关键词: 神经纤维瘤, 神经纤维瘤病, 搏动性突眼, 蝶骨翼发育不良

Abstract: 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.

Key words: Neurofibroma, Neurofibromatosis, Pulsatile proptosis, Sphenoid wing dysplasia