神经纤维瘤病累及眼部的临床表现

Clinical manifestations of ocular involving neurofibromatosis

神经纤维瘤病(neurofibromatosis,NF)是一种以中枢或周围神经系统肿瘤发展为特征的神经皮肤综合征,可累及脑、脊髓、皮肤、骨骼、眼部等器官.NF所致的眼部病变表现多样,既可累及眼睑、眼眶,也可累及晶状体、葡萄膜、视网膜等眼内结构,对于患者的眼部外观和视功能均可产生不同程度的损害,导致上睑下垂、面部畸形、视力下降、视野缺损等,严重影响患者的视觉质量和生活质量.

Neurofibromatosis(NF)is a neurocutaneous syndrome characterized by the development of tumors of the central or peripheral nervous system including the brain,spinal cord,eyes,skin and bones.When the eyes are involved by NF,the clinical manifestations are varied,which can affect not only the eyelids and orbits,but also the intraocular structures such as the lens,uvea,and retina.It can cause varying degrees of damage to the patient’s appearance and visual function,leading to eyelid ptosis,facial deformities,decreased vision,and visual field defects,seriously affecting the patient's visual quality and quality of life.