目的探索颅眶神经纤维瘤病致蝶骨翼发育不良的个性化手术治疗策略。方法回顾分析2016年4月至2017年9月收治的8例患者,总结影像学表现并分析手术方法和结果。本组病例的手术治疗方法包括(1)经颅额颞入路;(2)自前颅底向中颅底隧道式分离;(3)颞叶回纳技巧和术中对入眶神经复合体位置的判断。结果 8例患者术后突眼及眼球运动功能改善,搏动感消失,视力检查无明显变化。术后随访7~12个月,均无明显复发。结论颅眶神经纤维瘤病致蝶骨翼发育不良的手术治疗,由于眶上裂增宽及入眶神经血管复合体是否外移等问题,导致并不能完全依赖术前头部模型及导航模拟,但通过全面的术前评估、合理的手术计划、细致的术中操作和个性化的围术期管理,仍能取得良好的疗效。

Objective To explore the individual surgical treatment strategy for cranio-orbital neurofibromatosis(NF)with sphenoid wing dysplasia.Methods A retrospective analysis was made on the 8 cases with cranio-orbital neurofibromatosis from April 2016 to September 2017.The imaging findings and analysis of the surgical methods and results were summarized.The surgical treatment of this group includes:1 transcranial frontal temporal approach;2 from anterior cranial base to middle skull base and "tunnel" separate technique;3 the technique of temporal lobe repositioned and judgment of the position of supraorbital fissure nerve complex during operation.Results Postoperative pulsatile proptosis and ocular movement function were improved in 8 patients and there was no significant change in visual acuity.All patients were followed up for 7 to 12 months,no significant recurrence was observed.Conclusion Surgical treatment of cranio-orbital neurofibromatosis with sphenoid wing dysplasia is a challenge according to widening of the superior orbital fissure and the displacement of supraorbital fissure nerve complex.However,good results can be achieved through comprehensive preoperative evaluation,reasonable surgical planning,meticulous intraoperative operation and individualized perioperative management.