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(Investigative Ophthalmology and Visual Science. 2007;48:2895-2902.)
© 2007 by The Association for Research in Vision and Ophthalmology, Inc.
DOI:  10.1167/iovs.06-0429
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Screening and Diagnosis of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1 by Using Sweep Visual Evoked Potentials

Benjamin C. M. Chang,1,2 Giuseppe Mirabella,1 Ronit Yagev,1,3 Michael Banh,1 Eedy Mezer,1 Patricia C. Parkin,4 Carol A. Westall,1 and J. Raymond Buncic1

1From the Department of Ophthalmology and Vision Sciences and the 4Division of Pediatric Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada; the 2Department of Ophthalmology and Visual Sciences, Alexandra Hospital, Singapore; and the 3Department of Ophthalmology, Soroka University Medical Center, Beer-Sheva, Israel.

PURPOSE. Neurofibromatosis type 1 (NF-1) is an autosomal dominant phakomatosis with a prevalence of 1 in 2000 to 1 in 5000. Up to 24% of these patients have optic pathway gliomas (OPGs). In the present study, the use of sweep visual evoked potentials (SVEPs) was investigated as a screening tool for identifying patients with NF-1 who had OPGs by comparing them to those patients with no OPGs and to normally developing children.

METHODS. Contrast sensitivity and grating acuity were measured with the SVEP. Sixteen children with OPGs (OPG group), 14 children with NF-1 without OPGs (nOPG), and 16 aged-matched control subjects were recruited. All participants had best-corrected visual acuity of 6/9 or better. All were tested monocularly.

RESULTS. Comparisons between groups by using the Tukey B test showed a significant reduction of mean log contrast sensitivity in the OPG group (1.55) compared with the nOPG (1.9, P = 0.006) and control (2.10, P < 0.001) group. There was no significant difference between the nOPG and control groups (P = 0.195). Grating acuity was comparable between groups, and no statistically significant differences were found. Log contrast sensitivity was moderately sensitive in identifying patients with OPG and was highly specific in screening out patients with no OPG.

CONCLUSIONS. Children with OPGs have reduced contrast sensitivity when assessed using the SVEP. Children with no OPGs display no differences in visual functioning compared with control subjects. The findings suggest that the SVEP can be a useful and noninvasive screening tool for early detection of visual pathway gliomas in children with NF-1 and normal visual acuity.






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Copyright © 2007 by the Association for Research in Vision and Ophthalmology