title: [Management of craniofacial type 1 neurofibromatosis].
abstract: Type I neurofibromatosis (NF) is the most common autosomal dominant disease. It concerns one in 3000 births, the penetrance is close to 100% and 50% of new cases are de novo mutations (17q11.2 chromosome 17 location). Cranio-maxillofacial region is concerned in 10% of the cases, in different forms: molluscum neurofibroma, plexiform neurofibroma, cranio-orbital neurofibroma, parotido-jugal neurofibroma, cervical neurofibroma. These lesions have different prognosis depending on the craniofacial localization: ocular functional risk, upper airway compressive risk, nerve compression risk, aesthetic and social impact. The maxillofacial surgeon in charge of patients with type I NF should follow the patient from the diagnosis and organize the different surgical times in order to take care about the different issues: vital, functional and aesthetic. We describe the treatment of facial localizations of type 1 NF as it is done at the University Hospital of Lyon and at the Rhône-Alpes-Auvergne neurofibromatosis reference center.
authors.affiliation: Service de chirurgie maxillofaciale, centre hospitalier Lyon-Sud, chemin du Grand-Revoyet, 69495 Pierre-Bénite, France. Electronic address: jt125_2@hotmail.com.
authors.forename: J T
authors.initials: JT
authors.lastname: Bachelet
authors.affiliation: Centre de compétence neurofibromatoses Rhône-Alpes-Auvergne, CLCC Léon-Bérard, 69008 Lyon, France.
authors.forename: P
authors.initials: P
authors.lastname: Combemale
authors.affiliation: Unité Inserm 1052, centre de recherche en cancérologie Lyon-Est, centre Léon-Bérard, bâtiment Cheney-D, 69008 Lyon, France.
authors.forename: C
authors.initials: C
authors.lastname: Devic
authors.affiliation: Unité Inserm 1052, centre de recherche en cancérologie Lyon-Est, centre Léon-Bérard, bâtiment Cheney-D, 69008 Lyon, France.
authors.forename: N
authors.initials: N
authors.lastname: Foray
authors.affiliation: Multidisciplinary Skull Base Unit, Department of Neurosurgery B, Neurological Hospital Pierre-Wertheimer, hospices civils de Lyon, 59, boulevard Pinel, 69677 Lyon cedex, France.
authors.forename: E
authors.initials: E
authors.lastname: Jouanneau
authors.affiliation: Service de chirurgie maxillofaciale, centre hospitalier Lyon-Sud, chemin du Grand-Revoyet, 69495 Pierre-Bénite, France.
authors.forename: P
authors.initials: P
authors.lastname: Breton
mesh_terms: D005153:Facial Neoplasms / Q000175:diagnosis / Q000628:therapy; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D018318:Neurofibroma, Plexiform / Q000175:diagnosis / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000628:therapy*; D009918:Orbital Neoplasms / Q000473:pathology / Q000628:therapy; D019651:Plastic Surgery Procedures / Q000379:methods; D012888:Skull Neoplasms / Q000175:diagnosis / Q000628:therapy
keywords: Chirurgie reconstructrice; Neurofibromas; Neurofibromatose de type 1; Neurofibromatosis 1; Neurofibromes; Neurofibromes plexiformes; Plexiform neurofibromas; Reconstructive surgical procedures