Prague Medical Report (Feb 2024)

Gorlin-Goltz Syndrome – A Rare Case Entity in Young Child

  • Shovita Mondal,
  • Nikil Kumar Jain,
  • Abhishek Dutta,
  • Nishant,
  • Abhijit Dutta,
  • Moushumi Shil,
  • Suman Sen

DOI
https://doi.org/10.14712/23362936.2024.7
Journal volume & issue
Vol. 125, no. 1
pp. 69 – 78

Abstract

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Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant trait, which depicted presence of numerous basal cell carcinoma in conjunction with multiorgan abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the keratocystic odontogenic tumour are usually one of the first manifestations of the syndrome. This article includes a case report of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumour and treatment modalities.