Overview
Budd-Chiari syndrome (BCS) describes the blockage of the hepatic veins that drain from the liver into the inferior vena cava. It is commonly associated with underlying conditions leading to hypercoagulability.
Epidemiology
- BCS is very rare, with an incidence rate of 0.04 per 100,000 per year
Risk Factors
- Myeloproliferative disorders, such as polycythaemia vera and essential thrombocytosis
- Thrombophilias: Factor V Leiden, protein C and S deficiency, antithrombin III deficiency
- Combined oral contraceptive pill use
- Pregnancy and the postpartum period
Presentation
BCS has a classic triad of:
- Sudden-onset and severe abdominal pain, typically right upper quadrant pain
- Ascites
- Hepatomegaly that is usually tender
Differential Diagnoses
Spontaneous bacterial peritonitis (SBP)
- Although abdominal pain and ascites are common to both, hepatosplenomegaly is not commonly seen in SBP, and BCS does not have signs of infection, such as fever
Investigations
- FBC:
- May be abnormal if a myeloproliferative disorder is present
- LFTs:
- May show generalised derangement
- Thrombophilia screen:
- To identify underlying causes of hypercoagulable states
- Doppler ultrasound:
- High sensitivity and specificity
Management
Overview
- Management involves treating the underlying condition, such as using anticoagulation.
Complications
- Liver failure
- Hepatic encephalopathy
- Portal hypertension
- Spontaneous bacterial peritonitis
- Hepatorenal syndrome
Prognosis
- Untreated BCS has a very high mortality rate
- The prognosis depends on the underlying cause
