International Journal of Clinical Pediatric Dentistry

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VOLUME 9 , ISSUE 3 ( 2016 ) > List of Articles

CASE REPORT

Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child

Manish Jain, Yashwant Chauhan

Citation Information : Jain M, Chauhan Y. Gorlin-Goltz Syndrome: A Rare Case Report of a 11-Year-Old Child. Int J Clin Pediatr Dent 2016; 9 (3):264-268.

DOI: 10.5005/jp-journals-10005-1374

License: CC BY-NC 4.0

Published Online: 00-09-2016

Copyright Statement:  Copyright © 2016; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Gorlin-Goltz Syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant trait caused due to mutations in the patched tumor suppressor gene (PTCH) gene found on the long arm of chromosome 9. The syndrome is characterized by the presence of odontogenic keratocysts (OKCs), basal cell carcinomas, and skeletal malformations. Early diagnosis of the syndrome can be done by pedodontist as OKC is one of the early manifestations of the syndrome. Early diagnosis and treatment is important for long-term prognosis of the syndrome by reducing the severity of cutaneous carcinomas and deformities due to jaw cyst. The present case describes an 11-year-old patient with some typical features of NBCCS, which were diagnosed through its oral and maxillofacial manifestations. This case emphasizes the importance of pedodontist in early recognition of the syndrome.


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