The aim of the study is to evaluate the efficacy and safety of sirolimus (oral form), to decrease the volume and symptoms due to superficial arteriovenous malformations (AVM).

Sirolimus has properties that reduce the activity of the immune system (immunosuppressant), to fight against the proliferation of cancer cells (anti- tumor) and also reduce the proliferation of blood vessels (anti -vascular). Sirolimus is primarily used in transplant patients to prevent organ transplant rejection. Many animal and laboratory studies were carried out and demonstrate in particular the activity of sirolimus on vessels. It is this anti- vascular effect that could help treat arteriovenous malformations.

Anti-proliferative and anti-angiogenic properties of Sirolimus (Rapamycin®) are the basis of the rationale to use it in the treatment of arteriovenous malformations, for which the pathophysiology remains poorly understood. The interest of this class of drug is that inhibition of mTOR (mammalian target of rapamycin) may also block growth and / or angiogenic factors (other than VEGF) involved in the development of AVM. More specifically anti-VEGF drugs does not have that potential.

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