Gorlin-goltz syndrome

• Main Authors: B V Shobha, S Mosby, N Barkha, Biswajeet, M Ajit, T Deepak
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2011-01-01
• Series: Journal of Indian Academy of Oral Medicine and Radiology
• Subjects: Gorlin-Goltz syndrome; Keratocvstic odontogenic tumor; Basal cell nevus syndrome
• Online Access: http://www.jiaomr.in/article.asp?issn=0972-1363;year=2011;volume=23;issue=5;spage=487;epage=490;aulast=Shobha
• ISSN: 0972-1363; 0975-1572

Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS) is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.