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

Abstract EN

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.