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 arteriovenousmalformation stage II + III or IV (according to Schöbinger's classification) : activeor quiescent, marked or not by hemorrhagic phenomena.
• Patients (parents for minors) must sign a consent form established after clearinformation risks and expected benefits of the study.
• Patients (major and minor of childbearing age) must have effective contraceptionduring 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 investigationalproduct
• 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