Diagnosis and treatment of familial cherubism characterized by early onset and rapid development.
Identifieur interne : 001E65 ( PubMed/Curation ); précédent : 001E64; suivant : 001E66Diagnosis and treatment of familial cherubism characterized by early onset and rapid development.
Auteurs : Carmen Mortellaro [Italie] ; Lucilla Bello ; Alberta Greco Lucchina ; Angela PucciSource :
- The Journal of craniofacial surgery [ 1536-3732 ] ; 2009.
Descripteurs français
- KwdFr :
- Biopsie, Chérubinisme (), Chérubinisme (diagnostic), Chérubinisme (génétique), Curetage, Diagnostic différentiel, Enfant d'âge préscolaire, Extraction dentaire, Humains, Jeune adulte, Mâchoire partiellement édentée (étiologie), Mâle, Radiographie panoramique, Régénération osseuse (physiologie), Tomodensitométrie, Éruption dentaire (physiologie), Études de suivi.
- MESH :
- diagnostic : Chérubinisme.
- génétique : Chérubinisme.
- physiologie : Régénération osseuse, Éruption dentaire.
- étiologie : Mâchoire partiellement édentée.
- Biopsie, Chérubinisme, Curetage, Diagnostic différentiel, Enfant d'âge préscolaire, Extraction dentaire, Humains, Jeune adulte, Mâle, Radiographie panoramique, Tomodensitométrie, Études de suivi.
English descriptors
- KwdEn :
- Biopsy, Bone Regeneration (physiology), Cherubism (diagnosis), Cherubism (genetics), Cherubism (surgery), Child, Preschool, Curettage, Diagnosis, Differential, Follow-Up Studies, Humans, Jaw, Edentulous, Partially (etiology), Male, Radiography, Panoramic, Tomography, X-Ray Computed, Tooth Eruption (physiology), Tooth Extraction, Young Adult.
- MESH :
- diagnosis : Cherubism.
- etiology : Jaw, Edentulous, Partially.
- genetics : Cherubism.
- physiology : Bone Regeneration, Tooth Eruption.
- surgery : Cherubism.
- Biopsy, Child, Preschool, Curettage, Diagnosis, Differential, Follow-Up Studies, Humans, Male, Radiography, Panoramic, Tomography, X-Ray Computed, Tooth Extraction, Young Adult.
Abstract
Cherubism is a benign maxillary bone dysplasia of childhood, usually showing an autosomically dominant inheritance with variable penetrance and spontaneously resolving after puberty. Only maxillary bones are affected and develop pseudocystic osteolytic lesions. This article presents an early and rapidly evolving familial case of cherubism. The 3-year-old child underwent conservative curettage of lesions, with a conservative approach that allowed a normal permanent dentition in adolescence. Family history revealed that the father had been treated for similar lesions between 14 and 21 years of age, but the late treatment caused edentulism. In conclusion, although cherubism represents a benign and localized maxillary dysplasia, it requires prompt surgical but conservative treatment and careful follow-up to avoid permanent lesions, that is, malocclusion and/or edentulism.
DOI: 10.1097/SCS.0b013e318190e23c
PubMed: 19165006
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<front><div type="abstract" xml:lang="en">Cherubism is a benign maxillary bone dysplasia of childhood, usually showing an autosomically dominant inheritance with variable penetrance and spontaneously resolving after puberty. Only maxillary bones are affected and develop pseudocystic osteolytic lesions. This article presents an early and rapidly evolving familial case of cherubism. The 3-year-old child underwent conservative curettage of lesions, with a conservative approach that allowed a normal permanent dentition in adolescence. Family history revealed that the father had been treated for similar lesions between 14 and 21 years of age, but the late treatment caused edentulism. In conclusion, although cherubism represents a benign and localized maxillary dysplasia, it requires prompt surgical but conservative treatment and careful follow-up to avoid permanent lesions, that is, malocclusion and/or edentulism.</div>
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