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Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children
J Raymond Buncic1, Carol A Westall, Carole M Panton
1Department of Ophthalmology, The Hospital for Sick Children, Toronto, ON, Canada.
Insights
Vigabatrin, an anticonvulsant, can cause peripheral retinal atrophy and optic nerve damage in children. Early detection through retinal light reflexes and electroretinography is crucial for prompt treatment.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Vigabatrin is an anticonvulsant medication used to treat seizures in children.
- Potential ocular toxicity associated with vigabatrin treatment requires thorough investigation.
Observation:
- A case series involving 138 pediatric patients evaluated for vigabatrin toxicity.
- Clinical and electroretinographic (ERG) assessments were performed every six months.
Findings:
- Three children exhibited peripheral retinal nerve fiber layer atrophy and nasal optic nerve atrophy.
- Electroretinography revealed progressive decreases in retinal function, particularly the 30-Hz flicker response.
- Macular wrinkling was noted in one case, suggesting potential early macular toxicity.
Implications:
- Vigabatrin can induce a characteristic pattern of peripheral retinal and optic nerve atrophy in children.
- Clinical recognition is aided by observing superficial retinal light reflexes.
- Discontinuation of vigabatrin should be considered due to observed retinal dysfunction.
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.
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