Evaluation of dental maturity in Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis

Author:

Choi Tsun M1,Kramer Gem J C2,Goos Jacqueline A C3,Mathijssen Irene M J3,Wolvius Eppo B1,Ongkosuwito Edwin M1

Affiliation:

1. Department of Oral Maxillofacial Surgery, Special Dental Care and Orthodontics, Dutch Craniofacial Center, Erasmus MC, University Medical Center Rotterdam, The Netherlands

2. Department of Orthodontics, Academic Center for Dentistry Amsterdam, University of Amsterdam and Vrije Universiteit Amsterdam, The Netherlands

3. Department of Plastic and Reconstructive Surgery and Hand Surgery, Dutch Craniofacial Center, Erasmus MC, University Medical Center Rotterdam, The Netherlands

Abstract

Abstract Objectives To determine whether dental maturity (dental development) was delayed in patients with Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis, compared with a Dutch control group without syndromes. Materials and methods This study included 60 patients (38 patients with Muenke syndrome, 17 patients with Saethre-Chotzen syndrome, and 5 with TCF12-related craniosynostosis), aged 5.8–16.8 years that were treated at the Department of Oral Maxillofacial Surgery, Special Dental Care, and Orthodontics, in Sophia Children’s Hospital, Erasmus University Medical Center, Rotterdam, the Netherlands. Dental age was calculated according to Demirjian’s index of dental maturity. The control group included 451 children without a syndrome. Results Compared with the control group, dental development was delayed by an average of one year in 5- to 8-year-old patients with Muenke syndrome (P = 0.007) and in 8- to 10-year-old patients with Saethre-Chotzen syndrome (P = 0.044), but not in patients with TCF12-related craniosynostosis. Conclusions Our results indicated that dental development was delayed by one year, on average, in patients with Muenke syndrome and Saethre-Chotzen syndrome, compared with a Dutch control group without syndromes. Implications Our findings have improved the understanding of dental development in patients with Muenke and Saethre-Chotzen syndrome. These results can provide guidance on whether the orthodontist needs to consider growth disturbances related to dental development.

Publisher

Oxford University Press (OUP)

Subject

Orthodontics

Reference34 articles.

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3. Craniosynostosis in Western Australia, 1980–1994: a population-based study;Singer;American Journal of Medical Genetics,1999

4. A population-based study of craniosynostosis in metropolitan Atlanta, 1989–2003;Boulet;American Journal of Medical Genetics,2008

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