title: Oral manifestations of neurofibromatosis type 1 in children with facial plexiform neurofibroma: report of three cases.
abstract: Neurofibromatosis type 1 (NF1) is a common autosomal genetic disorder with a prevalence of 1 in 3,000 births. NF1 is a complex syndrome characterized by many abnormalities and may affect all organ systems. Oral manifestations of NF1 occur frequently, but reports including NF1 children with facial plexiform neurofibromas and oral alterations are scant. Facial plexiform neurofibroma may cause asymmetry, disfigurement and usually arises from the trigeminal nerve. The aim of this paper is to to report three pediatric NF1 cases with facial plexiform neurofibroma presenting with oral manifestations, which were evaluated clinically and radiographically, and also to briefly review the literature. Patients presented with changes in the oral soft tissues, jaws, and teeth ipsilateral to the tumor.
authors.affiliation: School of Medicine, Fluminense Federal University, Niterói, RJ, Brazil.
authors.forename: K S
authors.initials: KS
authors.lastname: Cunha
authors.affiliation: Department of Specific Formation, School of Dentistry, Nova Friburgo University Pole, Fluminense Federal University, Nova Friburgo, RJ, Brazil.
authors.forename: R E
authors.initials: RE
authors.lastname: Rozza-de-Menezes
authors.affiliation: School of Medicine, Fluminense Federal University, Niterói, RJ, Brazil.
authors.forename: R M
authors.initials: RM
authors.lastname: Andrade
authors.affiliation: School of Medicine, Fluminense Federal University, Niterói, RJ, Brazil.
authors.forename: Lms
authors.initials: L
authors.lastname: Almeida
authors.affiliation: Department of Oral Pathology and Oral Diagnosis, School of Dentistry, Federal University of Rio de Janeiro, RJ, Brazil.
authors.forename: Mer
authors.initials: M
authors.lastname: Janini
authors.affiliation: Department of Microbiology and Immunology, School of Medicine, Serra dos Órgãos University Center (UNIFESO), Teresópolis, RJ, Brazil.
authors.forename: M
authors.initials: M
authors.lastname: Geller
mesh_terms: D002648:Child; D063169:Dentofacial Deformities / Q000175:diagnosis*; D005153:Facial Neoplasms / Q000175:diagnosis*; D005260:Female; D005887:Gingival Neoplasms / Q000175:diagnosis; D019214:Gingival Overgrowth / Q000175:diagnosis; D006801:Humans; D008260:Macroglossia / Q000175:diagnosis; D008297:Male; D008310:Malocclusion / Q000175:diagnosis; D008334:Mandible / Q000002:abnormalities; D008335:Mandibular Condyle / Q000002:abnormalities; D018318:Neurofibroma, Plexiform / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D014062:Tongue Neoplasms / Q000175:diagnosis
keywords: Neurofibromatosis 1; child; oral manifestations; plexiform neurofibroma