Clinical Analysis Of Neurofibromatosis Type 1 In Oral And Maxillofacial Regions

Objective To investigate the growth characteristic, clinical manifestations, diagnosis and treatment of neurofibromatosis type 1 (NF1) in oral and maxillofacial regions for the better curative effect.Patients and Methods 17 cases of NF1 from 1984 to 2004 were analyzed retrospectively, among which, 7 underwent total excision, 2 with subtotal excision, 5 with reshaping, 1 with extended excision, 1with incision and drainage, 1 with biopsy.Results Many features of NF1 have the age-dependent nature. Diagnosis coincidence rate was 76.5% (13/17), 3 cases were misdiagnosised, 1 with missed diagnosis. 2 cases recurred once, 1 case recurred twice.Conclusions The choice of treatment methods should be based on the patient's age, anatomical site of involvement, histologic pattern. Surgical excision is indicated to be a main method of the symptomatic therapy. Multidisciplinary approach and integral care should be advocated. Gene therapy is a promising treatment approach for genetic diseases including NF1 in the future.