Title of article :
Multiple, Multiloculated, and Recurrent Keratocysts of the Mandible and Maxilla in Association with Gorlin-Goltz (Nevoid Basal-Cell Carcinoma) Syndrome: A Pediatric Case Report and Follow-up over 5 Years
Author/Authors :
Santander, P. Department of Orthodontics - University Medical Center, Göttingen, Germany , Schwaibold, E. M. C. Institute of Human Genetics - University Medical Center, Göttingen, Germany , Bremmer, F. Institute of Pathology - University Medical Center, Göttingen, Germany , Batschkus, S. Department of Orthodontics - University Medical Center, Göttingen, Germany , Kauffmann, P. Department of Maxillofacial Surgery - University Medical Center, Göttingen, Germany
Abstract :
Background. We report a case of multiple keratocysts first diagnosed in an 8-year-old boy. Case report. The incidental radiographic
finding of a cystic lesion in an 8-year-old boy led to the surgical enucleation and further diagnosis of a keratocyst associated with a
tooth crown. In the course of dental maturation from deciduous to permanent teeth, the boy presented new lesions, always
associated with the crowns of teeth. Gorlin-Goltz (nevoid basal-cell carcinoma) syndrome was suspected, and the genetic
analysis detected a previously undescribed germline variant in the PTCH1 gene. Treatment. This included a surgical removal of
the cystic lesions, as well as the affected teeth. Follow-up. Due to the high recurrence rate of the keratocysts, frequent
radiological checks were performed over a 5-year period.
