The Budd-Chiari syndrome

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Published Jun 24, 2013
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Vol. 91 No. 6 (2013): Issue Jun 2013

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Rania Hefaiedh
Mariem Cheikh
Lobna Marsaoui
Rym Ennaifer
Service de Gastro-entérologie Hôpital H. Thameur Tunis
Hayfa Romdhane
Service de Gastro-entérologie A. Hôpital la Rabta. Tunis
Houda Ben Nejma
Najet Bel Hadj
Nafaa Arfa
Mohamed Taher Khalfallah

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.
Conclusion: 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.

Keywords:

Budd-Chiari syndrome, Ascites, Thrombosis, Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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