Gorlin-Goltz Syndrome Associated with Cleft Lip and Palate a Case Report

  

  Priyanthi M. , I. Packia Raj , N. Dhineksh Kumar , Kala Bagavathy

   DOI : https://doi.org/

 Abstract

Gorlin-Goltz syndrome, also known as Nevoid Basal Cell Carcinoma Syndrome (NBCCS), was first described by Gorlin and Goltz in 1960. It is a rare autosomal dominant disease characterized by several major manifestations and less common minor manifestations. One of the infrequent findings of this syndrome is orofacial clefts. Diagnosis of this syndrome is made based on clinical and radiological criteria. In this article, we present the case of a 19-year-old girl who underwent surgery for complete unilateral Cleft Lip and Palate (CLP) and later developed a cleft alveolus. Radiological examination revealed the presence of multiple Keratocystic Odontogenic Tumors (KCOT). Following the criteria given by Kimonis et al., the patient was diagnosed with Gorlin-Goltz syndrome. A combination of enucleation and Pogrel's technique was used to treat the cysts.

 Keywords

Gorlin-Goltz Syndrome, Nevoid Basal Cell Carcinoma Syndrome, Orofacial Clefts, Cleft Lip and Palate, Keratocystic Odontogenic Tumor, KCOT, Enucleation, Pogrel's Technique.

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