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2020, Number 1

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Rev Cubana Estomatol 2020; 57 (1)

Early diagnosis and treatment of Gorlin-Goltz syndrome: eightyear follow-up

Silva QCL, Farias HS, Varela CA, Gonçalves FJ, Andrade CJ

Language: Portugués
References: 15
Page:
PDF size: 214.92 Kb.


ABSTRACT

Introduction: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder of high level penetrance and variable expressiveness. In 1960 Gorlin and Goltz listed the disease cases previously reported in the literature, concluding that it was a triad syndrome (basal cell carcinomas, multiple odontogenic keratocysts and skeletal anomalies). It is now known that a broad spectrum of other systemic manifestations may be present, such as neurological, ophthalmic, genital, cardiovascular and endocrine.
Objective: Describe a clinical case of Gorlin-Goltz syndrome and its eight-year follow-up, and highlight the importance of the dentist in the early diagnosis and treatment of the syndrome.
Case report: A 10-year-old male patient attended the Oral and Maxillofacial Surgery Service of Santa Casa de Misericordia Hospital in Sao Felix, Bahia, Brazil, in February 2004, accompanied by his grandmother, who reported that her grandson's teeth "were crooked". Physical examination revealed an increase in the volume of the right side of the face, hypertelorism, broad nasal base, frontal bossing, mild mandibular prognathism and shortened toes, whereas oral examination found ill-positioned teeth and midline deviation. The patient was followed up for eight years, and during this time imaging tests were performed which showed large areas of recurrent radiolucent lesions. Diagnosis of odontogenic keratocyst was verified by histopathological examination, confirming the diagnostic hypothesis of Gorlin-Goltz syndrome. The patient was referred for genetic evaluation and is currently being followed up at the State University of Feira de Santana, Bahia.
Conclusions: Multidisciplinary long-term follow-up is essential in cases of this syndrome to improve the quality of life of patients.


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