Orthodontic and surgical management of cleidocranial dysplasia
Park Tina Keun Nan, Vargervik Karin, Oberoi Snehlata,
소속 상세정보
( Park Tina Keun Nan ) - USA University of Southern California Ostrow School of Dentistry
( Vargervik Karin ) - USA University of California Orofacial Sciences
( Oberoi Snehlata ) - USA University of California Orofacial Sciences
KMID : 0361920130430050248
Abstract
Cleidocranial dysplasia (CCD), an autosomal dominant disorder with a prevalence of 1 in 1,000,000 individuals, is mainly caused by mutations in Runx2, a gene required for osteoblastic differentiation. It is generally characterized by hypoplastic clavicles, narrow thorax, and delayed or absent fontanel closure. Importantly, its orofacial manifestations, including midfacial hypoplasia, retained primary teeth, and impacted permanent and supernumerary teeth, severely impede the well-being of affected individuals. Successful treatment of the orofacial problems requires the combined efforts of dental specialists. However, only a few successfully treated cases have been reported because of the rarity of CCD and complexity of the treatment. This article presents the University of California, San Francisco (UCSF) treatment protocol for the dentofacial manifestations of CCD based on two treated and 17 diagnosed cases. The records of two patients with CCD who had been treated at the UCSF School of Dentistry and the treatment options reported in the literature were reviewed. The UCSF treatment protocol produced a successful case and a partially successful one (inadequate oral hygiene in the retention stage resulted in decay and loss of teeth). It provides general guidelines for successfully treating the orofacial manifestations of CCD.
키워드
Advancement; Craniofacial anomalies; Tooth impaction; Impaction
원문 및 링크아웃 정보
등재저널 정보