Budd-Chiari Syndrome

Budd -Chiari Syndrome (BCS) is a rare condition which occurs when there is obstruction of the hepatic veins.

Aetiology¹

• Haematological:
  • Polycythaemia vera and other myeloproliferative disorders
  • Thrombophilic conditions eg deficiencies of Protein C, Protein S, Antithrombin III or Factor V Leiden
  • Antiphospholipid antibody syndrome
  • Essential thrombocytosis
  • Paroxysmal nocturnal haemoglobinuria
  • Post bone marrow transplant
• Reduced blood flow: Webs in vena cava, right heart failure, constrictive pericarditis, right atrial myxoma
• Obstetric: The condition can occur during pregnancy and post-partum
• Drugs: Combined oral contraceptives, HRT, urethane
• Chronic infections: Hydatid disease, amoebic abscesses, aspergillosis, syphilis, tuberculosis
• Chronic Inflammatory Conditions: Inflammatory bowel disease, sarcoid, SLE, Sjogren's syndrome, Behcet's disease (3.2% in one study),² mixed connective tissue disease
• Malignancy: Hepatocellular carcinoma, renal cell carcinoma, Wilms tumour, adrenal carcinoma, leiomyosarcoma
• Trauma
• Others: Alpha 1-antitrypsin deficiency, idiopathic(30%)

The global prevalence of BCS is unknown. A study in Japan estimated a prevalence of 2.4/million with 20 new cases being diagnosed each year.

A Swedish review estimated a mean age-standardised incidence in 1990-2001 of 0.8 per million per year and a prevalence rate of 1.4 per million inhabitants.⁴

In the US, haematologic disorders account for 18% of the cases and a history of pregnancy or the postpartum conditions accounts for another 20%. Tumours account for 9%. Inferior vena caval webs are the main cause in most patients from eastern Asia, India and South Africa.

In Turkey, the commonest causes are hydatid disease and Behçet's disease.⁵

Risk factors

In the Swedish series, the main risk factors identified were myeloproliferative disorders (38%), thrombophilic factors (31%) and oral contraceptives (30%). About half of the patients had a multifactorial aetiology.⁴

Presentation can be sudden with abdominal pain and rapidly developing ascites, hepatomegaly, jaundice and renal failure (±fulminant hepatic failure ±hepatic coma). Most commonly it presents gradually with ascites (jaundice is commonly absent). Just under 50% of such patients will also have renal impairment.

• Cirrhosis
• Portal hypertension
• Portal vein thrombosis
• Hepatic veno-occlusive disease (common after bone marrow transplantation)
• Right-sided heart failure

• Liver function tests - these may show a mild elevation.
• Ascitic fluid -this usually has high protein content (except if the onset is very acute).
• Radio-imaging - CT scan or MRI may show a prominent caudate lobe (the bit to the left of the portal vein when facing the patient).
• Doppler ultrasound may help to exclude hepatic venous or inferior vena cava thrombosis. One study identified altered hepatic and/or caval veins and caudate lobe hypertrophy as being the findings most commonly associated with Budd-Chiari Syndrome.⁷
• Caval venography excludes caval webs and occluded hepatic veins.
• Liver biopsy often shows centrilobular congestion.

• Any underlying haematological condition should be treated (e.g. with anticoagulation).
• Ascites should be managed with diuretics plus fluid and salt restriction.
• Surgical decompression of liver is performed in cases of persistent congestion. e.g via transjugular intrahepatic portosystemic shunt (TIPS) and gives excellent results, even in high risk patients.⁹
• Balloon angioplasty ±/ stent is indicated for inferior vena caval web and sometimes in hepatic vein thrombosis if the affected length of vein is not extensive. The percutaneous route may be used and has proved safe and effective.^10,11
• Liver transplantation may be appropriate if there is decompensated liver cirrhosis.⁶ Reconstructing hepatic venous outflow post-transplant is sometimes a problem but successful venoplasty using autologous vein grafts has been reported.¹²

Hepatic failure ±encephalopathy, portal hypertension, oesophageal varices ±haemorrhage, hepatorenal syndrome (renal failure in patients with advanced chronic liver disease).

Factors associated with a good prognosis include younger age at diagnosis, absence or a small amount of ascites and a low serum creatinine level.

Identification of the cause of the symptom using modern radio-imaging techniques has had a major effect on prognosis.¹³ Interventional and medical treatment has helped to keep patients alive for up to eight years, after which liver transplantation is considered. One series reported a survival rate of 68% after ten years.¹⁴ Liver transplant is an effective treatment, providing it is done before renal failure sets in.¹⁵

Portal hypertension and portal vein thrombosis carry a poor prognosis.⁷