• P-ISSN 2277-3525 E-ISSN 2582-7901

Journal of Multidisciplinary
Dental Research

Article

Journal of Multidisciplinary Dental Research

Volume: 10, Issue: 1, Pages: 17-20

Case Report

Gorlin-Goltz Syndrome with Multiple OKC in a 10-Year-Old Child: A Case Report

Received Date:26 February 2024, Accepted Date:01 April 2024, Published Date:22 July 2024

Abstract

Gorlin-Goltz syndrome is rare multi-system disease, which is, characterized by neoplasms and other developmental abnormalities. 1 It is a hereditary condition inherited as an autosomal dominant trait and caused by abnormalities in the PTCH1 (Patched1) gene which is traced to the long arm of chromosome 9q22.3-q31. It is characterized by the triad of multiple baso-cellular epitheliomas, odontogenic keratocysts (OKC) in the jaws and skeletal anomalies. Early diagnosis and treatment are important for long-term prognosis of the syndrome by reducing the severity of cutaneous carcinomas and deformities due to jaw cyst. Current case discusses a 10-year-old child suffering from Gorlin-Goltz syndrome, this case report emphasizes on early diagnosis and prompt treatment of such case.

Keywords

OKC, Gorlin-Goltz syndrome, Multi nodular radiolucency

References

  1. Kamil AH, Tarakji B. Odontogenic Keratocyst in Children: A ReviewOpen Dentistry Journal2016;10:117123. Available from: http://dx.doi.org/10.2174/1874210601610010117
  2. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, Digiovanna JJ, et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndromeAmerican Journal of Medical Genetics1997;69(3):299308. Available from: https://pubmed.ncbi.nlm.nih.gov/9096761/
  3. Rafiq S, Manzoor F, Dar MA, Aslam R. Imaging of Gorlin - Goltz syndrome: Series of 2 casesJournal of Oral and Maxillofacial Pathology2021;25(2):373. Available from: httpa://doi.org/10.4103/0973-029X.325261
  4. Manfredi M, Vescovi P, Bonanini M, Porter S. Nevoid basal cell carcinoma syndrome: A review of the literatureInternational Journal of Oral and Maxillofacial Surgery2004;33(2):117121. Available from: https://doi.org/10.1054/ijom.2003.0435
  5. Chaudhary S, Sinha A, Barua P, Mallikarjuna R. Keratocystic odontogenic tumour (KCOT) misdiagnosed as a dentigerous cystBMJ Case Reports2013;2013:15. Available from: https://doi.org/10.1136/bcr-2013-008741
  6. Kimonis VE, Mehta SG, Digiovanna JJ, Bale SJ, Pastakia B. Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndromeGenetics in Medicine2004;6(6):495502. Available from: https://doi.org/10.1097/01.gim.0000145045.17711.1c
  7. Lima FBDJB, Viana APC, Lima LHF, Ribeiro BC, Dutra CEA, Stabile GAV, et al. A Rare Case of Gorlin-Goltz Syndrome in ChildrenCase Reports in Dentistry2019;2019:15. Available from: https://doi.org/10.1155/2019/1608783
  8. Borghesi A, Nardi C, Giannitto C, Tironi A, Maroldi R, Bartolomeo FD, et al. Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviourInsights Imaging2018;9(5):883897. Available from: https://doi.org/10.1007/s13244-018-0644-z
  9. Tandon S, YC, Sharma M, Jain M. Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old ChildInternational Journal of Clinical Pediatric Dentistry2016;9(3):264268. Available from: https://doi.org/10.5005/jp-journals-10005-1374

COPYRIGHT

© 2024 Published by International Dental Educationists’ Association (IDEA). This is an open-access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/

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