神经纤维瘤病(Neurofibromatosis)由Von Recklinghausen于1882年首先报道,因此又称Von Recklinghausen病。本病国外报道较多,近年国内亦有报道。一般认为神经纤维瘤病是一种常染色体显性遗传的全身性疾病,但也有隐性遗传病例的报道。我们曾诊治34例病人,其中12例有家族遗传史,12例做了手术治疗。现将神经纤维瘤病的遗传与治疗总结如下。 临床资料 1975年6月～1991年6月16年中天津医科大学总医院和第二医院共诊治具有眼部表现的神经纤维瘤病患者34例,男21例,女13例。初诊年龄6个月～10岁13例,11岁～20岁12例,21岁～40岁8例,41岁～54岁1例。发病年龄出生～1岁21例,1岁～5岁3例,6岁～ Neurofibromatosis was first reported by Von Recklinghausen in 1882 and is therefore also known as Von Recklinghausen’s disease. More foreign reports of the disease, in recent years, there are also reports. Neurofibromatosis is generally considered an autosomal dominant systemic disease, but there are also reports of recessive genetic cases. We have treated 34 patients, of whom 12 had a family history, 12 patients underwent surgery. The genetic and treatment of neurofibromatosis are summarized below. Clinical data June 1975 ~ June 1991 16 years in Tianjin Medical University General Hospital and the Second Hospital, a total of 34 cases of neurofibromatosis patients with ocular manifestations, 21 males and 13 females. Initial diagnosis of 6 months to 10 years old in 13 cases, 11 years old to 20 years old in 12 cases, 21 years old to 40 years old in 8 cases, 41 years old to 54 years old in 1 case. Age of onset was ~ 1 year old 21 cases, 1 year old to 5 years old in 3 cases, 6 years old