Bijal Bhavsar¹*, Hiren Patel², Haren Pandya³, Smit Vithalani⁴, Nupur Hundia⁴ and Chetas Bhavsar⁵

¹Associate Professor, Department of Oral and Maxillofacial Surgery, Faculty of Dental Science, Dharamsinh Desai University, Nadiad, Gujarat, India
²Dean and Head of Department, Department of Oral and Maxillofacial Surgery, Faculty of Dental Science, Dharamsinh Desai University, Nadiad, Gujarat, India
³Professor, Department of Oral and Maxillofacial Surgery, Faculty of Dental Science, Dharamsinh Desai University, Nadiad, Gujarat, India
⁴Post Graduate Trainee, Department of Oral and Maxillofacial Surgery, Faculty of Dental Science, Dharamsinh Desai University, Nadiad, Gujarat, India
⁵B.D.S, Private Practitioner, Harsh Dental Clinic, Anand, India

*Corresponding Author: Bijal Bhavsar, Associate Professor, Department of Oral and Maxillofacial Surgery, Faculty of Dental Science, Dharamsinh Desai University, Nadiad, Gujarat, India.

Received: October 10, 2022; Published: November 08, 2022

Abstract

Gorlin-Goltz syndrome (GGS) is a rare uncommon multisystemic disease with an autosomal dominant inherited disorder characterized by the presence of multiple odontogenic keratocysts, basal cell nevi carcinomas and skeletal abnormalities are unique features of this autosomal dominant inherited disorder known as Goltz syndrome. Genetically abnormalities were noticed in the long arm of chromosome 9 and failure in mutation of human gene PTCH1 gene. Presence of major and minor criteria (Table 2) playing a major role in diagnosis of this kind of syndrome. Presenting a case of 18-year-old boy in favor of Gorlin syndrome having one major and six minor criteria adding to confirm diagnosis. Along with clinical features, radiological findings with the help of orthopantomogram, CT scan, chest X-ray help in the diagnosis of this particular syndrome, thus helps in prevention of recurrence and better survival rates because of early detection.

Keywords:Gorlin-Goltz Syndrome; Odontogenic Keratocysts; Basal Cell Nevi Carcinomas; Polysyndactaly; Bifid Ribs; Hypertelorism; Frontal Bossing

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Citation

Citation: Bijal Bhavsar., et al. “Gorlin Goltz Syndrome A Rare Entity: Case Report and Review of Literature". Acta Scientific Dental Sciences 6.11 (2022): 35-39.

Copyright

Copyright: © 2022 Bijal Bhavsar., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.