Gliomas of visual pathways and hypothalamus in children--a preliminary report

26 children with gliomas of the visual pathway and hypothalamus were seen during 5 years. 4 of them had intraorbital tumors, in 22 children tumors were localized in the chiasmal/hypothalamic regions. In 25 patients the tumors were surgically explored. All 4 intraorbital gliomas were radically removed using a transcranial approach. Exploration without biopsy was performed 7 times, exploration plus limited biopsy in 3 cases. In the remaining 11 operated children partial or subtotal removal of the tumor was possible. One of the 18 histologically verified tumors was an anaplastic glioma, the others were typical pilocytic astrocytomas. 12 children had hydrocephalus. 7 patients died, 19 are still alive. The operative mortality was 4 out of our 25 operated cases (16%). All of these patients had large tumors with posterior spread into the hypothalamus. In future, surgery in these children should be confined to exploration and limited biopsy followed by radiotherapy. We advocate exploration and biopsy in all chiasma/hypothalamic tumors. The growth potential of these tumors is individually variable. We recommend to follow-up all patients at 3 months' intervals by means of visual evoked potentials, visual acuity, fundoscopy and visual field testing. CT scans and X-rays of the optic canals should be performed once a year. Surgery should only be considered if clinical and/or neuroradiological progression has been documented.