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. 2022 May 24;44(3):287-293.
doi: 10.1093/ejo/cjab056.

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

Tsun M Choi  1 , Gem J C Kramer  2 , Jacqueline A C Goos  3 , Irene M J Mathijssen  3 , Eppo B Wolvius  1 , Edwin M Ongkosuwito  1
Affiliations

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

Tsun M Choi et al. Eur J Orthod. .

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.

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Figures

Figure 1.
Figure 1.
Flowchart displays the patient selection process. DPR, dental panoramic radiograph.
Figure 2.
Figure 2.
Age distributions of patients with Muenke syndrome (a), Saethre-Chotzen syndrome (SCS) (b), or TCF12-related craniosynostosis (TCF12) (c), and the control group (d).
Figure 3.
Figure 3.
Dental maturity scores according to age. Logistic curve fit shows that, in most groups, dental maturation occurs before the age of 10 years. Vertical lines indicate the age groups analysed.
Figure 4.
Figure 4.
Dental maturity scores according to different ages and groups. Comparisons of dental maturity are shown for (a) children 5–8 years old; (b) children 8–10 years old; (c) children 10–15 years old. Data are presented as the median and 5–95 percentiles. *Statistical significantly difference between the four groups P < 0.05. **Statistical significantly difference in Muenke patients compared with the controls P < 0.05. SCS, Saethre-Chotzen syndrome; TCF12, TCF12-related craniosynostosis.
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