- 期刊
經篩骨之腦膜腦膨出─病例報告
Transethmoidal Meningoencephalocele – Case Report
摘要
病例係一名2歲6個月大之女童，因左鼻疼痛及持續之水樣鼻漏，被發現有左側鼻內腫瘤，而至本醫院求診。磁振造影顯示左鼻腔內有莖狀腫瘤穿越篩板與腦膜組織相連，鼻內視鏡檢查發現白色柔軟腫瘤自鼻腔頂部垂下於中鼻甲內側，且Furstenberg test為陽性。患者於1995年4月10日接受額顱切開術及鼻內腫瘤切除。手術發現顱底之骨性缺損，並證明腫瘤與大腦額葉確有連結。病理切片顯示以星狀細胞為主的神經膠質細胞散佈於神經細纖維之背景中，故最後診斷為經篩骨型腦膜腦膨出。術後並無腦脊髓液鼻漏、腦膜炎或復發跡象。因本疾病與鼻神經膠質在胚胎學上起源相近，於組織學上亦不易區分，故應參考病史、臨床特徵及影像檢查以達成正確的鑑別判斷，使病患獲得最好的治療與預後。
並列摘要
Differential diagnosis of intranasal masses in children consists mainly of congenital tumors and developmental anomalies. Among the latter, meningoencephalocele, nasal glioma and dermoid cyst should be ruled out before straightforward biopsy to avoid the formidable outcome of CSF rhinorrhea and meningitis. We encountered a 30-month-old female child presenting with left intranasal mass, nasal pain and persisting watery rhinorrhea for 15 months. Telecanthus and flattened nasal ridge were noted while no neurologic abnormality was found. Magnetic resonance image of the brain showed a pedunculated nasal mass connecting with the frontal lobe through the cribriform plate. The mass hung over the left nasal roof and was glistening-white in color and soft to the touch under endoscopic examination. The patient underwent bifrontal craniotomy and intranasal tumor resection. A bony defect measuring about 0.6 cm×0.8 cm in diameter was identified, through which the herniated brain tissue passed to the nasal cavity. After dividing the herniated tissue, the dura was repaired and the bony defect was obliterated. Postoperative course was uneventful with no evidence of recurrence. The tumor was histologically characterized by numerous astrocytic neuroglial cells interspersed among neurofibrillary ground. Some fibrovascular tissue was noted while no mitosis was documented. Though pathologic pictures offered no clues in differentiating meningoencephalocele from nasal glioma, the final diagnosis was made on the basis of clinical history, physical examination, MRI and operative findings, which were discussed elaboratively in the text.