Elsevier

Ophthalmology

Volume 111, Issue 10, October 2004, Pages 1935-1942
Ophthalmology

Original article
Characteristic retinal atrophy with secondary “inverse” optic atrophy identifies vigabatrin toxicity in children

Presented at: Neuro-ophthalmology Society of Australia meeting, April 21, 2002; Sydney, Australia, and Canadian Ophthalmological Society meeting, June 26, 2003; Ottawa, Canada.
https://doi.org/10.1016/j.ophtha.2004.03.036Get rights and content

Abstract

Objective

To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin.

Design

An interventional case series report.

Participants

One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto.

Method

Sequential clinical and electroretinographic (International Society for Clinical Electrophysiology of Vision standards) evaluations every 6 months.

Main outcome measures

Presence of recognizable retinal and optic atrophy in the presence of abnormal electroretinogram (ERG) and other clinical findings.

Results

Three children being treated for seizures with vigabatrin showed definite clinical findings of peripheral retinal nerve fiber layer atrophy, with relative sparing of the central or macular portion of the retina and relative nasal optic nerve atrophic changes. Some macular wrinkling was evident in 1 case. Progressive ERG changes showing decreased responses, especially the 30-Hz flicker response, supported the presence of decreased retinal function.

Conclusions

A recognizable and characteristic form of peripheral retinal atrophy and nasal or “inverse” optic disc atrophy can occur in a small number of children being treated with vigabatrin. The changes in superficial light reflexes of the retina in children facilitate the clinical recognition of nerve fiber layer atrophy. The macula is relatively spared, although superficial retinal light reflexes indicating wrinkling of the innermost retina suggest early macular toxicity as well. Because these changes are accompanied by electrophysiologic evidence of retinal dysfunction, discontinuation of vigabatrin should be strongly considered.

Section snippets

Participants and methods

To monitor possible vigabatrin visual toxicity, the current practice at The Hospital for Sick Children (HSC) is to assess each child with seizure activity before and during drug treatment with neuro-ophthalmologic examination and with an ERG (under sedation in most cases). Electroretinograms are recorded according to the standards of The International Society for Clinical Electrophysiology of Vision (ISCEV).19 Because ERGs in young children who are still developing, ERGs are compared with

Results

During this protocol, 3 cases of a specific, identifiable, vigabatrin-associated peripheral retinal atrophy with secondary optic nerve atrophy in children have been identified and selected for this communication. The authors report the characteristic pattern of atrophy that is found in association with the peripheral field loss caused by this drug in young children. In 1 case, ERGs were recorded sequentially, and results show dramatic parallel deterioration. This clinically characteristic

Discussion

Three children, aged 12 years, 15 years, and 212 years are described, in whom a severe and atypical form of retinal and optic atrophy has been recognized in association with extended use of the anticonvulsant vigabatrin, peripheral field loss (presumed or clinically detectable), and ERG abnormalities. Because clinical assessment of peripheral visual loss, especially in its milder forms, is so difficult and unreliable in young children, clinicians have turned to investigational support of

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  • Cited by (0)

    Manuscript no. 230580.

    Technical support for electroretinogram and visual evoked potential measures was partially funded by The Hospital for Sick Children Research Institute, Toronto, Canada (seed grant), and the University of Toronto Vision Science Research Program, Toronto, Canada.

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