神经导航技术外科治疗颅骨纤维异常增生

目的 评价神经导航技术在外科手术治疗颅骨纤维异常增生中的作用和效果,并讨论颅骨纤维异常增生患者的视神经减压手术的适应证。方法 采用经颅视神经管减压手术治疗15例颅骨纤维异常增生导致视力下降的患者,其中男6例,女9例;年龄5～21岁,平均10．3岁。回顾性分析病变部位、症状、体征、CT影像特点、手术方法和结果。将患者视力分为完全丧失、光感、眼前手动、眼前指数和可辨视力表5个级别。提高一个级别或者视力表检查提高0．1为有效,视野缺损的改善也为有效。结果 颅骨纤维异常增生常侵犯额骨、蝶骨体、颧弓、蝶骨大翼和小翼及筛骨,并导致视神经管和眶上、下裂狭窄。主要症状表现为视力下降、突眼和额眶部隆起。手术15例,其中单侧眶上壁、视神经管减压术13例,双侧眶上壁、视神经管减压术2例。全部病例均经病理证实为颅骨纤维异常增生。术后随访3个月至2年1个月,平均11个月,有效为11只(65％),无变化为6只(35％),无视力下降及其他副损伤者。结论 经颅视神经管减压手术是治疗颅骨纤维异常增生引起的视力下降的有效方法,早期手术效果明显,视力低于0．1者提高视力可能性小。但视神经由于压迫移位,用神经导航技术能提高手术的安全性。

Surgical treatment of fibrous dysplasia of the skull with neuro-navagation

OBJECTIVE: To evaluate the role and effect of neuro-navagation in the surgical treatment of fibrous dysplasia of the skull and to discuss the indication and advantages of transcranial optic nerve decompression are discussed. METHODS: Fifteen patients with fibrous dysplasia of skull, 6 males and 9 females, aged 10.3 (5 approximately 21), were surgically treated by transcranial approach. The location of lesion, symptoms and signs, CT characteristics, surgical approaches, and outcomes were analyzed. The postoperative outcomes of visual acuity were grouped into 5 categories: blindness, light perception, hand motion, counting fingers, and recognizing acuity chart. Improvement of visual acuity of one grade or more or increase of 0.1 by acuity chart, and improvement of visual field were defined as effective. RESULTS: Fibrous dysplasia of skull in children most often involved the frontal, sphenoid, and ethmoid bones and resulted in stenosis of optic canal and superior optic fissure. The most common symptoms were decreased vision, proptosis and facial asymmetry. 13 patients underwent decompression of optic canal in lateral side, and two patients in both side. All the cases had pathological diagnosis of Fibrous dysplasia of the skull. After 3-to-25-month follow-up, improvement was found in 11 eyes (65%), and no improvement was found in 6 eyes (35%). Decreased vision was not seen. CONCLUSION: Transcranial optic nerve decompression is an effective treatment for decreased vision induced by Fibrous dysplasia of the skull.