Budd-Chiari Syndrome (BCS)

 

  • – Obstruction of the hepatic veins or terminal inferior vena cava
  • Primary BCS
  •    – Arise from venous anomaly
  • Secondary BCS
  •     – Arise from an initial lesion outside the veins
  • Mechanism of secondary BCS:
  • – Invasion by A malignant tumor/alveolar echinococcosis
  • – Compression by cysts or FNH, usually without clinically significant liver disease
  • – Compression/inflammation due to polycystic liver disease/liver abscess
  • – Blunt trauma to abdomen/chest

                   Figure: Hepatic Veins

  • Risk Factors for Budd-Chiari Syndrome
  • – Myeloproliferative neoplasms
  • – Antiphospholipid syndrome
  • – Paroxysmal nocturnal hemoglobinuria
  • – Factor V Leiden mutation
  • – Factor II (prothrombin) mutation G20210A
  • – Protein C deficiency
  • – Protein S deficiency
  • – Antithrombin deficiency
  • – Hyperhomocysteinemia
  • – Recent pregnancy
  • – Recent oral contraceptive use
  • – Systemic disease
  • Pathogenesis of Budd-Chiari Syndrome
  • – 2 major hepatic veins needs to be obstructed before clinical manifestation
  • – Pattern and speed of occlusive process varies among major veins and from patients to patients
  • – A collateral circulation develop
  • – When IVC is obstructed, collaterals form from lumbar and azygos veins
  • – Restoration hepatic venous drainage through large collaterals may alleviate all symptoms and signs and carries a good sign
  • – Increased hepatic venous and sinusoidal pressures translates into sinusoidal dilatation and congestion, predominantly in centrilobular area and causes ascites formation
  • – Due to stasis and underlying prothrombotic state both intra and extrahepatic portal vein thrombosis is common
  • – Due to decreased hepatic blood flow   ⇒     Ischemic coagulative necrosis, apoptosis, predominantly in central parts of the lobule leading to liver dysfunction or liver failure
  •            Clinical Features:
  • – Asymptomatic
  • – Fulminant hepatic failure
  • – Chronic liver disease
  • Major features
  •         • Ascites
  •         • Abdominal pain
  •         • Fever
  • All complications of CLD may occur e.g. GI bleeding, bacterial infection, hepatorenal syndrome, encephalopathy

Investigations

 

  • – Diagnosis is based on direct/indirect evidence of hepatic vein/IVC obstruction
  • CBC:
  •        -Blood cell counts influenced by underlying cause
  •        – Normal blood counts with severe portal hypertension suggests myeloproliferative disorder as hypersplenism associated with portal HTN is expected to reduce cell count
  • LFT:
  •    – Bilirubin, ALT, AST, Albumin, PT    Variably altered
  •    – ALP moderately increased
  • USG/ Doppler USG:
  •          – Hepatic vein outflow tract obstruction (non visualization of hepatic veins)
  •          – Ascites
  •          – Splenomegaly
  •         – Transformation of hepatic vein into a chord-like remnant
  •         – Liver sectoral atrophy/hypertrophy, segment I(caudate lobe) enlargement
  • Dynamic CT:
  •      – Arterial + Portal phase: Patchy enhancement
  •     – Late phase: Disappearance of the enhancement
  • MRI:
  •      – Regenerative nodule
  •     – Nodules result from perfusion disturbance
  •     – Nodules are small (<4cm), multiple (frequently >10 lesion), hypervascularized, disseminated throughout the liver
  • Liver biopsy
  • Not required for diagnosis
  • Exception:  Variant of BCS in which large hepatic veins and IVC are patent and small veins are thrombosed
  • Differentiating benign regenerative macronodules from hepatocellular carcinoma (when nodules are ≤ 3 in number, size ≥ 3cm, heterogeneity or washout on venous phase, changes in 2 consecutive imaging, raised AFP)
  • Investigations for underlying Cause of BCS
  •          – General examination for systemic disease
    • – Imaging for secondary BCS
    • – CBC
    • – Assessment for JAK2 V617 mutation
    • – Flow cytometry of blood cells for PNH
    • – Factor V Leiden and prothrombin G20210A mutation
    • – For antiphospholipid syndrome : Lupus anticoagulant, β₂ glycoprotein I antibody
    • – Antithrombin
    • – Protein C, S level if prothrombin level is normal
    • – Bone Marrow biopsy : when Jak2,V617 is negative

    Natural History of BCS:

    • – Not well known
    • – Report from early studies: 90% patients die of liver disease within 3 year of diagnosis
    • – Patients with IVC obstruction        High risk for HCC development
    • – Asymptomatic patients: excellent medium & long term outcome

Treatment of BCS

 

                                            Figure. Recommended stepwise therapeutic algorithm of Budd-Chiari syndrome

 

  • – Primary BCS: Anticoagulation and specific therapy for the underlying disease for all patients
  • – Interrupt anticoagulation when an invasive procedure is performed,including paracentesis
  • Guideline for anticoagulation for venous thromboembolism should be followed
  •           ♦  Anticoagulant for indefinite period to reduce the risk of clot extension and new thrombotic episode
  •           Low molecular weight heparin (LMWH) for at least 5-7 days and oral anticoagulant with VKA (Target INR 2-3)
  •           LMWH can be stopped when INR is within target for 2 consecutive measurements
  • Treatment for portal hypertension : Medical and endoscopic as appropriate
  • – Short stenosis of major hepatic vein/IVC : Percutaneous angioplasty
  • – Symptomatic patients with venous lesion amenable to percutaneous angioplasty       Investigate and treat accordingly
  • -TIPS: when symptoms/signs are not well controlled, angioplasty not feasible
  • Surgical portacaval shunt       When TIPS not feasible, No improvements by TIPS
  • – Liver transplantation: when derivative technique (TIPS, surgery) have failed
  • – Screen BCS patients for HCC

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