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Retinal defect in children with infantile spasms of varying etiologies: An observational study
Michelle T McFarlane1, Tom Wright1, Blathnaid McCoy1
1From the Departments of Ophthalmology and Vision Sciences (M.T.M., C.A.W.) and Neurology (B.M., O.C.S.), The Hospital for Sick Children; Kensington Eye Institute (T.W.), Toronto; and Institute of Medical Science (O.C.S., C.A.W.) and Ophthalmology and Vision Sciences (C.A.W.), University of Toronto, Canada.
Insights
Retinal defects affect nearly 19% of infants with infantile spasms (IS) before vigabatrin treatment. The highest prevalence was observed in infants with perinatal-acquired structural causes of IS.
Area of Science:
- Ophthalmology
- Pediatric Neurology
- Clinical Electrophysiology
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome in infants.
- Vigabatrin is a common treatment for IS, but can cause retinal toxicity.
- The prevalence and etiology-specific risk of retinal defects in IS patients prior to vigabatrin treatment are not well-established.
Purpose of the Study:
- To determine the prevalence of retinal defects in children with infantile spasms (IS) who have not been treated with vigabatrin.
- To investigate if specific primary etiologies of IS are associated with a higher incidence of retinal defects.
Main Methods:
- An observational cohort study of 312 vigabatrin-naive infants diagnosed with IS.
- Electroretinograms (ERGs), specifically the 30-Hz flicker ERG, were used to assess retinal function.
- Primary etiologies for IS were classified into nine subgroups based on patient health records.
Main Results:
- A retinal defect was identified in 18.9% (59/312) of the studied infants.
- The prevalence of retinal defects was highest (24.4%) in the subgroup with perinatal-acquired structural causes, including hypoxic-ischemic defects.
- No significant differences in retinal function were found when comparing across the different etiological subgroups.
Conclusions:
- Retinal defects are present in a significant proportion of infants with IS before vigabatrin initiation.
- Baseline electroretinogram (ERG) testing may be beneficial for children with IS prior to starting vigabatrin therapy to establish a baseline and aid in diagnosing potential retinal toxicity.
Objective:
To determine the prevalence of retinal defect in children with infantile spasms (IS) unrelated to treatment with vigabatrin and clarify if specific primary etiologies for IS are associated with retinal defect more than others.
Methods:
This was an observational cohort study including 312 patients (176 male, 136 female) with IS who were vigabatrin-naive. Participants ranged from 1.7 to 34.7 months of age (mean 8.8 months). Electroretinograms (ERGs) were performed according to the International Society for Clinical Electrophysiology of Vision. Retinal defect was identified as abnormal if the 30-Hz flicker ERG amplitude was lower than the age-corrected normal 95% prediction interval. The primary etiology for IS, as determined by the treating pediatric neurologist(s), was obtained from patient health records and classified into 1 of 9 etiologic subgroups: (1) genetic disorders alone, (2) genetic-structural disorders, (3) structural-congenital, (4) structural-acquired (perinatal), (5) structural-acquired (postnatal), (6) metabolic disorders, (7) immunologic disorders, (8) infectious, and (9) unknown causes.
Results:
Fifty-nine of the 312 vigabatrin-naive children (18.9%) showed retinal defect and the prevalence of retinal defect was highest (24.4%) in the structural-acquired (perinatal) subgroup, which included hypoxic-ischemic defect. Retinal function compared across subgroups showed no significant difference.
Conclusions:
Care is required in diagnosing retinal toxicity, which would be enhanced by baseline flicker ERG in children with IS prior to starting vigabatrin.
