de Vries J, Freihofer H P, Menovsky T, Cruysberg J R
Department of Neurosurgery, University of Nijmegen, The Netherlands.
J Neurosurg. 1998 Dec;89(6):1032-5. doi: 10.3171/jns.1998.89.6.1032.
A case of surgical repair of progressive exophthalmos of the right eye in a 43-year-old woman with neurofibromatosis Type 1 (NF1) is presented. Preoperatively, the patient's ocular movements and visual fields were intact. Visual acuity was 20/30 on the right side and 20/20 on the left. Computerized tomography scanning demonstrated complete absence of the superolateral orbital wall on the right side with a large meningocele protruding into the right orbit. Intraoperatively, a new superolateral wall was constructed using the inner table of the left frontal bone as a bone transplant. A free galeoperiosteum flap was used for water-tight dural reconstruction. A few weeks postoperatively the patient's exophthalmos showed remarkable resolution. Her ocular movements, visual acuity, and visual fields remained unchanged. In conclusion, reconstruction of the superolateral wall and repair of a meningocele in a patient with NF1 is worthwhile and can be followed by excellent cosmetic results. More important, the patient's visual functions remain preserved.
本文介绍了一例43岁1型神经纤维瘤病（NF1）女性患者右眼进行性眼球突出的手术修复病例。术前，患者的眼球运动和视野正常。右眼视力为20/30，左眼视力为20/20。计算机断层扫描显示右侧眶顶外侧壁完全缺失，有一个大的脑膜膨出突入右侧眼眶。术中，使用左额骨内板作为骨移植构建了一个新的眶顶外侧壁。采用游离帽状腱膜骨膜瓣进行水密性硬脑膜重建。术后几周，患者的眼球突出明显消退。她的眼球运动、视力和视野保持不变。总之，对NF1患者进行眶顶外侧壁重建和脑膜膨出修复是值得的，并且可以获得良好的美容效果。更重要的是，患者的视觉功能得以保留。
