颅眶神经纤维瘤病合并眶内脑膜脑膨出的显微手术治疗  

Microsurgical management of cranio-orbital neurofibromatosis complicated with intraorbital meningoencephalocele

作　　者：李钟铭 李鑫[1] 何心 谢森[1] 张明[1] 王建祯 LI Zhong-ming;LI Xin;He Xin;XIE Sen;ZHANG Ming;WANG Jian-zhen(Department of Neurosurgery,Chinese People's Liberation Army General Hospital,Beijing 100039,China)

机构地区：[1]中国人民解放军总医院神经外科医学部,北京100039

出　　处：《中国临床神经外科杂志》2022年第11期892-894,共3页Chinese Journal of Clinical Neurosurgery

摘　　要：目的探讨颅眶神经纤维瘤病(CON)合并眶内脑膜脑膨出的显微手术方法及疗效。方法回顾性分析2014年1月至2021年10月分期手术治疗的20例CON合并眶内脑膜脑膨出的临床资料。一期手术采用经扩大额颞入路重建颅底蝶骨缺损治疗眶内脑膜脑膨出,3个月后二期手术切除眶内神经纤维瘤。结果眶内神经纤维瘤近全切除16例,次全切除4例。术后视力好转4例,无变化16例。20例突眼均明显缓解,16例眼球运动障碍好转,8例患侧上睑下垂恢复,3例角膜溃疡恢复。无手术死亡,术后无颅内感染、脑脊液漏、癫痫等。术后随访12~36个月,平均21.5月;无脑膜脑膨出复发。结论对CON合并眶内脑膜脑膨出,制定合理的手术计划,尽早修补颅底骨缺损,择期切除眶内神经纤维瘤,是较好的手术方式,治疗效果满意。Objective To explore the treatment strategy of cranio-orbital neurofibromatosis(CON)with intraorbital meningoencephalocele(IME).Methods The clinical data of 20 patients with CON complicated with IME who underwent staging operation from January 2014 to October 2021 were analyzed retrospectively.During the first stage operation,the skull base sphenoid defect was reconstructed through the enlarged frontotemporal approach to treat the IME.The orbital neurofibromas were resected by the second stage operation 3 months later.Results Gross-total resection of orbital neurofibroma was achieved in 16 patients and subtotal resection in 4.Visual acuity was significantly improved in 4 patients and no change in 16.Exophthalmia was significantly relieved in all the patients,eye movement disorder was improved in 16 patients,affected side upper eyelid ptosis was recovered in 8 patients,and corneal ulcer was recovered in 3 patients.There were no surgical death,postoperative intracranial infection,cerebrospinal fluid leakage,and epilepsy.The postoperative follow-up ranged from 12 months to 36 months(mean,21.5 months).No IME recurred.Conclusions For patients with CON complicated with IME,staging operation,repairing the skull base defect during the first stage operation and resection of intraorbital neurofibroma at second stage operation,can achieve good surgical outcomes.

关 键 词：颅眶神经纤维瘤病 脑膜脑膨出 显微手术 

分 类 号：R739.41[医药卫生—肿瘤] R651.11[医药卫生—临床医学]