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Home The role of the dentist and the orthodontist in early diagnosis of...

The role of the dentist and the orthodontist in early diagnosis of Gorlin-Goltz syndrome: a cephalometric and photometric study

Authors:

  • M.C. Meazzini
    DMD, MMSc, Regional Centre for Cleft lip and Palate, Orthodontic Service, Department of Maxillo Facial Surgery, Santi Paolo and Carlo Hospital, Milan
  • L.P. Demonte
    DDS, Regional Centre for Cleft lip and Palate, Orthodontic Service, Department of Maxillo Facial Surgery, Santi Paolo and Carlo Hospital, Milan - Department of Orthodontics, Vita e Salute University, San Raffaele Hospital, Milan
  • L. Conti
    DDS, Regional Centre for Cleft lip and Palate, Orthodontic Service, Department of Maxillo Facial Surgery, Santi Paolo and Carlo Hospital, Milan
  • L. Autelitano
    MD, Regional Centre for Cleft lip and Palate, Department of Maxillo Facial Surgery, Santi Paolo and Carlo Hospital, Milan
  • N. Cohen
    DDS, Regional Centre for Cleft lip and Palate, Orthodontic Service, Department of Maxillo Facial Surgery, Santi Paolo and Carlo Hospital, Milan
  • F. Mazzoleni
    MD, Department of Maxillo Facial Surgery, San Gerardo Hospital, Monza

DOI:

https://doi.org/10.23804/ejpd.2023.24.02.03

ABSTRACT


Aim

The aim of this study was to analyse the facial characteristics and the craniofacial morphology in GGS patients in order to enable an early
diagnosis.

Background

Gorlin-Goltz syndrome (GGS) is a autosomic dominant disease, characterised by basal cell carcinoma, palmar/plantar pits, maxillary and mandibular keratocysts and dental abnormalities.

Methods

Nine out of a sample of 24 GGS patients had complete cephalometric and photographic records at an average age of 8.7 years. Cephalometric and photometric analysis were carried out with standard analyses and compared with healthy patients matched for sex and age.

Results

Photometric data: Significantly increased facial convexity, transverse facial dimensions, telecanthus and increased transverse nasal dimensions were found compared to the matched control patients. Cephalometric data: Hyperdivergence of the skeletal bases, skeletal Class III malocclusion associated to retrusion and hypoplasia of the maxilla and increased lower facial height were found in all patients compared to control patients. Patients affected by GGS have a characteristic craniofacial phenotype which clinicians might be able to identify for early diagnosis of developing jaw keratocysts.

Conclusion

Early diagnosis of GGS based on clinical features could be useful to identify the presence of keratocysts through x-ray examination proceeding with surgical removal at an early stage, limiting space occupying damages.

PLUMX METRICS

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Publication date:

June /2023

Keywords:

Gorlin-Goltz syndrome, Keratocyst, Photometric analysis, basal cell carcinoma, cephalometric analysis, craniofacial morphology

Issue:

Vol.24 – n.2/2023

Page:

161 – 165

Publisher:

Ariesdue

Topic:

Any other topic

Cite:


Harvard: M.C. Meazzini, L.P. Demonte, L. Conti, L. Autelitano, N. Cohen, F. Mazzoleni (2023) "The role of the dentist and the orthodontist in early diagnosis of Gorlin-Goltz syndrome: a cephalometric and photometric study", European Journal of Paediatric Dentistry, 24(2), pp161-165. doi: 10.23804/ejpd.2023.24.02.03
Vancouver: M.C. Meazzini, L.P. Demonte, L. Conti, L. Autelitano, N. Cohen, F. Mazzoleni. The role of the dentist and the orthodontist in early diagnosis of Gorlin-Goltz syndrome: a cephalometric and photometric study. European Journal of Paediatric Dentistry [Internet]. 2023Jun.22 [cited 2023May.28];24(2):161-165. Available from: https://www.ejpd.eu/abstract-pubmed/the-role-of-the-dentist-and-the-orthodontist-in-early-diagnosis-of-gorlin-goltz-syndrome-a-cephalometric-and-photometric-study/
MLA: M.C. Meazzini, L.P. Demonte, L. Conti, L. Autelitano, N. Cohen, F. Mazzoleni The role of the dentist and the orthodontist in early diagnosis of Gorlin-Goltz syndrome: a cephalometric and photometric study. European Journal of Paediatric Dentistry. 2023;24(2):161-165

Copyright (c) 2021 Ariesdue

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

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    Editor in chief: dott. Luigi Paglia
    European Journal of Paediatric Dentistry © | ISSN (Online): 2035-648X
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    European Journal of Paediatric Dentistry © | ISSN (Online): 2035-648X
    Registrazione del Tribunale di Milano n. 285 del 14.04.1998 | ROC 1946 - 26.09.2001
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