Oral manifestations of neurofibromatosis type 1 in children with facial plexiform neurofibroma: report of three cases

J Clin Pediatr Dent. 2015 Winter;39(2):168-71. doi: 10.17796/jcpd.39.2.972220046g774534.

Abstract

Neurofibromatosis type 1 (NF1) is a common autosomal genetic disorder with a prevalence of 1 in 3,000 births. NF1 is a complex syndrome characterized by many abnormalities and may affect all organ systems. Oral manifestations of NF1 occur frequently, but reports including NF1 children with facial plexiform neurofibromas and oral alterations are scant. Facial plexiform neurofibroma may cause asymmetry, disfigurement and usually arises from the trigeminal nerve. The aim of this paper is to to report three pediatric NF1 cases with facial plexiform neurofibroma presenting with oral manifestations, which were evaluated clinically and radiographically, and also to briefly review the literature. Patients presented with changes in the oral soft tissues, jaws, and teeth ipsilateral to the tumor.

Keywords: Neurofibromatosis 1; child; oral manifestations; plexiform neurofibroma.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Dentofacial Deformities / diagnosis*
  • Facial Neoplasms / diagnosis*
  • Female
  • Gingival Neoplasms / diagnosis
  • Gingival Overgrowth / diagnosis
  • Humans
  • Macroglossia / diagnosis
  • Male
  • Malocclusion / diagnosis
  • Mandible / abnormalities
  • Mandibular Condyle / abnormalities
  • Neurofibroma, Plexiform / diagnosis*
  • Neurofibromatosis 1 / diagnosis*
  • Tongue Neoplasms / diagnosis