From the diagnosis of a mandibular cystic lesion to the surgical management of a case of a nevoid basal cell carcinoma syndrome

Introduction: Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is a rare hereditary disease with autosomal dominant transmission. Multiple basal cell carcinomas, keratocysts located in the jaw, and developmental abnormalities are common clinical manifestations. Observation: The article reports the case of a 10-year-old patient addressed for the diagnostic and therapeutic management of a mandibular cystic lesion. Keratocysts can be the inaugural manifestation of Gorlin-Goltz syndrome. The originality of this case lies in the conservative approach adopted in this disease to treat a wide-ranging cystic lesion, using a cystic decompression method. Clinically, the patient had macrocephaly and hypertelorism, clinical signs frequently associated with Gorlin-Goltz syndrome, confirmed by the discovery of a mutation of the PTCH1 gene through genetic testing. Conclusion: This case highlights the importance of a multidisciplinary approach in the management of Gorlin-Goltz syndrome, by integrating innovative surgical techniques such as cystic decompression.