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
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
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: © 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.
ff
© 2024 Acta Scientific, All rights reserved.