Le syndrome de Budd-Chiari
Mots-clés :
Syndrome Budd-Chiari, Ascite, Thromboses, Shunt Transjugulaire Intrahépatique PortosystémiqueRésumé
Prérequis : Le syndrome de Budd-Chiari est une affection rare,souvent fatale en l’absence de traitement optimal. Il est caractérisé par une obstruction des voies de drainage veineux hépatique.But : Préciser la physiopathologie, les étiologies, les moyens diagnostiques et les modalités thérapeutiques de ce syndrome.
méthodes : Revue de la littérature
résultats : Le syndrome de Budd-Chiari est une entité complexe avec un large spectre d’étiologies et de présentations. Les anomalies hématologiques, en particulier le syndrome myéloprolifératif, sont la cause la plus fréquente du syndrome de Budd-Chiari. La présentation clinique dépend de l’étendue et la rapidité de l’obstruction veineuse hépatique. L’échodoppler, la tomodensitométrie et l’imagerie par résonnance magnétique des veines hépatiques et de la veine cave inférieure sont des moyens non invasifs permettant le diagnostic de la maladie. Les anticoagulants sont utilisés en première ligne avec les diurétiques et le traitement prophylactique de l’hypertension portale.Pour les sténoses courtes, une angioplastie peut être proposée. En cas d’échec, le recours au TIPS est indiqué et en dernier lieu, une transplantation hépatique. Cette stratégie thérapeutique a permis une survie à 5 ans de 70% environ. Le pronostic à moyen terme dépend de la sévérité de la maladie hépatique.
Conclusion : Le diagnostic du syndrome de Budd-Chiari doit être considéré chez tout patient ayant une maladie aigue ou chronique du foie. La prise en charge thérapeutique devrait suivre une stratégie par étapes.
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