The reduction of plexiform neurofibroma in the craniofacial and neck areas

摘要: Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disease of high penetrance and variable expression. Epidemiologic data on craniofacial manifestations are still lacking. Up until now 74 patients with NF1 have been treated at the Department Oral Maxillofacial Surgery University Hamburg. Forty-two presented periorbital orbital neurofibromas varying in extension severity findings affected site. Surgical therapy mainly based tumour reduction, frequently combined face-lifting. In our experience neck tend to be pseudo-encapsulated, facilitating preparation tumour. On other hand, identification diffuse infiltrating trigeminal nerve region difficult local recurrence must expected.