Skip to main content

Advertisement

Log in

Cranio-maxillofacial, orthodontic and dental treatment in three patients with Apert syndrome

  • Case Report
  • Published:
European Archives of Paediatric Dentistry Aims and scope Submit manuscript

Abstract

Background

Apert syndrome is a severe developmental malformation, clinically characterised by craniosynostosis, midface hypoplasia, a cone-shaped calvarium, ocular manifestations, typical dental findings and syndactyly of the hands and feet. Early craniosynostosis of the coronal suture, the cranial base and agenesis of the sagittal suture are prodromal characteristics for the typical craniofacial appearance in patients with Apert syndrome.

Case reports

The aim of this report was to describe the maxillofacial and orthodontic management of three patients with Apert syndrome who attended the Craniofacial, Maxillofacial and Orthodontic clinics of the University Hospitals of the KU Leuven (Belgium). The typical clinical features, the general orthognathic treatment approach as well as individual approaches of three patients with Apert syndrome are being highlighted.

Follow-up

The three patients with Apert syndrome have been followed up very closely by all involved specialised departments as well as by multidisciplinary teams from birth.

Conclusion

This report demonstrated that a combined orthodontic and orthognathic surgical treatment plan could significantly improve the occlusal function as well as the facial and occlusal aesthetics in patients with Apert syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  • Allam KA, Wan DC, Khwanngern K, et al. Treatment of Apert syndrome: a long-term follow-up study. Plast Reconstr Surg. 2011;127:1601–11.

    Article  PubMed  Google Scholar 

  • Apert ME. De l’acrocephalosyndactylie. Bull Mem Soc Med Hop (Paris). 1906;23:1310–30.

    Google Scholar 

  • Avantaggiato A, Carinci F, Curioni C. Apert’s syndrome: cephalometric evaluation and considerations on pathogenesis. J Craniofac Surg. 1996;7:23–31.

    Article  PubMed  Google Scholar 

  • Cohen MM Jr, Kreiborg S. The central nervous system in the Apert syndrome. Am J Med Genet. 1990;35:36–45.

    Article  PubMed  Google Scholar 

  • Cohen MM Jr, Kreiborg S, Lammer EJ, et al. Birth prevalence study of the Apert syndrome. Am J Med Genet. 1992;42:655–9.

    Article  PubMed  Google Scholar 

  • Cohen MM Jr. Sutural biology and the correlates of craniosynostosis. Am J Med Genet. 1993;47:581–616.

    Article  PubMed  Google Scholar 

  • Cohen MM Jr, Kreiborg S. Cutaneous manifestations of Apert syndrome. Am J Med Genet. 1995;58:94–6.

    Article  PubMed  Google Scholar 

  • Figueroa AA, Polley JW, Friede H, et al. Long-term skeletal stability after maxillary advancement with distraction osteogenesis using a rigid external distraction device in cleft maxillary deformities. Plast Reconstr Surg. 2004;114:1382–92.

    Article  PubMed  Google Scholar 

  • Iannetti G, Fadda T, Agrillo A, et al. LeFort III advancement with and without osteogenesis distraction. J Craniofac Surg. 2006;17:536–43.

    Article  PubMed  Google Scholar 

  • Kreiborg S, Cohen MM Jr. Characteristics of the infant Apert skull and its subsequent development. J Craniofac Genet Dev Biol. 1990;10:399–410.

    PubMed  Google Scholar 

  • Letra A, de Almeida AL, Kaizer R, et al. Intraoral features of Apert’s syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;103:38–41.

    Article  Google Scholar 

  • Proff P, Will F, Bokan I, et al. Cranial base features in skeletal class III patients. Angle Orthod. 2008;78:433–9.

    Article  PubMed  Google Scholar 

  • Reitsma JH, Ongkosuwito EM, Buschang PH, et al. Craniofacial stability in patients with the syndrome of Crouzon and Apert after Le Fort III distraction osteogenesis. Cleft Palate Craniofac J. 2012 (Epub ahead of print).

  • Rynearson RD. Case report: orthodontic and dentofacial orthopedic considerations in Apert’s syndrome. Angle Orthod. 2000;70:247–52.

    PubMed  Google Scholar 

  • Shetye PR, Davidson EH, Sorkin M, et al. Evaluation of three surgical techniques for advancement of the midface in growing children with syndromic craniosynostosis. Plast Reconstr Surg. 2010;126:982–94.

    Article  PubMed  Google Scholar 

  • Vadiati Saberi B, Shakoorpour A. Apert syndrome: report of a case with emphasis on oral manifestations. J Dent (Tehran). 2011; 8:90–95.

    Google Scholar 

  • Verdonck A, Bertrand J, Carels C, et al. Orthodontic and orthognathic management of a patient with Apert syndrome: a case report. J Orthod. 2010;37:121–7.

    Article  PubMed  Google Scholar 

  • Wilkie AO, Wall SA. Craniosynostosis: novel insights into pathogenesis and treatment. Current Opin Neurol. 1996;9:146–52.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to A. Verdonck.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Carpentier, S., Schoenaers, J., Carels, C. et al. Cranio-maxillofacial, orthodontic and dental treatment in three patients with Apert syndrome. Eur Arch Paediatr Dent 15, 281–289 (2014). https://doi.org/10.1007/s40368-013-0105-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40368-013-0105-9

Keywords

Navigation