title: A cephalometric method to diagnosis the craniovertebral junction abnormalities in osteogenesis imperfecta patients.
abstract: Osteogenesis imperfecta (OI) is a hereditary bone fragility disorder that in most patients is caused by mutations affecting collagen type I. Their typical oral and craneofacial characteristics (Dentinogenesis imperfecta type I and class III malocclusion), involve the dentist in the multidisciplinary team that treat these patients. It is usual to perform lateral skull radiographs for the orthodontic diagnosis. In addition, this radiograph is useful to analyse the junctional area between skull base and spine, that could be damaged in OI. Pathology in the craneovertebral junction (CVJ) is a serious complication of OI with a prevalence ranging from rare to 37%. To diagnosis early skull base anomalies in these patients, previously the neurological symptoms have been appear, we make a simple cephalometric analysis of the CVJ. This method has four measurements and one angle. Once we calculate the values of the OI patient, we compare the result with the mean and the standard deviations of an age-appropriate average in healthy controls. If the patient has a result more than 2,5 SDs above the age-appropriate average in healthy controls, we should to refer the patient to his/her pediatrician or neurologist. These doctors have to consider acquiring another diagnostic images to be used to determine cranial base measurements with more reliability. Thereby, dentists who treat these patients, must be aware of the normal radiological anatomy of the cervical spine on the lateral cephalogram. Key words:Osteogenesis imperfecta, craniovertebral junction, cephalometric.
authors: Profesora asociada de Odontopediatría (Universidad Alfonso X El Sabio). Investigadora Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Mercedes
authors: M
authors: Ríos-Rodenas
authors: Profesor Titular de Odontopediatría (Universidad Complutense de Madrid (UCM)). Responsable Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Joaquín
authors: J
authors: de Nova
authors: Especialista en Endocrinología Pediátrica. Unidad de Osteogénesis Imperfecta (Hospital Universitario de Getafe (Madrid)). Investigadora Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: María-Pilar
authors: MP
authors: Gutiérrez-Díez
authors: Investigador Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Gonzalo
authors: G
authors: Feijóo
authors: Profesora Contratada Doctora de la UCM. Investigadora Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Maria-Rosa
authors: MR
authors: Mourelle
authors: Investigador Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Mario
authors: M
authors: Garcilazo
authors: Profesor asociado (UCM). Investigador Proyecto. Proyecto (FMM2013AP123942013) Fundación Mutua Madrileña (X Convocatoria).
authors: Ricardo
authors: R
authors: Ortega-Aranegui