先天性鼻内脑膜脑膨出合并异位神经胶质瘤（附1例报道并文献复习）

 目的探讨先天性鼻部脑膜脑膨出合并鼻外神经胶质瘤的诊断和治疗。方法收集上海交通大学医学院附属第九人民医院收治的1例鼻内脑膜脑膨出合并鼻外神经胶质瘤患儿，并结合文献进行复习。入院后行鼻腔鼻窦CT扫描及头颅MRI，根据影像学检查结果制定手术方案，在全麻下行鼻内镜下鼻腔肿物切除术及颅底骨质缺损修补术联合鼻外径路外鼻肿物切除术，术后予应用透过血脑屏障的抗炎治疗。结果鼻根部及鼻腔肿块一次性切除，愈合良好，无脑脊液鼻漏、脑膜炎、鼻中隔穿孔、视力障碍及癫痫等并发症，随访1年无复发。术后病检提示鼻根部及鼻腔内送检肿物见神经胶质细胞，考虑为异位神经胶质瘤和脑膜脑膨出。结论对于婴幼儿先天性鼻部脑膜脑膨出，鼻腔鼻窦CT及头颅增强MRI是重要的诊断依据，根据病变部位制定不同的手术的方式。鼻内镜具有视野清晰、损伤小、出血少、并发症少的优点。颅底骨质缺损修补的关键是根据骨缺损面积，予肌肉、筋膜、软骨瓣或骨片填塞漏口。

Congenital intranasal meningoencephalocele with concurrent neuroglial heterotopias (one case report with literature review)

 ObjectiveTo explore the diagnosis and surgical treatment of congenital intranasal meningoencephalocele with concurrent ectorhinal glioma. MethodsClinical data of one child suffering from intranasal meningoencephalocele with concurrent ectorhinal glioma surgically treated in our department were collected and analyzed in combination with literature review. Imageological examinations including sinus CT scanning and cranial MRI were given to the child and surgical program was made according to the results. Under general anesthesia, the child received intranasal tumor resection and anterior skull defect repair endoscopically combined with ectorhinal tumor resection via an extranasal approach. Blood brain barrier permeable antibiotic was used postoperatively to prevent infection. ResultsThe masses located in both the nasal root and nasal cavity were removed simultaneously, and the child got cured without complications such as cerebrospinal fluid leakage, meningitis, septum perforation, visual impairment and epilepsy. One year postoperative follow up showed no tumor recurrence. The postoperative pathology revealed glial cells in masses from both nasal root and nasal cavity, which confirmed the diagnoses of heterotopic glioma and meningoencephalocele. ConclusionSinus CT and enhanced cranial MRI are important for the diagnosis of congenital nasal meningoencephalocele in infants. Proper surgical approach should be adopted according to the position of the neoplasm. Endoscopic neoplasm resection has the advantages of clear vision, small damage, less bleeding and fewer complications. The key to repair the skull base defect is the choice of suitable materials, such as muscle, fascia, cartilage flap or bone sheet, to fill the leak according to the defect size.