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

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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.

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