BCS

1. Budd-Chiari syndrome
Dr Sunil Thomas George

2. Introduction
 Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction, independent
of the level or mechanism of obstruction, provided the obstruction is not due to cardiac disease,
pericardial disease, or sinusoidal obstruction syndrome (veno-occlusive disease)

3. Primary Budd-Chiari syndrome
 Obstruction due to a predominantly venous process (thrombosis or phlebitis)

4. Secondary Budd-Chiari syndrome
 Compression or invasion of the hepatic veins and/or the inferior vena cava by a lesion that
originates outside of the vein (eg, a malignancy).

5. Epidemiology
 More common in women
 In the non-Asian countries, pure hepatic vein blockage is more common, whereas in Asia, pure
inferior vena cava or combined inferior vena cava and hepatic vein blockage predominate.

6. Categorization - by disease duration and severity
 Acute (fulminant) liver failure
 Acute
 Subacute
 Chronic
Patients with acute liver failure or acute (non-fulminant) liver disease have not yet developed venous
collaterals, whereas venous collaterals are seen in patients with subacute and chronic liver disease.

7. Clinical manifestations
 Fever,
 Abdominal pain,
 Abdominal distension (from ascites),
 Lower extremity edema,
 jaundice,
 Gastrointestinal bleeding (from varices or portal hypertensive gastropathy),
 and/or hepatic encephalopathy

8. Diagnosis
 A diagnosis of Budd-Chiari syndrome can usually be established with Doppler ultrasonography.
(USG,MRI,CT,Venography to supplement the diagnosis)
 Evaluation for conditions that may have predisposed to the development of Budd-Chiari
syndrome such as prothrombotic disorders.
A newer approach, three-dimensional contrast-enhanced magnetic resonance
venography, is proving to be helpful for diagnosing pathology of the vena cava,
including Budd-Chiari syndrome
The gold standard for diagnosing Budd-Chiari syndrome is venography if non invasive testing are negative
under a strong suspicion

9. When to consider Budd-Chiari syndrome ?
 Among patients with acute liver failure, the presence of hepatomegaly, right upper quadrant
pain, and ascites should increase the suspicion for Budd-Chiari syndrome.

10. Management of Budd-Chiari
syndrome

11. Budd-Chiari syndrome
diagnosed
Initiate anticoagulation and
evaluate predisposing
conditons
Does patient have acute
liver failure ?
Refer to a liver
transplantation center for
evaluation & managment
Does the patient have
cirrhosis?
Treat complications of
PHT,Screen HCC,Consider
Liver transplantation
Acute? Well-defined clot
present?
YES NO
YES NO

12. Acute? Well-defined clot
present?
Are there contraindications
to thrombolytic therapy?
Is patient symptomatic from the obstruction?
Give thrombolytic therapy
Continue anticoagulation
and monitor for disease
progression
NO
YES
NO
YES
SUCCESSFUL UNSUCCESSFUL

13. Is patient symptomatic from the obstruction?
Is the obstruction amenable
to angiographic treatment?
Perform
angiography/stenting
Continue
anticoagulati
on and
monitor for
disease
progression
Continue
anticoagulati
on and
monitor for
disease
progression
Is TIPS placement technically feasible?
YES?= TIPS placment No?= Place surgical shunt
NOYES
YES NO
SUCCESSFUL
UNSUCCESSFUL

14. Does patient improve?
Continue
anticoagulati
on and
monitor for
disease
progression
Continue
anticoagulation
and refer for liver
transplantation
YES NO

15. Thank you