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National Journal of Maxillofacial Surgery
 
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CASE REPORT
Year : 2010  |  Volume : 1  |  Issue : 1  |  Page : 58-62

Gorlin-Goltz syndrome with situs oppositus


Department of Oral and Maxillofacial Surgery, Government Dental College and Research Institute, Bangalore, Karnataka, India

Correspondence Address:
Yadavalli Guruprasad
Department of Oral and Maxillofacial Surgery, AME'S Dental College Hospital and Research Centre, Raichur - 584 103, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-5950.69167

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Gorlin-Goltz syndrome, also known as nevoid basal cell carcicoma syndrome, is caused due to a genetic alteration produced by a mutation in the "Patched" tumor suppressor gene, and it is inherited in a dominant autosomal way, though sporadic cases have been found. This syndrome shows a high penetrance and variable expressiveness. It is a multisystemic process that is characterized by the presence of multiple pigmented basocellular carcinomas, keratocysts in the jaws, palmar and/or plantar pits and calcification of the falx cerebri. Together with these major features, a great number of processes considered as minor features have also been described. The latter include numerous skeletal, dermatology related and neurological anomalies, among others. In some occasions, the presence of very aggressive basocellular carcinomas has been described as well as other malignant neoplasia. Due to the importance of oral maxillofacial manifestations of this syndrome, it is fundamental to know its characteristics in order to make a diagnosis, to provide an early preventive treatment and to establish right genetic advice. We report a rare association of Gorlin-Goltz syndrome with situs oppositus.


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