Year : 2015

Volume : Volume 5

Issue : Issue 2

Address for correspondence :
Dr. Naveen Mynam
naveenm@gmail.com

Abstract

Background:  The Gorlin–Goltz syndrome, also known as Nevoid Basal Cell Carcinoma syndrome(NBCCS) is an infrequent multisystemic disease that is inherited as an autosomal dominant condition shows a high level of penetrance & variable expressivisity. It is characterized by the presence of multiple Keratocystic odontogenic tumor (KCOT) in the jaws, multiple basal cell nevi or carcinomas and skeletal abnormalities. As most of the clinical manifestations of this syndrome are pertained to head and neck region, this syndrome is commonly encountered maxillofacial surgeon. Having sound knowledge about diagnostic criteria and genetic biology of this syndrome improves the treatment outcome and avoid associated complications. This case report along with review, highlights possible percentage of commonly appearing clinical manifestations of syndrome and importance of awareness among physicians or surgeons who encounter this condition so as to improve prognosis and prevent further risks.

Keywords: Gorlin - Goltz Syndrome, Nevoid basal cell carcinoma syndrome, Multiple  odontogenic keratocyst, Falx  cerebri.