Optic gliomas in children with neurofibromatosis type 1

Robert Listernick*, Joel Charrow, Mark J. Greenwald, Nancy B. Esterly

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

236 Scopus citations

Abstract

To determine the frequency and natural history of tumors of the optic nerves and chlasm in patients with neurofibromatosis type 1, we obtained computed tomographic scans of 65 children who had no known visual or ocular abnormalities before their initial evaluation. Optic gliomas were detected in 10 children (15%). The median age of children with gliomas was 4.3 years (mean 5.8 years, range 9 months to 21 years). Three children (30%) had isolated, unilateral tumors, three (30%) had bilateral tumors, and four (40%) had involvement of the optic chiasm and of one or both nerves. Definite abnormalities of vision were found in only two children (20%). Five additional children were referred to the clinic after evaluation of ophthalmologic complaints led to the diagnosis of neurofibromatosis type 1: three had unilateral exophthalmos and two had plexiform neurofibromas of the eyelid with associated glaucoma. Ipsilateral optic gliomas were found in all five children; one child also had a contralateral tumor. Optic gliomas are commonly identified in young children with neurofibromatosis type 1 who have no ocular or visual abnormalities. Optic nerve gliomas may be associated with plexiform neurofibromas of the eyelid and glaucoma.

Original languageEnglish (US)
Pages (from-to)788-792
Number of pages5
JournalThe Journal of Pediatrics
Volume114
Issue number5
DOIs
StatePublished - Jan 1 1989

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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