The Budd-Chiari syndrome.

Abstract The Budd-Chiari syndrome is a rare disease, often fatal if not treated optimally. It is characterized by a blocked hepatic venous outflow tract. aim: This review attempted to present pathophysiology, aetiologies,diagnosis and therapeutic modalities of the Budd-Chiari syndrome. Review of literature. Budd-Chiari syndrome is a complex disease with a wide spectrum of aetiologies and presentations. Hematologic abnormalities, particularly myeloproliferative disorders, are the most common causes of the Budd-Chiari syndrome. The clinical presentation is governed by the extent and rapidity of the hepatic vein occlusion. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for shortlength venous stenosis; then Transjugular Intrahepatic Portosystemic Shunt (TIPS); and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 70%. Medium-term prognosis depends on the severity of liver disease. The diagnosis of the Budd-Chiari syndrome must be considered in any patients with acute or chronic liver disease. Management of this syndrome should follow a step by step strategy.