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VOL. 8, ISSUE 2 (2026)
Multiple odontogenic keratocysts in siblings with Gorlin-Goltz Syndrome: A two case series
Authors
Sandy Victoria Azevedo de Souza, Jonathan Ribeiro da Silva, Laryssa dos Santos Pinheiro, Júlia Morais Moreira
Abstract
Gorlin-Goltz syndrome (GGS) is a rare hereditary disorder characterized
by multisystem developmental abnormalities involving the skin, skeleton, and
jaws, most associated with pathogenic variants in the PTCH1 gene. We
report a familial occurrence of multiple odontogenic keratocysts (OKCs) in two paediatric
siblings from South America. A 9-year-old boy and his 7-year-old sister
presented with multiple cystic jaw lesions detected incidentally. Imaging
revealed multiple hypodense lesions involving the jaws, with calcification of
the falx cerebri in Case 1 and bifid ribs in Case 2. Histopathological analysis
confirmed the diagnosis of OKC. Management consisted of a staged conservative
surgical approach, including decompression followed by enucleation and
curettage. Satisfactory healing was observed, with no recurrence of treated
lesions during a minimum follow-up period of 24 months. This report highlights
the importance of early diagnosis, multidisciplinary evaluation, and
conservative management to preserve function and craniofacial development in paediatric
patients.
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Pages:139-144
How to cite this article:
Sandy Victoria Azevedo de Souza, Jonathan Ribeiro da Silva, Laryssa dos Santos Pinheiro, Júlia Morais Moreira "Multiple odontogenic keratocysts in siblings with Gorlin-Goltz Syndrome: A two case series". International Journal of Dental Sciences, Vol 8, Issue 2, 2026, Pages 139-144
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