title: The Budd-Chiari syndrome.
abstract: BACKGROUND
  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.
  
  
  METHODS
  Review of literature.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: Rania
authors.initials: R
authors.lastname: Hefaiedh
authors.forename: Mariem
authors.initials: M
authors.lastname: Cheikh
authors.forename: Lobna
authors.initials: L
authors.lastname: Marsaoui
authors.forename: Rym
authors.initials: R
authors.lastname: Ennaifer
authors.forename: Hayfa
authors.initials: H
authors.lastname: Romdhane
authors.forename: Houda
authors.initials: H
authors.lastname: Ben Nejma
authors.forename: Najet
authors.initials: N
authors.lastname: Bel Hadj
authors.forename: Nafaa
authors.initials: N
authors.lastname: Arfa
authors.forename: Mohamed Taher
authors.initials: MT
authors.lastname: Khalfallah
mesh_terms: D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006801:Humans; D011379:Prognosis