Neurofibromatosis Type 1 with Unusual Oral Manifestations

Abstract

ABSTRACT Neurofibromatosis (NF) is a genetically transmitted autosomaldominant disorder with variable penetrance and about 50% ofcases representing new mutations. It is progressive in natureand one of its unique feature is the diversity of clinical expressionfrom one patient to another and even within a family. The diseaseis often characterized by complex and multicellularneurofibroma. It may also lead to different complicationsthroughout the life of an affected individual. We report a case ofNF involving alveolus of maxilla and mandible causing expansionof the buccal and lingual cortical plates in a 10-year-old girl. Keywords: Neurofibromatosis, Mandible, Hypoplastic, Cafe aulait spots. How to cite this article: Kini R, Naik V, Baliga A.Neurofibromatosis Type 1 with Unusual Oral Manifestations.J Indian Aca Oral Med Radiol 2012;24(3):229-232. Source of support: Nil Conflict of interest: None declared INTRODUCTION Neurofibroma is the most common type of peripheral nerveneoplasm, arising from Schwann cells, perineural fibroblastsor both. The term neurofibroma was coined by vonRecklinghausen in 1881.