• M. Vafiadis
  • G. Exarchakos
  • N. Simaskos

ΧVIIth World Congress of the International Federation of Oto – Rhino – Laryngological Societies (IFOS), 28 September – 3 October 2001, Cairo, Egypt.

Ανακοινώθηκε και δημοσιεύτηκε στα Πρακτικά του Συνεδρίου.

Κλινική εργασία – case report.

Neurofibromatoses are genetic disorders of the nervous system that primarily affect the development and growth of neural cell tissues. The most common is neurofibromatosis type I, previously known as Von Recklinghausen neurofibromatosis, an autosomal dominant hereditary disease. This condition uncommonly affects the oral cavity.

A case is described of a patient who presented for treatment of an enlarged solitary, firm nodule, which had the coloration of the normal oral mucosa occurred at the left buccal site. The lesion caused malocclusion of the jaws disturbing also swallowing and eating. From the rest clinical examination smooth – surfaced, cutaneous lesions scattered over the body and pigmented “café au lait” spots on the trunk were found.

After surgical removal of the nodule, the region healed without recurrence. The histological findings revealed a typical Reclinghausen’s neurofibroma.

Oral manifestations of Recklinghausen neurofibromatosis are rarely showing an incidence ranging from 2 to 7% in most series of different authors. The tongue is the most common site. The floor of the mouth, palate, and maxillary – mandibular bones are more rare localizations of the disease. Reviewing the literature we did not found other cases of the disease manifested from buccal mucosa.