Von Recklinghausen disease/type I neurofibromatosis and its association with orofacial changes

Literature review and case report

Authors

  • Monah Sampaio Santos Universidade de São Paulo
  • Wladimir Gushiken de-Campos Universidade de São Paulo https://orcid.org/0000-0002-2086-3087
  • Camilla Vieira Esteves Universidade de São Paulo
  • Camila Eduarda Zambon Universidade de São Paulo
  • André Caroli Rocha Universidade de São Paulo
  • Gustavo Grothe Machado Universidade de São Paulo
  • Celso Augusto Lemos Universidade de São Paulo

DOI:

https://doi.org/10.5935/2525-5711.20180023

Keywords:

Neurofibromatosis 1, Maxilla, Mandible, Oral Manifestations.

Abstract

Neurofibromatosis is an autosomal dominant genetic disorder and an inherited condition. This pathology represents 90% of the cases found in the general population. Oral manifestations of neurofibromatosis type I can occur in 70 to 92% of cases, especially when a detailed clinical examination is performed associated with complementary imaging tests. Because of this, it is not uncommon for the dental surgeon to be the first health professional to perform the diagnosis of the disease. The importance of having an early diagnosis correlates with the possible complications of the disease. The carrier may have offspring that manifest the disease in its severe form because of the large capacity for variation, which can be avoided with prior genetic counseling. In this study we report a case of a woman diagnosed and treated for central giant cell lesion 44 years ago, with dental, spine and mandible alterations, also with a family history of neurofibromatosis.

Author Biographies

Monah Sampaio Santos, Universidade de São Paulo

Hospital das Clínicas, Universidade de Sao Paulo, Department of Oral and Maxillofacial Surgery

Wladimir Gushiken de-Campos, Universidade de São Paulo

Faculdade de Odontologia, Universidade de Sao Paulo, Department of Oral Medicine

Camilla Vieira Esteves, Universidade de São Paulo

Faculdade de Odontologia, Universidade de Sao Paulo, Department of Oral Medicine

Camila Eduarda Zambon, Universidade de São Paulo

Hospital das Clínicas, Universidade de Sao Paulo, Department of Oral and Maxillofacial Surgery

André Caroli Rocha, Universidade de São Paulo

Hospital das Clínicas, Universidade de Sao Paulo, Department of Oral and Maxillofacial Surgery

Gustavo Grothe Machado, Universidade de São Paulo

Hospital das Clínicas, Universidade de Sao Paulo, Department of Oral and Maxillofacial Surgery

Celso Augusto Lemos, Universidade de São Paulo

Faculdade de Odontologia, Universidade de São Paulo, Department of Oral Medicine

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Published

2019-01-15

How to Cite

1.
Santos MS, de-Campos WG, Esteves CV, Zambon CE, Rocha AC, Machado GG, et al. Von Recklinghausen disease/type I neurofibromatosis and its association with orofacial changes: Literature review and case report. J Oral Diagn [Internet]. 2019 Jan. 15 [cited 2024 Sep. 19];4:1-6. Available from: https://jordi.com.br/revista/article/view/113

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Section

Case Report