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European Archives of Paediatric Dentistry

, Volume 18, Issue 2, pp 127–133 | Cite as

Frontonasal dysplasia: oral features, restorative and orthodontic dental treatment in a child

  • R. A. ValérioEmail author
  • C. Scatena
  • F. R. R. Santos
  • F. L. Romano
  • A. M. Queiroz
  • F. W. G. Paula-Silva
Case Report
  • 211 Downloads

Abstract

Background

Frontonasal dysplasia is a complex rare malformation, characterised by abnormalities involving the central portion of the face, especially the eyes, nose and forehead. It can manifest independently or associated with other abnormalities as part of some syndromes.

Case report

The purpose of this case report was to describe a 5-year-old patient, diagnosed with frontonasal dysplasia. Among the abnormalities characterised with this disorder were ocular hypertelorism, broad nose tip with median notch, median facial cleft, bifid anterior skull, low set hairline, Poland’s syndactyly and ankyloglossia.

Treatment

Consisted of behavioural management, oral hygiene instruction, prophylaxis, topical fluoride application, extraction of primary teeth, composite resin restorations and sealants in pits and fissures. Preformed metal crowns were also applied to the right and left primary maxillary second molars.

Follow-up

Currently, the patient is 11 years-old in the permanent dentition and therefore was referred for corrective orthodontic and periodontal treatments due to the persistence of gingival retraction of the permanent mandibular right central incisor.

Conclusion

The treatment in this case was directed to the promotion of oral health and orthodontic corrections, which are of fundamental importance due to medical, physical and social limitations of children affected by this syndrome, hindering healing and rehabilitative treatment. Paediatric dentists should be included in multidisciplinary teams providing care to patients with special needs, improving their quality of life.

Keywords

Frontonasal dysplasia Craniofacial abnormalities Preventive dentistry 

Notes

Compliance with ethical standard

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. Antonelli MZ, Giacheti CM, Genaro KF, Zorzetto NL, Costa AR. Frontonasal dysplasia: clinical evaluation on audiological and brainstem electrophysiological profiles. Braz J Otorhinolaryngol. 2011;5:611–5.CrossRefGoogle Scholar
  2. Cohen MM Jr. Craniofrontonasal dysplasia. Birth Defects. 1979;15:85–9.PubMedGoogle Scholar
  3. De Menezes Oliveira MA, Torres CP, Gomes-Silva JM, et al. Microstructure and mineral composition of dental enamel of permanent and deciduous teeth. Microsc Res Tech. 2010;73:572–7.PubMedGoogle Scholar
  4. De Moor MMA, Baruch R, Human DG. Frontonasal dysplasia associated with tetralogy of Fallot. J Med Genet. 1987;24:107–9.CrossRefPubMedPubMedCentralGoogle Scholar
  5. DeMyer W. The median cleft face syndrome. Differential diagnosis of cranium bifidum occultum, hypertelorism, and median cleft nose, lip, and palate. Neurology. 1967;17:961–71.CrossRefPubMedGoogle Scholar
  6. Gaball CW, Yencha MW, Kosnik S. Frontonasal dysplasia. Otolaryngol Head Neck Surg. 2005;133:637–8.CrossRefPubMedGoogle Scholar
  7. Guion-Almeida ML, Richieri-Costa A, Saavedra D, et al. Frontonasal dysplasia: analysis of 21 cases and literature review. Int J Oral Maxillofac Surg. 1996;25:91–7.CrossRefPubMedGoogle Scholar
  8. Haro Montero MM, Romero Maroto M, Bravo Gonzalez LA, Sanchez del Pozo J. New dental findings in the median cleft facial syndrome. J Am Dent Assoc. 2005;136:631–4.CrossRefPubMedGoogle Scholar
  9. Howe RP, MacNamara JA, O’Conner KA. An examination of dental crowding and its relationship to tooth size and arch dimension. Am J Orthod. 1983;83:263–73.CrossRefGoogle Scholar
  10. Johnstone E, Glanville T, Pilling J, Dobbie A. Prenatal diagnosis of frontonasal dysplasia using 3D ultrasound. Prenat Diagn. 2008;28:1075–6.CrossRefPubMedGoogle Scholar
  11. Kayserili H, Uz E, Niessen C, et al. ALX4 dysfunction disrupts craniofacial and epidermal development. Hum Mol Genet. 2009;22:4357–66.CrossRefGoogle Scholar
  12. Kean J, Al-Busaidi SS, Quaba AA. A case report of frontonasal dysplasia. Int J Pediatr Otorhinolaryngol. 2010;74:306–8.CrossRefPubMedGoogle Scholar
  13. MacNamara JR. Influence of respiratory pattern on craniofacial growth. Angle Orthod. 1981;51:269–300.Google Scholar
  14. McCann MC, McCann KE. Frontonasal dysplasia and its orodental aspects. J Dent. 1973;2:69–71.CrossRefPubMedGoogle Scholar
  15. Mohammed SN, Swan MC, Wall SA, Wilkie AOM. Monozygotic twins discordant for frontonasal malformation. Am J Med Genet A. 2004;130A:384–8.CrossRefPubMedGoogle Scholar
  16. Pascual-Castroviejo I, Pascual-Pascual SI, Pérez-Higueras A. Fronto-nasal dysplasia and lipoma of the corpus callosum. Eur J Pediatr. 1985;144:66–71.CrossRefPubMedGoogle Scholar
  17. Petrova EG, Hyman M, Estrella MR, Inglehart MR. Children with special health care needs: exploring the relationships between patients’ level of functioning, their oral health, and caregivers’ oral health-related responses. Pediatr Dent. 2014;36:233–9.PubMedGoogle Scholar
  18. Rizvi M, Lypka MA, Kovacev T, Agris J. Median facial cleft dysmorphism in three siblings: case report and review of the literature. Cleft Palate Craniofac J. 2010;47:104–6.CrossRefPubMedGoogle Scholar
  19. Rodrigues dos Santos MT, Masiero D, Novo NF, Simionato MR. Oral conditions in children with cerebral palsy. J Dent Child. 2003;70:40–6.Google Scholar
  20. Sedano HO, Cohen MM Jr, Jirasek J, Gorlin RJ. Frontonasal dysplasia. J Pediatr. 1970;76:906–13.CrossRefPubMedGoogle Scholar
  21. Sedano HO, Gorlin RJ. Frontonasal malformation as a field defect and in syndromic associations. Oral Surg Oral Med Oral Pathol. 1988;65:704–10.CrossRefPubMedGoogle Scholar
  22. Schwarz AM, Gratzinger M. Removable orthodontic appliances. Philadelphia: WB Saunders; 1966. p. 61–83.Google Scholar
  23. Skinner L, Hickson L. A case study of partial agenesis of the corpus callosum: audiological implications. Aust N Z J Audiol. 2002;24:36–45.CrossRefGoogle Scholar
  24. Twigg SRF, Versnel SL, Nurnberg G, et al. Frontorhiny, a distinctive presentation of frontonasal dysplasia caused by recessive mutations in the ALX3 homeobox gene. Am J Hum Genet. 2009;84:698–705.CrossRefPubMedPubMedCentralGoogle Scholar
  25. Vegesna S, Gutthi L, Varanasi P, Gandhi TP. Frontonasal dysplasia with severe occipital lobe hypoplasia. Indian J Pediatr. 2014;81:1133–4.CrossRefPubMedGoogle Scholar

Copyright information

© European Academy of Paediatric Dentistry 2017

Authors and Affiliations

  • R. A. Valério
    • 1
    • 2
    Email author
  • C. Scatena
    • 1
  • F. R. R. Santos
    • 1
  • F. L. Romano
    • 1
  • A. M. Queiroz
    • 1
  • F. W. G. Paula-Silva
    • 1
  1. 1.Department of Paediatric Clinics, School of Dentistry of Ribeirão PretoUniversity of São PauloRibeirão PretoBrazil
  2. 2.School of Dentistry of Ribeirão PretoUniversity of São PauloRibeirão PretoBrazil

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