|本期目录/Table of Contents|

[1]陈贵敏,许建华,查 玮,等.头颈部Ⅰ型神经纤维瘤病23例临床分析[J].医学研究与战创伤救治(原医学研究生学报),2009,11(02):118-120.
　CHEN Gui-min,XU Jian-hua,CHA Wei,et al.Neurofibromatosis type I of the head and neck: clinical analysis of 23 cases[J].JOURNAL OF MEDICALRESEARCH —COMBAT TRAUMA CARE,2009,11(02):118-120.
点击复制

头颈部Ⅰ型神经纤维瘤病23例临床分析()
分享到62.8K

《医学研究与战创伤救治》（原医学研究生学报）[ISSN:1672-271X/CN:32-1713/R]

卷:
第11卷
期数:
2009年02期
页码:
118-120
栏目:
出版日期:
2009-03-20

文章信息/Info

Title:
Neurofibromatosis type I of the head and neck: clinical analysis of 23 cases
文章编号:
1672-271X(2009)02-0118-03
作者:
陈贵敏1; 许建华1; 查 玮2; 赵云富2
1.南京军区福州总医院口腔科，福建福州 350025；2.第二军医大学附属长征医院口腔科，上海 200003
Author(s):
CHEN Gui-min1; XU Jian-hua1; CHA Wei2; ZHAO Yun-fu2
1.Department of Stomatology,Fuzhou General Hospital of Nanjing Military Command, Fuzhou 350025,Fujian,China;2.Department of Stomatology,Changzheng Hospital,Second Military Medical University,Shanghai 200003,China
关键词:
Ⅰ型神经纤维瘤病; 头颈部; 诊断; 治疗
Keywords:
Neurofibromatosis type I;  Head and neck region; Diagnosis; Treatment
分类号:
R739.91
DOI:
-
文献标志码:
A
摘要:
目的 探讨头颈部I型神经纤维瘤病(NF-I)的临床特点、诊断及治疗。方法 对两个医院近20年来收治的23例头颈部NF-I患者的临床资料进行分析。结果 23例患者中位年龄21岁，男女之比3∶4。颈部12例、口腔内5例、颧部2例、颞部1例、睑额顶部1例、翼腭凹及下颌骨各1例；除了牛奶咖啡斑及皮肤赘瘤两大特征外，1例有枕骨缺损、1例有颈椎后凸畸形，1例瘤体自发性大出血死亡，1例恶变，2例合并血管外皮肉瘤、2例有颅内占位病变、1例伴有垂体瘤且智力低下。17例有详细家族史记载的5例有遗传史，其中1例父亲及同胞4人均有不同程度的临床表现。结论 NF-I是一种常染色体显性遗传性疾病，病程长，青春期为主要就诊年龄段。临床表现形式多样，恶变及自发性大出血应引起重视。手术仍为主要治疗手段，减少术中出血及提高修复水平是保证手术质量的关键。
Abstract:
Objective To study the clinical characteristic, diagnosis and surgical procedures of neurofibromatosis type I (NF-I) in the head and neck.Methods The clinical data of 23 cases of NF-I in the head and neck were collected and analyzed.Results The median age of 23 cases was 21 year-old, and the ratio of male to female in this group was 3/4. 12 tumors were located in cervicum, 5 in oral cavity, 3 in face and 1 each in eyelid-forehead, mandibular bone and pterygopalatina fossa.In addition to milk coffee maculae and skin neoplasm, the complications of occipital bone defect(1), back forward prominence abnormity in cervical vertebrae(1), canceration(1), angio-crust sarcoma(1), intracranial occupying lesion(2), hypophysoma with mental retardation(1) and a death case of spontaneous hemorrhea resulting from cervical part tumor break were found. 5 of 17 cases had detail records of family hereditary history. 1 case of which had different degree of clinical manifestation in his father and 4 brothers.Conclusion NF-I is an autosome dominant heredity disease, which has long course and the main visit age is puberty. The clinical manifestation of NF-I is variety. We should think highly of canceration and spontaneous hemorrhea of this disease. The main treatment is surgical procedure. The premise of guarantee the surgical treatment quality is decreasing hemorrhage during operation and elevating repair technique.

参考文献/References:

[1]陈贵敏，黄恒平.保留真皮下血管网皮肤游离移植的临床观察[J]. 实用口腔医学杂志，1991，7(3):178-179.
[2]毛天球.颌面外科手术与技巧[M].北京:人民军医出版社，2005:149.
[3]王一兵，曹永倩，王法刚.Ⅰ型神经纤维瘤病七例报告[J].中华肿瘤防治杂志,2006,13(12):943-944.
[4]牛艳坤，王 刚，陈卫国，等.Ⅰ型神经纤维瘤病的X线和MRI诊断(附15例报告)[J].南方医科大学学报,2006,26(6):849-850.
[5]毛小明，史大鹏，刘秋明，等.神经纤维瘤病型累及脊柱脊髓的MR表现[J].实用诊断与治疗杂志,2006,20(8):573-574.
[6]Wise JB, Patel SG, Shah JP. Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type Ⅰ[J]. Head Neck,2002,24(2):207-211.
[7]郭金才，王玉荣.神经纤维瘤病I型的整形外科治疗[J].华中医学杂志，2004，28(1):51-52.
[8]王 炜，林晓曦，祁佐良，等.面部神经纤维瘤的整形和显微外科治疗[J]. 上海医学，2000，23(7):387-389.

相似文献/References:

备注/Memo

备注/Memo:
-
更新日期/Last Update: 2013-11-20