Periapical cemental dysplasia is common in women with NF1

Eur J Med Genet. 2007 Jul-Aug;50(4):274-80. doi: 10.1016/j.ejmg.2007.04.001. Epub 2007 Apr 21.

Abstract

Background: Neurofibromatosis type 1 (NF1) is a genetic disorder with skeletal involvement. Periapical cemental dysplasia is a rare finding in the normal population.

Method: A total of 55 patients with NF1, 29 female and 26 male patients, were evaluated with orthopantomograms, supplemented with periapical radiographs if necessary. The vitality of the teeth was measured by two different testing methods.

Results: A novel finding was the occurrence of cemental dysplasia affecting the periapical area of vital mandibular teeth in 8 adult women with NF1. Thus, cemental dysplasia was detected in 34.8% of the adult female NF1 patients, while cemental dysplasia was not present in men or children with NF1.

Conclusion: Periradicular cemental dysplasia is indeed a new NF1 related bone lesion type. Our finding suggests that this is the first reported sexual dimorphism in the manifestations of NF1. Cemental dysplasia of NF1 patients should not be confused with periapical findings caused by endodontic pathoses. The former do not require active therapy whereas in the latter root canal treatment is necessary.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cementoma / diagnosis
  • Cementoma / etiology*
  • Child
  • Female
  • Humans
  • Male
  • Mandible / diagnostic imaging
  • Middle Aged
  • Neurofibromatosis 1 / complications*
  • Periapical Diseases / diagnosis
  • Periapical Diseases / etiology*
  • Radiography
  • Tooth / diagnostic imaging