A case of nasal glioma and neurofibromatosis A new association

Abstract

A man of 25 with von Recklinghausen's neurofibromatosis (VR) developed nasal polyps. Subarachnoid haemorrhage occurred shortly after surgical excision and he died 9 weeks later. Post-mortem examination showed chronic hydrocephalus due to aqueduct stenosis. Histologically the polyps were a nasal glioma, a forme fruste of anterior encephalocoele not previously associated with VR, though other cranial defects are well recognized. Nasal glioma should be considered in the differential diagnosis of nasal lesions in VR.