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Orthodontic treatment in a patient with cleidocranial dysostosis. 2009

Giampietro Farronato, and Cinzia Maspero, and Davide Farronato, and Silvia Gioventù
Department of Orthodontics, University of Milan, Milan, Italy.

Cleidocranial dysostosis is a rare congenital skeletal disorder, associated with clavicular hypoplasia or aplasia, delayed closure of cranial fontanels, brachycephalic skull, delayed exfoliation of primary dentition, eruption of permanent teeth, and multiple supernumerary and morphologic abnormalities of the maxilla and mandible. The disorder is caused by mutation in the CBFA1 gene, on the short arm of chromosome 6p21. The prevalence of cleidocranial dysostosis is estimated one per million, without sex or ethnic group predilection. The purpose of this paper is to describe the orthodontic treatment in a patient with cleidocranial dysostosis. Therapy may include removal of supernumerary teeth, surgical exposure of impacted teeth, and orthodontic treatment.

UI MeSH Term Description Entries
D008297 Male Males
D008313 Malocclusion, Angle Class III Malocclusion in which the mandible is anterior to the maxilla as reflected by the first relationship of the first permanent molar (mesioclusion). Angle Class III,Habsburg Jaw,Hapsburg Jaw,Prognathism, Mandibular,Underbite
D002973 Cleidocranial Dysplasia Autosomal dominant syndrome in which there is delayed closing of the CRANIAL FONTANELLES; complete or partial absence of the collarbones (CLAVICLES); wide PUBIC SYMPHYSIS; short middle phalanges of the fifth fingers; and dental and vertebral anomalies. Cleidocranial Dysostosis,Dysostosis, Cleidocranial,Cleidocranial Digital Dysostosis,Marie-Sainton Syndrome,Scheuthauer-Marie-Sainton Syndrome,Cleidocranial Digital Dysostoses,Cleidocranial Dysostoses,Cleidocranial Dysplasias,Dysostoses, Cleidocranial,Dysostoses, Cleidocranial Digital,Dysostosis, Cleidocranial Digital,Dysplasia, Cleidocranial,Dysplasias, Cleidocranial,Marie Sainton Syndrome,Scheuthauer Marie Sainton Syndrome,Syndrome, Marie-Sainton,Syndrome, Scheuthauer-Marie-Sainton
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014081 Tooth Extraction The surgical removal of a tooth. (Dorland, 28th ed) Extraction, Tooth,Extractions, Tooth,Tooth Extractions
D014094 Tooth, Deciduous The teeth of the first dentition, which are shed and replaced by the permanent teeth. Primary Tooth,Tooth, Primary,Baby Teeth,Baby Tooth,Dentition, Deciduous,Dentition, Primary,Milk Teeth,Milk Tooth,Primary Teeth,Teeth, Deciduous,Teeth, Primary,Deciduous Dentition,Deciduous Dentitions,Deciduous Teeth,Deciduous Tooth,Dentitions, Deciduous,Dentitions, Primary,Primary Dentition,Primary Dentitions,Teeth, Baby,Teeth, Milk,Tooth, Baby,Tooth, Milk
D014095 Tooth, Impacted A tooth that is prevented from erupting by a physical barrier, usually other teeth. Impaction may also result from orientation of the tooth in an other than vertical position in the periodontal structures. Teeth, Impacted,Impacted Teeth,Impacted Tooth
D014096 Tooth, Supernumerary An extra tooth, erupted or unerupted, resembling or unlike the other teeth in the group to which it belongs. Its presence may cause malposition of adjacent teeth or prevent their eruption. Molar, Fourth,Teeth, Supernumerary,Fourth Molar,Fourth Molars,Molars, Fourth,Supernumerary Teeth,Supernumerary Tooth
D014716 Vertical Dimension The length of the face determined by the distance of separation of jaws. Occlusal vertical dimension (OVD or VDO) or contact vertical dimension is the lower face height with the teeth in centric occlusion. Rest vertical dimension (VDR) is the lower face height measured from a chin point to a point just below the nose, with the mandible in rest position. (From Jablonski, Dictionary of Dentistry, 1992, p250) Mandibular Rest Position,Rest Vertical Dimension,Vertical Dimension at Rest,Vertical Dimension of Occlusion,Dimension, Rest Vertical,Dimension, Vertical,Dimensions, Rest Vertical,Dimensions, Vertical,Mandibular Rest Positions,Occlusion Vertical Dimension,Occlusion Vertical Dimensions,Position, Mandibular Rest,Positions, Mandibular Rest,Rest Position, Mandibular,Rest Positions, Mandibular,Rest Vertical Dimensions,Vertical Dimension, Rest,Vertical Dimensions,Vertical Dimensions, Rest

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