Asian Journal of Research in Surgery

Gorlin-Goltz Syndrome: A Case Report

Rami Ben Arab *

Department of Orthopaedic and Trauma Surgery, Mohamed Taher Al Maamouri Hospital, Nabeul, Tunisia.

Amira Gallas

Department of Orthopaedic and Trauma Surgery, Mohamed Taher Al Maamouri Hospital, Nabeul, Tunisia.

Khaled Khelil

Department of Orthopaedic and Trauma Surgery, Mohamed Taher Al Maamouri Hospital, Nabeul, Tunisia.

Khaled Bchir

Department Legal Medicine, Mohamed Taher Al Maamouri Hospital, Nabeul, Tunisia.

Monia Boussen

Department of Emergency, Mongi Slim Hospital, La Marsa, Tunisia.

Mohamed Ali Sbai

Department of Orthopaedic and Trauma Surgery, Mohamed Taher Al Maamouri Hospital, Nabeul,

*Author to whom correspondence should be addressed.

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Abstract

The Gorlin-Goltz syndrome, also known as Nevoid Basal Cell Carcinoma syndrome, is an uncommon autosomal dominant disorder caused by mutations found on chromosome 9.

This syndrome is characterized by multiple basal cell carcinomas, odontogenic keratocysts and various skeletal abnormalities. This case report presents a 63-year-old man diagnosed with Gorlin-Goltz syndrome by clinical, radiographic and histological findings. The basal cell carcinomas were treated with surgical excision for the biggest ones, and cryotherapy for some of the small ones that are located on the face with a good result.

Through this paper we aim to highlight the importance of an early diagnosis of this syndrome, leading to an early preventive treatment of basal cell carcinomas, a regular and prolonged monitoring of patients and their descendants, with a right genetic advice.

Keywords: Nevoid basal cell carcinoma, Gorlin-Goltz syndrome, odontogenic keratocysts, surgical excision.

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