A Rare Case of Gorlin-Goltz Syndrome in Children

The Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome is an autosomal dominant condition disorder with high variability expression. It presents a series of relevant clinical manifestations that suggest its diagnosis in cutaneous, bone, dental, soft tissue, ner...

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Bibliographic Details
Published in:Case reports in dentistry 2019, Vol.2019 (2019), p.1-5
Main Authors: Junior, Sergio Monteiro Lima, Dutra, Carlos Eduardo Assis, Ribeiro, Bruna Campos, Lima, Luciano Henrique Ferreira, Viana, Ana Paula Cota, Boos Lima, Fernanda Brasil Daura Jorge, Stabile, Glaykon Alex Vitti
Format: Article
Language:English
Subjects:
Basal cell carcinoma
Calcification
Carcinoma
Case Report
Case reports
Cysts
Diagnosis
Genetic disorders
Hereditary diseases
Infants
Maxillofacial
Maxillofacial surgery
Morbidity
Nevus
Patients
Pediatrics
Surgery
Teeth
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Summary:The Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome is an autosomal dominant condition disorder with high variability expression. It presents a series of relevant clinical manifestations that suggest its diagnosis in cutaneous, bone, dental, soft tissue, nervous, and ocular system disorders. This condition requires a great interaction of several specialists to improve the patient’s life. In this case, we presented a 9-year-old male patient referred to the Department of Oral and Maxillofacial Surgery reporting failure in the normal chronology of dental eruption. After evaluation, it was observed that the patient had 13 typical characteristics of the syndrome, including keratocysts, bifid ribs, palmoplantar pits, and 10 other minor characteristics. In conclusion, the expression of so many features of Gorlin-Goltz syndrome is rare in infants, and early diagnosis is important to decrease morbidity and mortality associated with basal cell carcinomas.
ISSN:2090-6447
2090-6455
DOI:10.1155/2019/1608783