Visual pathway tumors and hydrocephalus

The authors evaluated the impact of hydrocephalus on the clinical picture o f children with visual pathway tumor (VPT) with or without neurofibromatosi s (NF). Charts of children with VPT treated in the authors' center since 19 85 were retrospectively reviewed, and those with hydrocephalus were selecte d and summarized. Thirty-five children with VPT were found, of whom 20 had NF. Hydrocephalus was found in 4 children with NF (20%) and in 5 without NF (33.3%). In 6 of the children, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis an d the hydrocephalus was shunted at this time. In the other 3 children, all with NF, the hydrocephalus resulted from slowly developing aqueducted steno sis, leading in 2 to severe visual acuity deterioration. The results sugges t that in children with VPT and NF, hydrocephalus, and especially hydroceph alus resulting from aqueductal stenosis, is more frequent than in the gener al population of NF patients, and less frequent than in VPT patients withou t NF. The possibility of the indolent development of hydrocephalus should b e borne in mind while following children with NF. The optic nerve, when alr eady involved with a glioma, is more vulnerable to increased pressure. Thus , in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.