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 CDS score < 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 without a 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's activity (ketoconazole, voriconazole, itraconazole, telithromycin, clarithromycin, Diltiazem,Verapamil, nicardipine, clotrimazole, fluconazole , troleandomycin, bromocriptine,cimetidine, danazol, protease inhibitors) -patients requiring treatment by cisaprideand 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