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Letters |

Visual Function and Optic Pathway Glioma: A Critical Response—Reply

Cameron F. Parsa, MD
JAMA Ophthalmol. 2013;131(1):120-124. doi:10.1001/2013.jamaophthalmol.519.
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The letter by Gutmann and colleagues provides an opportunity to discuss issues outside the purview of the original report.1

The optic gliomas in question1 (grade I juvenile pilocytic astrocytomas) are indeed congenital in origin and may enlarge postnatally. A handful of early, amalgamated computed tomographic and magnetic resonance imaging studies had commented on initial neuroimaging failing to reveal a glioma that was subsequently detected.2,3 Such initial findings made these early neuroimaging results reportable in their own right. But as the authors of those reports had noted in their discussions, such impressions could simply have been due to the poorer resolution of the computed tomographic and magnetic resonance imaging scanners initially used.2,3 The lack of subsequent reports of supposed de novo tumor emergence in the last 2 decades may be attributed to the greater availability of high-resolution magnetic resonance imaging, which permits detection of the smaller lesions that are present congenitally and enlarge thereafter.

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Figure 1. A mass developing postnatally within already-formed visual axonal pathways may block all axonal transmission and cause considerable visual morbidity (A); a same-sized lesion in utero (B), however, may cause selective pruning and apoptosis of surplus axons and ganglion cells, allowing remaining ganglion cells and axons to permit normal visual function (C).

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Figure 2. A 16-month-old boy without neurofibromatosis with normal visual behavior, growth, and development with a large glioma had only asymmetric nystagmus and trace optic nerve atrophy and no hydrocephalus. Patients without neurofibromatosis type 1 generally present only when symptomatic; such selection bias creates the false impression that non–neurofibromatosis type 1 optic gliomas are more severe than those associated with neurofibromatosis type 1, in which asymptomatic lesions are frequently discovered during routine surveillance scans. R indicates right.




January 1, 2013
David H. Gutmann, MD, PhD; Robert Avery, DO, MSCE; Rosalie E. Ferner, MD; Robert Listernick, MD
JAMA Ophthalmol. 2013;131(1):120-124. doi:10.1001/jamaophthalmol.2013.571.
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