Acta Scientific Dental Sciences (ASDS)(ISSN: 2581-4893)

Review Article Volume 6 Issue 12

Gorlin Goltz Syndrome A Rare Entity: Case Report and Review of Literature

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

References

  1. Casaroto AR., et al. “Early diagnosis of Gorlin Goltz syndrome: Case report”. Head and Face Medicine 7 (2011): 2.
  2. Lo Muzio L. “Nevoid basal cell carcinoma syndrome (Gorlin syndrome)”. Orphanet Journal of Rare Diseases 3 (2008): 32.
  3. Habibi A., et al. “Keratocystic odontogenic tumor: A 10-year retrospective study of 83 cases in an Iranian population”. Journal of Oral Science 49 (2007): 229-235.
  4. Kannan KS., et al. “Nevoid basal cell carcinoma syndrome”. Indian Journal of Dental Research 17 (2006): 50-53.
  5. Gorlin RJ and Goltz RW. “Multiple nevoid basal cell epithelioma, jaw cysts and bifid rib. A syndrome”. The New England Journal of Medicine 262 (1960): 908-912.
  6. Madras J and Lapointe H. “Keratocystic odontogenic tumour: Reclassification of the odontogenic keratocyst from cyst to tumour”. Journal of the Canadian Dental Association 74 (2008): 165-165h.
  7. Maria A., et al. “Marsupialization as a treatment option of a large odontogenic keratocyst: A case report with the review of literature”. People's Journal of Scientific Research 5 (2012): 46-51.
  8. Tang JY., et al. “Inhibiting the hedgehog pathway in patients with the basal cell nevus syndrome”. The New England Journal of Medicine 366 (2012): 2180-2188.
  9. Narsapur SA., et al. “Nonsyndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management”. Journal of Indian Academy of Oral Medicine and Radiology 27 (2015): 268-272.
  10. Marsh A., et al. “DHPLC analysis of patients with Nevoid Basal Cell Carcinoma Syndrome reveals novel PTCH missense mutations in the sterol sensing domain”. Human Mutation 26 (2005): 283.
  11. Gorlin RJ. “Nevoid basal cell carcinoma (Gorlin) syndrome”. Genetic Medicine 6 (2004): 530-539.
  12. Manfredi M., et al. “Nevoid basal cell carcinoma syndrome: A review of the literature”. International Journal of Oral and Maxillofacial Surgery 33 (2004): 117-124.
  13. Bhat KR., et al. “Gorlin Goltz syndrome A rare presentation”. Journal of Clinical and Diagnostic Research 4 (2010): 2899-2902.
  14. Kimonis V., et al. “Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome”. American Journal of Medical Genetics 69 (1997): 299-308.
  15. Dominguez FV and Keszler A. “Comparative study of keratocysts, associated and non associated with nevoid basal cell carcinoma syndrome”. Journal of Oral Pathology 17 (1988): 39-42.
  16. Lindeboom JA., et al. “Multiple recurrent and de novo odontogenic keratocysts associated with oral facial digital syndrome”. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 95 (2003): 458-462.
  17. th edition, (2007).

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.




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