565 Gorlin Syndrome: A Case of Recurrence of Odontogenic Keratocysts

Abstract

Abstract Gorlin Syndrome (GS) is a rare condition with multisystemic manifestations. Known features include basal cell carcinomas, odontogenic keratocysts (OKCs), and skeletal abnormalities. Multidisciplinary involvement is paramount with surgical intervention as a common treatment modality. Multiple OKCs with recurrence is common in GS. However, limited up-to-date guidelines are available on the appropriate radiographic monitoring of such lesions, in the form of orthopantomogram, in these patients. This is a case of a 12-year-old girl with GS and multiple episodes of OKCs. She is monitored regularly with the Oral and Maxillofacial team since the age of 3, after her dentist referred her due to her condition and its association with OKCs. A multidisciplinary approach with Dermatology, Pediatrics, Ophthalmology, Ear Nose and Throat, and Orthodontics and Restorative Dentistry within the dental specialty is undertaken to manage her condition. Yearly clinical reviews of her mouth and radiographs biannually were done, with her first episode of OKCs presenting at 10 years old. Orthopantomogram and magnetic resonance imaging revealed 6 OKCs – 2 in the maxilla, 4 in the mandible. These were surgically enucleated with liquid nitrogen cryotherapy adjunct, alongside extractions of associated deciduous teeth. Eighteen months following this, her OPG showed 2 new OKCs – 1 in the maxilla, 1 in the mandible. These were removed successfully in a similar fashion and active monitoring is ongoing. This case underscores the importance of regular clinical and radiographic follow-ups due to the frequency of multiple OKCs with recurrence in GS. Long-term multidisciplinary approach in this multisystemic syndrome is also paramount.