Gliomas of the optic nerve and chiasm in children*

Abstract

Intrinsic tumours of the optic nerves and chiasm are uncommon tumours, the incidence in children being approximately 3% of all intracranial tumours (Koos & Miller, 1971). Despite extensive discussion in the literature by ophthalmologists, neurologists, neurosurgeons, and neuropathologists, there is still controversy as to the exact nature of the disease and the best method of treatment. Some authors consider that these tumours are potentially aggressive, and advocate surgical excision (Fowler & Matson, 1957) and radiotherapy (Taveras et ai, 1956), while others have considered the tumours to be benign hamartomas which only demand conservative treatment (Hoyt & Baghdassarian, 1969). In all reported series the association of this tumour with von Recklinghausen's disease has been noted, but little comment has been made regarding the natural history of the tumour in patients in whom this association exists. The purpose of this paper is to present the experience of management of this tumour at the Royal Children's Hospital, Melbourne, and in particular, to discuss the significance of this association.