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Budd-Chiari syndrome
By Ahmed Abdulghany
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.
ETIOLOGY
• An underlying disorder can be identified in
over 80 % of patients with the Budd-Chiarisyndrome.
• 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 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.
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
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
% 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
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.
DIAGNOSIS
• Doppler ultrasonography
• CT scan
• Magnetic resonance imaging
• Venography
• Liver biopsy
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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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 BuddChiari and ONLY in patients in whom the clot is well defined on venography
Surgical options
• Angioplasty
• TIPS
• Surgical shunts
• Liver Transplantation
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Thank you