Nasal Glioma and Encephalocele: Diagnosis and Management

Objective: To review the biology of nasal glioma and encephalocele and to present an algorithm for preoperative evaluation and surgical management. Design: Retrospective review and analysis. Setting: Tertiary care medical center: 1970 to 2002. Patient: Sixteen patients with glioma (n = 10) and encephalocele (n = 6). Outcome: Age at the time of presentation, sex, signs and symptoms, imaging findings, surgical approach, pathology, complications, rate of recurrence, and follow-up were recorded. Results: Ten patients presented with nasal glioma with a mean age of 9 months. All patients underwent surgical excision. No complication was encountered with a mean follow-up of 3.5 years. Six patients presented with encephaloceles with a mean age of 15.5 months. All patients underwent surgical excision. Complications included cerebrospinal fluid leak (n = 1) and epiphora (n = 1). Follow-up was 1 to 14 years (mean, 4 years). Conclusion: Nasal glioma and encephalocele are rare, benign, congenital lesions with a potential for intracranial extension. Evaluation should include a complete rhinologic and neurologic examination. Preoperative imaging with a thin-cut axial and coronal computed tomography scan and/or multiplanar magnetic resonance imaging is essential. Surgical intervention should be performed soon after diagnosis to alleviate the increased risk of meningitis. A frontal craniotomy approach is recommended if intracranial extension is identified based on preoperative evaluation, followed by an extracranial resection. If there is no evidence of intracranial extension, a conservative extracranial approach is recommended.