The korean journal of orthodontics (대한치과교정학회지)

The Korean Association Of Orthodontists (대한치과교정학회)
권호 표지
DOI QR코드

DOI QR Code

Orthodontic and surgical management of cleidocranial dysplasia

  • Park, Tina Keun Nan (Orthodontics Advanced Specialty Program, Ostrow School of Dentistry, University of Southern California) ;
  • Vargervik, Karin (Orofacial Sciences, University of California) ;
  • Oberoi, Snehlata (Orofacial Sciences, University of California)
  • Received : 2013.01.17
  • Accepted : 2013.06.03
  • Published : 2013.10.25
Download PDF
⟨ Previous Next ⟩

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.

Keywords

References

  1. Ishii K, Nielsen IL, Vargervik K. Characteristics of jaw growth in cleidocranial dysplasia. Cleft Palate Craniofac J 1998;35:161-6. https://doi.org/10.1597/1545-1569(1998)035<0161:COJGIC>2.3.CO;2
  2. Lou Y, Javed A, Hussain S, Colby J, Frederick D, Pratap J, et al. A Runx2 threshold for the cleidocranial dysplasia phenotype. Hum Mol Genet 2009; 18:556-68.
  3. Mundlos S, Otto F, Mundlos C, Mulliken JB, Aylsworth AS, Albright S, et al. Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia. Cell 1997;89:773-9. https://doi.org/10.1016/S0092-8674(00)80260-3
  4. Ducy P, Zhang R, Geoffroy V, Ridall AL, Karsenty G. Osf2/Cbfa1: a transcriptional activator of osteoblast differentiation. Cell 1997;89:747-54. https://doi.org/10.1016/S0092-8674(00)80257-3
  5. Baumert U, Golan I, Redlich M, Aknin JJ, Muessig D. Cleidocranial dysplasia: molecular genetic analysis and phenotypic-based description of a Middle European patient group. Am J Med Genet A 2005;139A:78-85. https://doi.org/10.1002/ajmg.a.30927
  6. Yamashiro T, Aberg T, Levanon D, Groner Y, Thesleff I. Expression of Runx1, -2 and -3 during tooth, palate and craniofacial bone development. Mech Dev 2002;119(Suppl 1):S107-10. https://doi.org/10.1016/S0925-4773(03)00101-1
  7. Lossdorfer S, Yildiz F, Gotz W, Kheralla Y, Jager A. Anabolic effect of intermittent PTH(1-34) on the local microenvironment during the late phase of periodontal repair in a rat model of tooth root resorption. Clin Oral Investig 2010;14:89-98. https://doi.org/10.1007/s00784-009-0263-7
  8. Manjunath K, Kavitha B, Saraswathi TR, Sivapathasundharam B, Manikandhan R. Cementum analysis in cleidocranial dysostosis. Indian J Dent Res 2008; 19:253-6. https://doi.org/10.4103/0970-9290.42960
  9. Counts AL, Rohrer MD, Prasad H, Bolen P. An assessment of root cementum in cleidocranial dysplasia. Angle Orthod 2001;71:293-8.
  10. Tanaka JL, Ono E, Filho EM, Castilho JC, Moraes LC, Moraes ME. Cleidocranial dysplasia: importance of radiographic images in diagnosis of the condition. J Oral Sci 2006;48:161-6. https://doi.org/10.2334/josnusd.48.161
  11. Suda N, Hattori M, Kosaki K, Banshodani A, Kozai K, Tanimoto K, et al. Correlation between genotype and supernumerary tooth formation in cleidocranial dysplasia. Orthod Craniofac Res 2010;13:197-202. https://doi.org/10.1111/j.1601-6343.2010.01495.x
  12. Becker A, Lustmann J, Shteyer A. Cleidocranial dysplasia: Part 1-General principles of the orthodontic and surgical treatment modality. Am J Orthod Dentofacial Orthop 1997;111:28-33. https://doi.org/10.1016/S0889-5406(97)70298-1
  13. Roberts T, Stephen L, Beighton P. Cleidocranial dysplasia: a review of the dental, historical, and practical implications with an overview of the South African experience. Oral Surg Oral Med Oral Pathol Oral Radiol 2013;115:46-55. https://doi.org/10.1016/j.oooo.2012.07.435
  14. Liu DG, Zhang WL, Zhang ZY, Wu YT, Ma XC. Threedimensional evaluations of supernumerary teeth using cone-beam computed tomography for 487 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:403-11. https://doi.org/10.1016/j.tripleo.2006.03.026

Cited by

  1. Combined surgical-orthodontic rehabilitation of cleidocranial dysplasia: 5 years follow-up vol.3, pp.8, 2013, https://doi.org/10.12998/wjcc.v3.i8.751
  2. Orthodontic Care of Cleidocranial Dysplasia Patients vol.28, pp.1, 2013, https://doi.org/10.13076/tjo-d-15-00013
  3. Surgical Management and Evaluation of the Craniofacial Growth and Morphology in Cleidocranial Dysplasia : vol.29, pp.4, 2013, https://doi.org/10.1097/scs.0000000000004334
  4. Delayed Eruption of Permanent Dentition and Maxillary Contraction in Patients with Cleidocranial Dysplasia: Review and Report of a Family vol.2018, pp.None, 2018, https://doi.org/10.1155/2018/6591414
  5. Characterization of dental phenotype in patients with cleidocranial dysplasia using longitudinal data vol.88, pp.4, 2018, https://doi.org/10.2319/092617-647.1
  6. Cleidocranial dysplasia-dental disorder treatment and audiology diagnosis vol.13, pp.1, 2018, https://doi.org/10.1515/med-2018-0001
  7. Therapeutic Protocol for Orthosurgical Management of Class III Malocclusion in Patients With Cleidocranial Dysostosis vol.29, pp.6, 2013, https://doi.org/10.1097/scs.0000000000004656
  8. Combined surgical-orthodontic treatment of patients with cleidocranial dysplasia: case report and review of the literature vol.13, pp.None, 2013, https://doi.org/10.1186/s13023-018-0959-3
  9. The Treatment Strategy of Cleidocranial Dysplasia : Combined Orthodontic and Orthognathic Treatment vol.30, pp.6, 2013, https://doi.org/10.1097/scs.0000000000005372