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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.
An underlying disorder can be identified in over
80 % of patients with the Budd-Chiari
More than one thrombotic risk factors are
present in many patients; 46 % had more than
one risk factor in one series
A 2009 guideline from the American Association for the
Study of Liver Diseases recommends the following
approach for investigating causes of Budd-Chiari
• 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
• Routinely evaluate for multiple, concurrent risk factors
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
• Commonly in women.
• Patients usually present with severe right upper
• Hepatomegaly. Jaundice and ascites often develop
• Ascites is detectable by ultrasound in more than 90
percent of patients. Variceal bleeding may also
• Serum aminotransferase concentrations can range
from 100 to 200 int. unit/L to more than 600
Subacute and chronic disease
• Present for several weeks to more than six months prior to clinical
• 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
• normal or mild to moderate elevation of serum aminotransferases,
• Jaundice is rare.
• Supportive: ascites
• Anticoagulation: AASLD recommend
anticoagulation only in patients with chronic
and subacute disease with well compensated
• Thrombolytic therapy: NOT in chronic Bud
Chiari and ONLY in patients in whom the clot
is well defined on venography