Indian Journal of Public Health Research & Development
  • Year: 2019
  • Volume: 10
  • Issue: 11

Gorlin-Goltz Syndrome: Multiple Approach to Diagnosis and Treatment

1Professor and Head, Department of Oral & Maxillofacial Pathology & Microbiology, Institute of Dental Sciences, Siksha ‘O’ Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India

2PhD Scholar, Central Research Laboratory, Institute of Dental Sciences, Siksha ‘O’ Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India

3Senior Lecturer, Department of Oral Medicine & Radiology, Institute of Dental Sciences, Siksha ‘O’ Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India

*Corresponding Author: Neeta Mohanty, Professor and Head, Department of Oral & Maxillofacial Pathology & Microbiology, Institute of Dental Sciences, Siksha ‘O ’Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India, e-mail: dr.neetamohanty@gmail.com

Online published on 27 March, 2020.

Abstract

Gorlin-Goltz syndrome (GGS) is an autosomal dominant disorder associated with multiple systemic disorders. The incidence rate is approximately 1 in 57000 to 1 in 250000. It has a rare characteristic of multiple odontogenic keratocysts (OKCs), bifid ribs and other abnormalities. Occasionally GGS is associated with malignant lesions like rhabdomyoma, medulloblastoma, leiomyosarcoma, etc. The major and minor criteria based on the clinical and radiological findings are accessed for the diagnosis of GGS. We report a case of an 18 years old female patient, presenting with a swelling in the right side of the face which was diagnosed as GGS by correlating the clinical and histological findings. Advanced imaging tools and techniques were used for analysis. Early diagnosis and treatment have significant importance in reducing the severity of this syndrome.

Keywords

Gorlin-Goltz syndrome, Odontogenic keratocysts, CBCT