Optic Nerve Glioma (Sporadic): A Baby with Monocular Nystagmus

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The parents of a 17-month-old girl reported that their daughter had developed shimmering horizontal movements of the left eye at about 9 months of age. Emergency pediatric evaluation suggested possible spasmus nutans. A head tilt developed one month later at 10 months of age. There was no head nodding. An ophthalmologic examination was requested. A neuro-ophthalmologic evaluation at 17 months of age showed a normal qualitative vision assessment in each eye with normal versions and no strabismus. She had periodic random appearing shimmering movements of the left eye. The pupils were equal, reactive, with no relative afferent pupillary defect. Visual fields were grossly full to toy movement. Moderate bilateral disc pallor was evident with direct ophthalmoscopy. Magnetic resonance imaging (MRI) of the brain demonstrated a chiasmal region solid mass (Fig. 15.1). There were no stigmata of neurofibromatosis type 1 (NF-1). Genetic testing revealed no mutations associated with NF-1. Three-month follow-up MRI and ophthalmologic examinations were scheduled. After 3 months, there was increased size of the mass on MRI with no change in qualitative visual acuity. A biopsy was performed that confirmed the diagnosis of a pilocytic astrocytoma, WHO grade I. A 15-month course of carboplatin with vincristine was initiated and completed.

Original languageEnglish (US)
Title of host publicationFundamentals of Pediatric Neuro-Ophthalmology
Subtitle of host publicationa Practical, Case-Based Approach to Diagnosis and Management
PublisherSpringer International Publishing
Pages83-85
Number of pages3
ISBN (Electronic)9783031161476
ISBN (Print)9783031161469
DOIs
StatePublished - Jan 1 2023

Keywords

  • Carboplatin
  • Neurofibromatosis
  • Optic nerve glioma
  • Pilocytic astrocytoma
  • Retinal nerve fiber layer
  • Selumetinib
  • Vincristine
  • Vision loss

ASJC Scopus subject areas

  • General Medicine

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