Head and neck manifestations of neurofibromatosis: A clinical report

Nadeem Ahmad, DDS,(a) Bushra Ahmad, BDS, MFDS RCS,(b) and Hom-Lay Wang, DDS, MSD(a)

(a)Department of Periodontics/Prevention/Geriatrics, School of Dentistry, University of Michigan, Ann Arbor, MI, USA, and (b)Department of Oral and Maxillofacial Surgery, Kent and Canterbury Hospital, Canterbury, United Kingdom.

Neurofibromatosis type 1 (NF1) or von Recklinghausen’s disease is an autosomal dominant set of heritable disorders of ectodermal tissues. This disease affects approximately one in 4,000 births. It causes tumors to grow primarily along nerves, but also on non-nerve-associated tissues resulting in developmental abnormalities. Generally, NF1 is characterized by multiple skin cafe-au-lait pigmentation lesions, as well as enromas. The anatomical compartmentalization in the head and neck region leads to a multitude of variable clinical presentations, such as asymmetrical pendulous masses, when these areas are afflicted with neurofibromas. In this paper, a patient with NF1 will be used to demonstrate the unique intra- and extra-oral manifestations of this condition .
(Int Chin J Dent 2003; 3: 69-75.)

Key Words: developmental abnormalities, neurofibromatosis, von Recklinghausen’s disease.