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Budd chiari syndrome

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budd chiari syndrome in short

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Budd chiari syndrome

  1. 1. Budd-Chiari syndrome
  2. 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. 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
  4. 4. 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.
  5. 5. 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
  6. 6. 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
  7. 7. 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.
  8. 8. Diagnosis • Doppler ultrasonography • CT scan • Magnetic resonance imaging • Venography • Arteriography • Liver biopsy
  9. 9. liver biopsy • In 2009 AASLD recommended liver biopsy ONLY when hepatic venous outflow obstruction cannot be demonstrated by non invasive imaging 11
  10. 10. Management
  11. 11. 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
  12. 12. Surgical options • Angioplasty • TIPS • Surgical shunts • Liver Transplantation 14