Neurofibromatose tipo 1 e suas manifestações bucomaxilofaciais
Oral and maxillofacial manifestations of neurofibromatosis type 1
Cunha Filho, J.J.; Scortegagna, A.; Martins, F.; Schwengber, M.M.B.; Robinson, W.M.
Rev. odontol. UNESP, vol.37, n3, p.283-289, 2008
Resumo
A neurofibromatose é uma doença hereditária autossômica dominante que afeta, sobretudo, os tecidos moles. O objetivo deste estudo foi revisar aspectos clínicos e radiográficos bucomaxilofaciais da Neurofibromatose tipo 1 (NF1). Foi realizada uma busca por artigos publicados nas bases de dados Pubmed e Lilacs. Estudos com grande poder de gerar evidência não foram encontrados na literatura consultada. Estudos transversais sugeriram que manifestações bucomaxilofaciais da NF1 são mais freqüentes do que anteriormente imaginado. Os sinais clínicos mais relatados envolvem a presença de neurofibromas intrabucais, assimetrias faciais e alargamento das papilas fungiformes da língua. Radiograficamente, os estudos determinaram alargamentos do forame e canal mandibulares, displasias ósseas e neurofibromas intra-ósseos como alterações relacionadas à síndrome.
Palavras-chave
Doença de von Recklinghausen, doença hereditária, neurofibroma, NF1
Abstract
Neurofibromatosis is an autosomal dominant hereditary disease that affects mainly soft tissues. The aim of this study was to review clinical and radiological features of Neurofibromatosis type 1 (NF1) in oral and maxillofacial tissues. A search for scientific articles was performed in Pubmed and Lilacs databases. Studies with an evidence of high power were not found. Cross-sectional studies suggested that oral and maxillofacial manifestations of NF1 are more frequently than previously believed. The most related clinical signs are oral neurofibromas, facial asymmetry and enlarged fungiform papillae of the tongue. Radiographically, studies found enlarged mandibular foramem and canal, osseous dysplasia and intraosseous neurofibromas as manifestations related to NF1.
Keywords
Von Recklinghausen disease, hereditary disease, neurofibroma, NF1
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