Budd Chiari Syndrome

DEFINITION

Hepatic venous outflow occlusion, as a result of a range of possible hypercoagulable states or anatomic abnormalities.

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EPIDEMIOLOGY

Older age - higher risk

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AETIOLOGY

Hypercoagulable states
Myoproliferative disorders
- e.g. polycythemia vera, essential thrombocytosis
Paroxysmal nocturnal haemoglobinuria
Factor V Leiden, antiphospholipid antibody
Deficiencies in proteins C and S

Anatomical problems
Vascular webbing and strictures



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BIOLOGICAL BEHAVIOUR

Pathophysiology
Outflow obstruction
Pathology shows sinusoidal congestion, inflammation.
- progressive hepatocyte atrophy and impaired cell regeneration if ongoing congestion.
Liver parenchyma may demonstrate characteristic regenerative nodules
- these may represent hyperplasia or adenoma
If chronic, progresses to cirrhosis and severe portal hypertension

DEABMIM  

MANIFESTATIONS

Classic triad
of:
- hepatomegaly
- RUQ pain
- ascites

Can be acute or chronic
- symptom onset directly correlates with rapidity of venous outflow obstruction
- up to 25% are asymptomatic (chronic)

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INVESTIGATIONS

Doppler USS

70% sensitive, procedure of choice.

CT or MRI
Characterize outflow and can assess parenchyma and degree of ascites
- and for caudate lobe hypertrophy (see implications below)

Hepatic Venography
Gold standard though less commonly used due to invasiveness.
Can measure caval pressures and biopsy at same time.

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MANAGEMENT

1. Principle is Multimodal Treatment
Mortality rate for those untreated is extremely high
Aggressive, multidisciplinary.
- previously, surgical shunting was central; now radiologic thrombolysis, angioplasty and stenting is central
Aim is to relieve obstruction, symptoms and prevent recurrence

2. Aggressive Workup for Hypercoagulable States

3. Anticoagulation
- as per cause

4. Sodium Intake and Diuresis
- as per portal hypertension

5. Invasive Procedures
- thrombolysis, TIPS, surgical shunts

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Selection of Therapy

Thrombolytic therapy
Poorly studied, generally limited to incomplete occlusions
Combination of balloon, stent and TIPS may be effective adjuncts.

Shunting
TIPS can alleviate outflow obstruction, with close follow up.
Best if performed early, high-volume centres, often as a bridge to surgery

OLT (orthotopic liver transplant)
Transplantation may be most viable long-term option.
- but organs are not freely available
--> interventional radiology and surgical shunting provide short-term alternatives.
Outcomes have been positively influenced by aggressive medical and interventional therapy including anticoagulation.
- 10 year survival ~70%
Technically challenging due to swollen liver, hyperplasia of caudate lobe makes dissection of IVC difficult, stents can cause problems and migration.
- portal vein thrombosis is a difficult problem and requires a plan (see algorithm).

Recurrent Disease
In up to 10%, often years later.
Lifelong anticoagulation to prevent.
Occasionally retransplantation may even be required.

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REFERENCES
Cameron 10th