title: [Cherubism: diagnosis and treatment in the pediatric age].
abstract: Cherubism is a benign bone dysplasia of childhood, exclusively involving maxillary bones and spontaneous resolving after puberty in different grades. Approximately, 280 cases have been reviewed in the literature. It is an autosomal dominant disorder in which the normal bone is replaced by cellular fibrous and immature bone, resulting in painless symmetrical enlargement of the jaws. Diagnosis is based in clinical and radiological findings, confirmed by histology. Treatment is a controversial issue, and it is recommended surgical management as conservative as possible during the rapid growth phases. An aggressive case of cherubism is reported, diagnosed and followed since early childhood until puberty, with progressive involvement of facial bones developing in a disruption of facial contours and occlusion. The patient is treated by several surgical interventions oriented to minimize the aesthetic impact of the disease being as conservative as possible. The highlights of this case are the great proportion of the lesions, the functional and emotional disturbances brought out by these lesions and the difficulty to choose the most appropriate age and form of treatment.
authors.affiliation: Servicio de Cirugía Oral y Maxilofacial, Hospital Universitario La Paz, Madrid. ro_sb@hotmail.com
authors.forename: R
authors.initials: R
authors.lastname: Sánchez Burgos
authors.forename: M
authors.initials: M
authors.lastname: Martín Pérez
authors.forename: M
authors.initials: M
authors.lastname: Ramírez Piqueras
authors.forename: E
authors.initials: E
authors.lastname: Gómez García
authors.forename: M
authors.initials: M
authors.lastname: Burgueño García
mesh_terms: D002636:Cherubism / Q000175:diagnosis* / Q000601:surgery*; D002675:Child, Preschool; D005260:Female; D006801:Humans