[Budd-Chiari syndrome]

Budd-Chiari syndrome is a rare condition characterized by obstruction of hepatic venous drainage, ranging from hepatic venules to the terminal part of the inferior vena cava. A thorough etiological workup to search for a pro-thrombotic disorder should be systematically performed. The most common cause of Budd-Chiari syndrome is myeloproliferative syndrome, present in more than 40% of cases. The clinical presentation of BCS is highly variable, ranging from asymptomatic patients (3% of cases) to those presenting with fulminant hepatitis. Diagnosis relies on imaging, notably abdominal ultrasound coupled with Doppler.A progressive therapeutic approach, combining medical measures (curative anticoagulation, treatment of the underlying cause, management of portal hypertension complications) and a strategy to restore hepatic venous flow, is recommended, preferably in a specialized center for vascular liver diseases. This strategy has significantly improved patient prognosis, with an overall 5-year survival rate exceeding 80%. The follow-up frequency for BCS patients is biannual, with hepatic imaging recommended, as more than 60% of patients may develop hepatic nodules and are at risk of hepatocellular carcinoma.