Gorlin-Goltz Syndrome with Multiple OKC in a 10-Year-Old Child: A Case Report

Abstract

Gorlin-Goltz syndrome is rare multi-system disease, which is, characterized by neoplasms and other developmental abnormalities. 1 It is a hereditary condition inherited as an autosomal dominant trait and caused by abnormalities in the PTCH1 (Patched1) gene which is traced to the long arm of chromosome 9q22.3-q31. It is characterized by the triad of multiple baso-cellular epitheliomas, odontogenic keratocysts (OKC) in the jaws and skeletal anomalies. Early diagnosis and treatment are important for long-term prognosis of the syndrome by reducing the severity of cutaneous carcinomas and deformities due to jaw cyst. Current case discusses a 10-year-old child suffering from Gorlin-Goltz syndrome, this case report emphasizes on early diagnosis and prompt treatment of such case. Keywords OKC, Gorlin-Goltz syndrome, Multi nodular radiolucency