Cranio-orbital-temporal neurofibromatosis with cerebral hemiatrophy presenting as an intraoral mass: a case report.

Department of Oral Diagnosis and Radiology, Faculty of Dentistry, Hacettepe University, Ankara, Turkey.
Journal of the California Dental Association 03/2009; 37(2):119-21.
Source: PubMed


Neurofibromatosis, NF, is a group of genetic disorders that primarily affect the cell growth of neural tissues. Cranio-orbital-temporal neurofibromatosis is an uncommon subtype of neurofibromatosis characterized by neurofibromas, cranial defects, and specific bone lesions. This case report presents the signs of cranial defects in a 24-year-old Caucasian woman with type 1 NF. Mandibular malformations due to NF and dental defects caused by intraoral masses and radiographic images are presented.

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    ABSTRACT: Oral health status is jeopardized in patients with neurofibromatosis (NF) type-1 (NF-1). The aim of the present study was to comprehensively review the oral manifestations in NF-1 patients. PubMed/Medline and Google-Scholar databases were explored using different keywords. Reviews, commentaries, letters to Editor and articles published in languages other than English were excluded. Results: Neurofibromas of oral and perioral soft tissues with subsequent periodontitis, impacted and supernumerary teeth, enlarged alveolar process with dental spacing, morphological changes in teeth and class III molar relationship have been reported in NF-1 patients. Plexiform neurofibromas were reported both in oral soft tissue, maxilla and mandible with evidence of malignant transformation in some cases. Facial skeletal abnormalities, including enlargement of mandibular foramen, increased dimensions of the coronoid and sigmoid notches and notching of the posterior border of the mandible have also been reported. Association between dental caries and NF-1 remains unclear.
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