Clinical features and management of odontogenic keratocysts in Gorlin-Goltz syndrome.

Abstract

One of the frequent manifestations of Gorlin-Goltz syndrome (GGS), a rare autosomal dominant disorder, is the development of odontogenic keratocysts (OKCs). The aim of this study was to evaluate the efficacy and safety of treatment modalities for OKCs in GGS patients, and their corresponding recurrence/new lesion rates and complications. Twelve GGS patients (eight male, four female) with 36 OKCs were included. Common clinical features of GGS were calcification of the falx cerebri (eight patients, 66.7%), macrocephaly (seven patients, 58.3%), and frontal bossing (six patients, 50%). Enucleation with Carnoy's solution was the primary treatment for 10 patients, while two underwent marsupialization. The mean follow-up was 13.3 years. Nine (75%) of the patients did not experience any early complications (up to 1 month postoperative). The OKC recurrence/new lesion rate was 83.3% after surgical treatment (enucleation or marsupialization), with five patients (41.7%) experiencing two occurrences of OKCs during follow-up (range 2.7-29.9 years). Enucleation of OKCs with Carnoy's solution is a safe and effective approach in GGS patients. However, the high recurrence/new lesion rate underscores the need for close long-term monitoring for at least 7 years and consideration of more aggressive surgical methods in these patients.