目的探讨Ⅰ型神经纤维瘤病的临床表现、诊断、治疗，为临床积累经验，提高治疗效果。
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.
方法回顾性分析1981 ~ 2005年我科收治的46例周围型神经纤维瘤病的临床资料。
Methods Retrospective analysis of the clinical date was carried out in 46 cases of peripheral neurofibromatosis admitted in our department from 1981 to 2005.
结论 四周型神经纤维瘤病采用整形外科方法切除及修复创面具有切除较彻底、修复塑形好的优点。
Conclusion Plastic surgical treatment of peripheral neurofibromatosis can yield good results with complete excision and better reconstruction.
目的检测中国人神经纤维瘤病1型(nf1)基因32、33外显子突变。
Objective to detect the mutation on exons 32, 33 of Chinese the neurofibromatosis type 1 (NF1) gene.
结果：神经纤维瘤病1型4例，表现为蝶骨大翼缺损2例，其中1例合并双侧桥小脑结合臂胶质瘤，1例合并眶内丛状神经纤维瘤；
Results:Among the4cases of NF1,2had defect of the great wings of the sphenoid, bilateral middle cerebellar peduncle gliomas and plexiform neurofibroma respectively.
蝶骨翼发育不良可以是一个孤立性的发现，也可以见于神经纤维瘤病I型患者。
Sphenoid wing dysplasia can occur as an isolated finding or in patients who have Neurofibromatosis type 1. Approximately 50% of cases are associated with neurofibromatosis type 1.
蝶骨翼发育不良可以是一个孤立性的发现，也可以见于神经纤维瘤病I型患者。
Sphenoid wing dysplasia can occur as an isolated finding or in patients who have Neurofibromatosis type 1. Approximately 50% of cases are associated with neurofibromatosis type 1.
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