두경부에 발생한 광범위한 신경 섬유종증의 시험례

Treatment of Extensive Neurofibromatosis on the Head and Neck

Neurofibromatosis is an autosomal deminant neurocutaneous systemic disease characterized by multiple Cafe-au-lait spots. generalized cutaneous neurofibromatosis. central nervous system tumors. skeletal defects and a myriad of somatic and endocrinologic abnormalities. It occurs in about 1:3000 live births and both sexes are equally affected. From 1989 to 1992, 21 patients who admitted to our department were evaluated in clinical aspects. Then we conclude as follows: 1) The onset was usually before 10 years of age. and the incidence between the sexes was approximately the same. 2) The familial tendency was approximately 48%. 3) The head and neck were the usual sites of the disease. 4) Bone changes occurred in about 24%. 5) Most of the tumor could be removed through early diagnosis to prevent recurrence. bone changes and malignant changes. And at the same time, trough reconstructive surgery, results both functionally as well as aesthetically satisfactory could be achieved.