Prospective Evaluation of the Efficacy of Sirolimus (Rapamune®) in the Treatment of Severe Arteriovenous Malformations

Inclusion Criteria: * Patients (adults, adolescents and children older than 2 years), with arteriovenous malformation stage II + III or IV (according to Schöbinger's classification) : active or quiescent, marked or not by hemorrhagic phenomena. * Patients (parents for minors) must sign a consent form established after clear information risks and expected benefits of the study. * Patients (major and minor of childbearing age) must have effective contraception during the study period and continuing until 12 weeks after the end of treatment * Negative pregnancy blood test for women of childbearing age. Exclusion Criteria: * Chronic or acquired immunosuppression : * patients with transplanted organ or who received a hematopoietic stem cell * patient with congenital immunodeficiency * Patients implanted with chronic active infection associated with hepatitis B , hepatitis C or HIV * Pregnant or nursing woman. * Allergy to macrolides * Allergy to peanut or soya * Hypersensitivity to " Sirolimus " or any of the excipients of the investigational product * Contraindications to performing an MRI * Leukopenia below 1 000 /mm3 * Thrombocytopenia lower to 80,000 /mm3 * Anemia with Hb \< 9 g/dl * Elevated transaminase \> 2.5 N * History of cancer less than two years before the inclusion * Surgery older than 2 months before inclusion * Active infection (viral and bacterial ) on the date of inclusion * Hypercholesterolemia \> 7 mmol / l despite appropriate medical treatment * Hyperlipidemia \> 2 mmol / l despite appropriate medical treatment * Uncontrolled diabetes * Patients unable to follow a clinical study * Major under guardianship, persons deprived of their liberty