Gorlin-goltz syndrome: A case report

Abstract

Gorlin- Goltz syndrome is an inherited autosomal dominant disorder. This shows a high level of penetrance and variable expressiveness. Early diagnosis of this syndrome is important for counseling of patients to prevent harmful exposure to Ultra-Violet and ionizing radiations that increase the risk of developing Basal Cell Carcinoma. A 18 years old male patient came with chief complaint of swelling in lower front region of jaw. Clinical examination done. Orthopantomograph revealed multiple multi-locular, well-defined radiolucency with sclerotic border located in maxilla and mandible. Relevant investigations were done including chest X-ray, Computed Tomography scan and Histopathological examination revealed presence of Gorlin- goltz syndrome. The Gorlin-Goltz syndrome is manifested by multiple defects involving the skin, nervous system, eyes, endocrine system, and bones. The presence of two major and one minor or one major and three minor criteria are necessary to establish diagnosis. The present reported case of Gorlin-Goltz syndrome fulfilled 3 major and 1 minor criteria. In the treatment of recurrent Odontogenic Keratocyst, associated with Gorlin- goltz syndrome, the overlying surface epithelium should be excised along with the cystic lining to prevent recurrence. This paper focus on the importance of oral and maxillofacial health professionals in the early diagnosis of Gorlin-Goltz syndrome and in a multidisciplinary approach to provide a better prognosis to the patient.