口腔颌面部Ⅰ型神经纤维瘤病临床病例分析  

Clinical Analysis of Neurofibromatosis Type 1 in Oral and Maxillofacial Regions

作　　者：丁志江[1] 韩斐斐[2] 

机构地区：[1]潍坊医学院口腔医学院口腔颌面外科学教研室,山东潍坊261042 [2]潍坊医学院临床学院

出　　处：《潍坊医学院学报》2007年第5期420-422,共3页Acta Academiae Medicinae Weifang

摘　　要：目的探讨Ⅰ型神经纤维瘤病的临床表现、诊断、治疗,旨以提高治疗效果。方法对17例住院病人进行分析,其中采用全切术者9例,改形术5例,扩大切除术1例。结合文献资料进行分析。结果Ⅰ型神经纤维瘤病的临床表现随年龄增长有一定的规律性。诊断符合率为76.5%.结论应根据患者年龄、发病部位、病变大小、病变类型选择合适的治疗方法,目前主要是以手术治疗为主,并提倡多学科协作和整体治疗。提示基因治疗是Ⅰ型神经纤维瘤病的治疗的发展方向。Objective To investigate the growth characteristic ,clinical manifestations, diagnosis and treatment of neurofihrotnatosis type 1 （ NF1 ） in oral and maxillofacial regions for the better curative effect. Methods Seventeen cases of NFI from 1984 to 2004 were analyzed retrospectively,among which,9 cases underwent total excision ,5 eases underwent reshaping, 1 eases underwent expanded resection. And related paper were reviewed. Results Many NFI had the age-dependent nature. Diagnosis coincidence rate was 76.5%. Conclusions The choice of treatment methods sh（m]d be based on the patient＇s age, anatomical site of involvement, histologic pattern. Surgical excision is indicated to be a main method of the symptornatic therapy. Muhidisciplinary approach and integral care should be advocated. Gene therapy is a promising treatment approach for genetic diseases inclnding NF1 in the future.

关 键 词：Ⅰ型神经纤维瘤病 治疗 口腔颌面部 

分 类 号：R730.264[医药卫生—肿瘤]