Craniomaxillofacial anomalies in gorlin–goltz syndrome: A description of clinical cases

Gorlin–Goltz syndrome is an autosomal dominant rare phacomatosis characterized by multiple odontogenic keratocysts, rib splitting, multiple basal cell carcinomas and other anomalies. The most frequent early manifestation of this disease is multiple keratocysts, therefore the task of it often falls with dentists, maxillofacial and pediatric surgeons. Materials and methods: authors performed a retrospective analysis of treated patients to assess a correlation between the presence of large odontogenic cysts and Gorlin–Goltz syndrome. All children underwent computed tomography at the preoperative stage, followed by surgical treatment that included cystectomy. Results: multiple or large odontogenic cysts in young patients require a complete examination with computed tomography of the entire skull to detect a calcification falx cerebri, which was found in 4 out of 7 patients with Gorlin-Goltz syndrome. In all the cases, the lower jaw was affected by keratocysts prior to foci appearing on the posterior parts of the upper jaw. The surgical treatment must include cysts resection with the embedded teeth and placement of the xeroform turunda into the tumor bed to reduce the risk of relapse. Conclusion: an early diagnosis of Gorlin-Goltz syndrome plays a crucial role in avoiding severe complications such as maxillofacial deformities associated with jaw cysts. © 2020, Pediatria Ltd.. All rights reserved.