
    1. https://www.ncbi.nlm.nih.gov/books/NBK558941/
  2. Ferral (2012) AJR Am J Roentgenol 199(4): 737-45 +PMID: 22997363 [PubMed]
  3. Martens (2015) United European Gastroenterol J 3(6): 489-500 +PMID: 26668741 [PubMed]

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