Efficacy of Rapamycin in the Treatment of Cervico-facial Lymphatic Malformations

Inclusion Criteria:

• Patient from 0 to 18 years of age, presenting with poly-cystic suprahyoid ormediastinal lymphatic.

• with chronic pain or functional respiratory or swallowing impairment with a CDSscore < 8

• Curative treatment is not possible or associated with a high risk of morbidity ,mortality and functional and cosmetic impairment

• Karnofsky Score (> 10 years of age) or Lansky score (≤10 years of age) > 50%

• Biology

• Neutrophils count≥1.0 x 109/L

• Platelets count ≥ 100 x 109/L

• Hemoglobin ≥ 8 g/dL

• Bilirubin ≤ 1,5 ULN

• Transaminases < 2,5 ULN

• Serum albumin ≥ 2 g/dL.

• LDL cholesterol <160 mg/dL

• Triglycerides < 150 mg/dL

• Negative test of pregnancy if relevant

• Social security affiliation

• At least 2 months after a previous procedure on the malformation

Exclusion Criteria:

• Non-respect of inclusion criteria

• Other immunosuppressive therapy or long-term general corticosteroid therapy withouta 28-day washout period

• renal failure

• Liver failure

• Digestive disease leading to rapamycin malabsorption

• uncontrolled or severe infectious disease

• Patients requiring treatment interfering with CYP3A4 isoenzyme (rifampicin,rifabutin, carbamazepine, phenobarbital, phenytoin) or inhibiting CYP3A4 isoenzyme'sactivity (ketoconazole, voriconazole, itraconazole, telithromycin, clarithromycin,Diltiazem, Verapamil, nicardipine, clotrimazole, fluconazole , troleandomycin,bromocriptine, cimetidine, danazol, protease inhibitors) -patients requiringtreatment by cisapride and metoclopramide

• Concomitant administration of mTOR inhibitor

• Peanuts or soya allergy

• Impossibility to receive informed consent

• Absence of social security affiliation

• refusal to sign consent

• Ongoing pregnancy or breastfeeding

• refusal to participate