Interdisciplinary management of the diagnosis and pediatric dentistry treatment plan of Gorlin-Goltz Syndrome: Case report

Abstract

The Gorlin-Goltz Syndrome (GGS) also known as the Cell Nevoid Basal Carcinoma, is a hereditary autosomal dominant disorder, was described by Robert Gorlin and Robert Goltz as a syndrome which presenting multiple basal cell carcinomas, keratocyst and bifid ribs. The diagnosis is established by various clinical signs called primary and secondary, which involving both clinical and radiological signs. This case is rare because to be a pediatric patient with delayed in his diagnosis, who is referred with a sequel of extensive bone destruction in the jaw, caused by aggressive odontogenic keratocyst tumor.This case shows the important role of the pediatric dentist who interelations with various medical specialties to obtain a definitive diagnosis, surgical treatment planning and establishment of preventive measures to reduce the consequences both medical and dental to improve the quality of life of the patients.