[Primitive Budd-Chiari syndrome: a diagnostic and therapeutic challenge].

Primary Budd-Chiari syndrome is an hepatopathy characterized by obstruction of the hepatic venous flow in the vascular space between the hepatic venules and the junction between the inferior vena cava and the right atrium, thus excluding the causes of upstream (sinusoidal obstruction syndrome) and downstream (cardiac causes). This endoluminal venous obstruction is mainly due to thrombosis or its fibrotic consequences. This is a rare disease, mainly affecting young adults. Clinical manifestations are extremely variable; it can be asymptomatic, acute, subacute or chronic. The diagnosis is mainly based on Doppler ultrasound and/or magnetic resonance imaging. Several causes have been identified, in particular, myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, inherited thrombotic disorders. Symptomatic and etiologic treatments are the commonly used approaches, treatment recommendations are organized in algorithm. Recent therapeutic advances can significantly improve the prognosis.