Korean J Orthod.  2013 Oct;43(5):248-260. 10.4041/kjod.2013.43.5.248.

Orthodontic and surgical management of cleidocranial dysplasia

• Park TK
• Vargervik
• Oberoi S

• KMID: 2273417
• DOI: http://doi.org/10.4041/kjod.2013.43.5.248

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.

Keyword

Advancement; Craniofacial anomalies; Tooth impaction; Impaction

MeSH Terms

Clavicle
Cleidocranial Dysplasia*
Clinical Protocols
Dentistry
Humans
Oral Hygiene
Osteoblasts
Prevalence
Thorax
Tooth, Deciduous
Tooth, Supernumerary

Figure

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  Figure 1 Initial record of patient 1 at age 10 years and 11 months. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view; E, panoramic radiograph; and F, lateral cephalogram.

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  Figure 2 Progress record of patient 1 at age 17 years. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view; E, panoramic radiograph; and F, lateral cephalogram.

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  Figure 3 Progress record of patient 1 at age 23 years. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view; E, panoramic radiograph; and F, lateral cephalogram.

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  Figure 4 Final record of patient 1 at age 24 years. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view.

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  Figure 5 Superimposition of the pretreatment (dotted line) and posttreatment (solid line) lateral cephalograms in case 1.

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  Figure 6 Initial record of patient 2 at age 14 years. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view; E, panoramic radiograph; and F, lateral cephalogram.

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  Figure 7 Progress record of patient 2 at age 17 years (A and B), 15 years (C and D), and 16 years (E and F). A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view; E, panoramic radiograph; and F, lateral cephalogram.

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  Figure 8 Final record of patient 2 at age 18 years. A, Frontal facial view; B, lateral facial view; C, frontal intraoral view; D, lateral intraoral view.

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  Figure 9 Superimposition of the pretreatment (dotted line) and posttreatment (solid line) lateral cephalograms in case 2.

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