Gorlin-Goltz syndrome– a case report and review of the literature
Journal Title: Forum Ortodontyczne - Year 2014, Vol 10, Issue 2
Abstract
[b]Introduction.[/b] Gorlin-Goltz Syndrome (GGS) is a genetic disease autosomal inherited and dominant with completed penetration. In about 35-40% of cases, it results from a new mutation, hence family history is negative. At the present time there are one hundred clinical signs and symptoms known, most of which are present within craniofacial area. The syndrome is characterized by multiple basal cell carcinomas, odontogenic keratocysts of the jaw, congenital skeletal anomalies, palmar and plantar pits and congenital abnormalities. In view of huge numbers of clinical signs within craniofacial area dentist or orthodontist can be the first one who will recognize above mentioned disease. Symptoms of the syndrome usually manifest themselves in the second decade of life, but in some patients they can be observed in childhood. [b]Aim[/b]. The aim of the study is to present GorlinGoltz Syndrome with special underlining of changes within craniofacial area; based on literature and clinical observations. [b]Case report.[/b] The authors of the study report a case of a fiveyear-old girl who presented typical for Gorlin-Goltz Syndrome signs and features: odontogenic cysts, skeletal abnormalities – polidactylia and facial dysmorphy. Odontogenic cysts and surgical procedures performed in order to remove them caused loss of deciduous and permanent teeth, and consequently malocclusion. The implemented orthodontic treatment in the presented case was to restore occlusion and maintain space for permanent teeth, growth observation and replacement of the teeth. [b]Conclusions[/b]. Common knowledge and awareness among physicians make the early recognition possible and allow to implemet a proper treatment and prophylactic especially important in case of neoplasm. Treating the patient with above mentioned disease demands multidisciplinary approach with special highlighting of orthodoncy to provide the best solutions. (Bałkota B, Radomska A, Tarnawska M. Gorlin-Goltz syndrome - a case report and review of the literature. Orthod Forum 2014; 10: 127-34). Received 6.05.2014 Accepted 10.06.2014
Authors and Affiliations
Barbara Bałkota, Agnieszka Radomska, Maria Tarnawska
Keywords
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