Khairallah M, Messaoud R, Ladjimi A, Hmidi K, Chaouch K
Service d'Ophtalmologie, Hôpital Universitaire Fattouma Bourguiba, Monastir, Tunisie.
J Fr Ophtalmol. 1999 Nov;22(9):975-8.
We report a case of plexiform neurofibroma of the upper eyelid associated with spheno-orbital dysplasia in a 18-year-old woman with von Recklinghausen neurofibromatosis. Visual acuity was 20/40 in the right eye and 20/20 in the left. Plexiform neurofibroma involving the right upper eyelid was associated with mild ptosis and ipsilateral facial hypertrophy. Biomicroscopic examination showed lisch nodules. Funduscopic examination, visual field and neurologic examinations were normal. Café au lait spots involved the trunk with neck plexiform neurofibroma. Computed tomography disclosed spheno-orbital dysplasia. The patient's status remained unchanged at 6 months follow-up. Cranial features of von Recklinghausen neurofibromatosis are found in 3 to 7% of patients. In patients with plexiform neurofibroma of the eyelid the ophthalmologist should look for associated spheno-orbital dysplasia.
我们报告一例18岁患有冯雷克林霍增氏神经纤维瘤病的女性，其右上睑丛状神经纤维瘤合并蝶眶发育异常。右眼视力为20/40，左眼视力为20/20。累及右上睑的丛状神经纤维瘤伴有轻度上睑下垂和同侧面部肥大。生物显微镜检查发现了Lisch结节。眼底检查、视野检查和神经系统检查均正常。躯干有咖啡牛奶斑伴颈部丛状神经纤维瘤。计算机断层扫描显示蝶眶发育异常。随访6个月时患者状况未变。3%至7%的患者有冯雷克林霍增氏神经纤维瘤病的颅骨特征。对于患有眼睑丛状神经纤维瘤的患者，眼科医生应留意是否存在相关的蝶眶发育异常。
