摘要
通过22例3月至63岁多发性神经纤维瘤病(NF)患者的检查发现脸神经纤维瘤12例,眶骨异常4例,虹膜结节20例(91%),先天性青光眼3例4眼,其中3眼房角异常,晶体混浊2例,高度近视7眼,低中度2眼,共占16%,4例听力下降,其中2例排除了听神经瘤。11例分属5个家系,呈常染色体显性遗传。20名患者及家属的 ABO 及 MN 血型显示家系患者中 ABO 系统较MN 系统与 NF 病的关系较为密切。本文对眼改变特别是虹膜结节的性质、形态、数目结合年龄和诊断意义进行讨论,7眼高度近视多与神经纤维瘤同侧 1例伴视罔膜色素变性其意义有待探讨。
Through the analyses of 22 cases of neurofibromatosis (NF) patient ranging from 3 months to 63 yrs.old.We found that 4 cases had bi- lateral hearing loss and 2 of them were excluded from acoustic neuroma.The ocular appearance show-ed lid neurofibroma in 12 cases,orbital bone ab-normalities in4 cases,iris nodules in 20 cases (90%),congenital glaucoma (4eyes) in 3 cases which show-ed abnormal gonioscopy in 3 eyes,lens opacity in
2 cases,mild and moderate myopia in 8 eyes,and high myopia in 7 eyes,occupying 16% of the total.11 cases belong to 5 pedigrees showing autosomal dominant inheritance.The ABO and MN blood gro-uping of the NF Cases and families in the pedigree showed that NF was more related to ABO than MN blood group.The ocular appearance,especially the
shape and number of iris nodules in relation to age and diagnosis is discussed.The significance of 1 case of refinitis pigmentosa,7 cases of high myopia in relation with NF should be further studied.
出处
《眼科学报》
1989年第3期113-117,共5页
Eye Science
