EC Paediatrics

Review Article Volume 13 Issue 9 - 2024

Gorlin-Goltz Syndrome: Surgical Resolution of Keratocysts in 3 Generations of a Family. Case Presentation

Jeremias Roman1, Sebastian Miguelez2, Guillermo Sica3, Loredana Alessandra Antonucci Farias4, Jonathan Bavaro5, Romina Testi6 and Christian Oscar Mosca7*

1Dentist unlp, Specialist in oral implantology Maimonides, Od. HIGA Pte. Perón Plant, Argentina
 2Dentist HIGA Pte Perón, Specialist in Oral Maxillofacial Surgery and Traumatology, Specialist in Prosthodontics, Associate Professor of Surgery U.A.J.F.K, Professor in Seminar Workshop 1 U.A.I., Argentina
 3Dentist, Specialist in Oral and Maxillofacial Surgery and Traumatology, Professor of the Postgraduate Course in Surgery at U.C.A. and U.N.N.E., Surgeon at the Sanatorio Trinidad de Quilmes and Mitre, Surgeon at the Sanatorio Bernal in the Head and Neck Service, Argentina
 4Dentist from UNLP, Third Year Resident at the Interzonal General Acute Hospital Pte Perón, Argentina
 5Dentist from UNLP, Full-Time Dentist, Teaching Coordinator and Head of the Prosthesis Unit at the Interzonal General Acute Hospital Pte Perón, Argentina
 6Dentist uba, Dentist Interim HIGA Pte Perón, Argentina
 7Dentist at UBA, Specialist in Surgery and Traumatology at BMF, Doctor in Public Health, Associate Professor of the Microbiology and Parasitology Course at UNO, Professor of the Infectology Course at the Esp de CBMF UMAI, Adjunct Professor of Microbiology and Immunology at UK, Advisory Professor at HIGA Pte Perón, Argentina

*Corresponding Author: Christian Oscar Mosca, HIGA Pte Peron Dental Service, HIGA Pte Peron, Cuidad Autonoma de Buenos Aires, Argentina.
Received: August 09, 2024; Published: September 02, 2024




Gorlin Goltz syndrome (GGS) was reported in 1984. It is a genetic disease with autosomal dominant inheritance. Its 3 typical characteristics are: basal cell carcinomas, multiple maxillary keratocysts (OCK) and bifid rib syndrome. Its diagnosis is established when 2 major criteria are met or one major and 2 minor criteria originally defined by Evans., et al. in 1993 and subsequently modified by Kimonis., et al. in 1997 and Bree., et al. in 2011.

Its treatment requires a multidisciplinary approach. The dentist is a main actor when multiple osteolytic lesions are found in the jaws in panoramic radiographs requested during the consultation.

The case of a family where 3 generations, a mother, 5 children and her grandson present clinical manifestations of the syndrome is presented below. These patients were treated for maxillary keratocysts and are still under treatment.

All surgeries performed have been conservative approaches so far.

 Keywords: Basal Cell Nevus Syndrome; Odontogenic Cysts; Congenital Anomalies; Medulloblastoma; Keratocyst; Gorlin-Goltz Syndrome

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Christian Oscar Mosca., et al. "Gorlin-Goltz Syndrome: Surgical Resolution of Keratocysts in 3 Generations of a Family. Case Presentation". EC Paediatrics 13.9 (2024): 01-33.

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