Budd-Chiari syndrome is a condition where there is obstruction of the hepatic veins or inferior vena cava, preventing normal blood flow out of the liver. It can be caused by multiple underlying disorders or risk factors for thrombosis in over 80% of patients. Common clinical manifestations include ascites in 84% of patients, hepatomegaly in 76%, and abdominal pain. Diagnosis involves Doppler ultrasound, CT, MRI, or venography. Treatment depends on whether the disease is acute, subacute, or chronic, and may involve medical management with anticoagulation or thrombolytic therapy, or surgical options like angioplasty, TIPS, shunts, or liver transplantation.

1. Budd-Chiari syndrome

2. INTRODUCTION
Pathophysiologic process that results in an
interruption or diminution of the normal flow of
blood out of the liver, However, as commonly
used, the Budd-Chiari syndrome implies
thrombosis of the hepatic veins and/or the
intrahepatic or suprahepatic inferior vena cava.

3. ETIOLOGY
An underlying disorder can be identified in over
80 % of patients with the Budd-Chiari
syndrome.
More than one thrombotic risk factors are
present in many patients; 46 % had more than
one risk factor in one series

5. A 2009 guideline from the American Association for the
Study of Liver Diseases recommends the following
approach for investigating causes of Budd-Chiari
Syndrome:
• Evaluate for space occupying lesions or malignant
tumors compressing or invading the hepatic venous
outflow tract with sonography, CT scan, or MRI.
• Seek evidence for ulcerative colitis, celiac disease, and
systemic diseases.
• Routinely evaluate for multiple, concurrent risk factors
for thrombosis.

6. CLINICAL MANIFESTATIONS
One of the largest published series included a total of 237 patients who had been
treated at four centers (in the United States, the Netherlands, and France)
between 1984 and 2001. The following observations were made:
• The median age was 35 (range 13 to 76)
• 67 % were female
• An overt myeloproliferative disorder was present in 23 % (the
majority of whom had polycythemia vera)
• The two most common symptoms were ascites (84 %) and
hepatomegaly (76 %); 11 patients (5 %) were asymptomatic
• The hepatic outflow obstruction was in the hepatic veins (62 %)
inferior vena cava (7 %) or both (31 %); 34 patients (14 %) had
associated portal vein thrombosis

7. Acute disease
• Commonly in women.
• Patients usually present with severe right upper
quadrant pain
• Hepatomegaly. Jaundice and ascites often develop
rapidly.
• Ascites is detectable by ultrasound in more than 90
percent of patients. Variceal bleeding may also
occur
• Serum aminotransferase concentrations can range
from 100 to 200 int. unit/L to more than 600
int. unit/L

8. Subacute and chronic disease
• Present for several weeks to more than six months prior to clinical
presentation
• Hypertrophy of the caudate lobe of the liver
• Cirrhosis may have developed in the chronically congested liver.
• Patients may then develop ascites, which may be massive.
• Hepatomegaly and abdominal pain are also common.
• encephalopathy is infrequent.
• Hepatopulmonary syndrome has been described in up to 28 % of
patients.
• normal or mild to moderate elevation of serum aminotransferases,
alkaline phosphat.
• Jaundice is rare.

9. Diagnosis
• Doppler ultrasonography
• CT scan
• Magnetic resonance imaging
• Venography
• Arteriography
• Liver biopsy

11. liver biopsy
• In 2009 AASLD recommended liver biopsy
ONLY when hepatic venous outflow
obstruction cannot be demonstrated by non
invasive imaging
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12. Management

13. Medical therapy
• Supportive: ascites
• Anticoagulation: AASLD recommend
anticoagulation only in patients with chronic
and subacute disease with well compensated
liver.
• Thrombolytic therapy: NOT in chronic Bud
Chiari and ONLY in patients in whom the clot
is well defined on venography

14. Surgical options
• Angioplasty
• TIPS
• Surgical shunts
• Liver Transplantation
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