Does vigabatrin treatment for infantile epileptic spasms syndrome cause visual impairment in infants and young children??
Date First Published:
May 23, 2026
Last Updated:
May 23, 2026
Report by:
Aileen O' Mahony, SHO (CHI Temple Street)
Search checked by:
Dr Kathleen Gorman, Consultant Paediatric Neurologist
Three-Part Question:
In infants and young children with infantile spasms does treatment with vigabatrin compared with other antiseizure medications or no vigabatrin exposure result in an increased risk of visual field loss or visual dysfunction?
Clinical Scenario:
An 8-month-old infant is diagnosed with infantile epileptic spasms syndrome, confirmed by video EEG showing hypsarrhythmia. Following diagnosis, oral prednisolone and vigabatrin were prescribed as per UKISS guidelines. (1) However, parents are concerned regarding potential visual field constriction and retinal toxicity associated with vigabatrin use in young children.
Search Strategy:
MEDLINE and Embase were searched using the following search terms from 1997 to 2025:
(vigabatrin AND vision OR visual OR optic AND spasms OR epilepsy)
And searched with:
("Infantile Spasms"[Mesh] OR "West Syndrome" OR "infantile spasm*" OR "west syndrome")
AND
("Vigabatrin"[Mesh] OR vigabatrin OR Sabril)
AND
("Vision Disorders"[Mesh] OR "Visual field defects" OR "Blindness"[Mesh] OR "Visual impairment" OR "Retinal toxicity" OR "Retinopathy" OR "Optic atrophy" OR "visual field loss" OR "peripheral vision loss" OR "ocular toxicity")
Secondary sources:
The Cochrane Library was searched using a combination of the terms as outlined for primary sources.
Citation tracking was used on applicable papers to ensure no relevant literature was missed.
(vigabatrin AND vision OR visual OR optic AND spasms OR epilepsy)
And searched with:
("Infantile Spasms"[Mesh] OR "West Syndrome" OR "infantile spasm*" OR "west syndrome")
AND
("Vigabatrin"[Mesh] OR vigabatrin OR Sabril)
AND
("Vision Disorders"[Mesh] OR "Visual field defects" OR "Blindness"[Mesh] OR "Visual impairment" OR "Retinal toxicity" OR "Retinopathy" OR "Optic atrophy" OR "visual field loss" OR "peripheral vision loss" OR "ocular toxicity")
Secondary sources:
The Cochrane Library was searched using a combination of the terms as outlined for primary sources.
Citation tracking was used on applicable papers to ensure no relevant literature was missed.
Search Details:
Secondary sources:
The Cochrane Library was searched using a combination of the terms as outlined for primary sources.
Citation tracking was used on applicable papers to ensure no relevant literature was missed.
The Cochrane Library was searched using a combination of the terms as outlined for primary sources.
Citation tracking was used on applicable papers to ensure no relevant literature was missed.
Outcome:
212 papers were found with OVID and 84 from EMBASE = 296 papers. 43 duplicates were removed. The titles were screened for applicability and 210 were removed. 43 remaining papers were reviewed and 8 abstracts, narrative reviews and case reports were removed. 35 papers remained with 2 additional papers added from citation tracking. The content of the papers was fully reviewed for relevance and 7 papers remained.
Relevant Paper(s):
| Study Title | Patient Group | Study type (level of evidence) | Outcomes | Key results | Study Weaknesses |
|---|---|---|---|---|---|
| Visual field defects after vigabatrin treatment during infancy: retrospective population-based study. Jonsson, H., Lehto, M., Vanhatalo, S., Gaily, E., & Linnankivi, T. 2022 Finland | 26 prescribed vigabatrin for Infantile Epileptic Spasms Syndrome in the first year of life. Examined between age of 11-19 years | Duration: 1997 - 2008. Method of visual assessment: Visual field testing. Retrospective population based study. | Developed VAVFD: 5/26 (19%) | Associations: | Limitations: Patients with cortical visual impairment or developmental delay were not examined. Selection bias. 88 treated with VBN. Only 28 available (30% exposed) could complete perimetry – potentially underestimation of true VAVFD. No baseline perimetry or OCT – cannot exclude pre-existing disease. Potential confounding from underlying disease |
| Risk increased with longer treatment duration (31% of infants treated for >12 mths). | |||||
| Duration of treatment: Developed VAVFD | No significant correlation between cumulative dose & VAVFD. | ||||
| < 6 mths: 0/6 (0%) | 3/5 patients affected, constriction was mild and allowed to drive within EU regulations. | ||||
| 6-12 mths: 1/7 (14%) | |||||
| > 12 mths: 4/13 (31%) | Strengths: | ||||
| Use of formal perimetry with correlation on OCT | |||||
| Long term follow-up | |||||
| Efficacy and safety of vigabatrin in Japanese patients with infantile spasms: Primary short-term study and extension study. Ohtsuka, Y. 2018 Japan | 13 children with infantile epileptic spasms treated with vigabatrin aged >4 weeks and <2 years | Phase III single blind study of vigabatrin. Assessed visual field using ERG and confrontation assessment |
0/13 developed vigabatrin associated visual field defect | No vigabatrin associated visual field defect found. Patients were assessed and excluded if previously had opthalmological defect. | Limitations: Small sample size Ceased visual field assessment at 8 month mark |
| A lack of clinically apparent vision loss among patients treated with vigabatrin with infantile spasms: The UCLA experience Schwarz, M. D., Li, M., Tsao, J., Zhou, R., Wu, Y. W., Sankar, R., Wu, J. Y., & Hussain, S. A. 2016 USA | Treatment group: 143 identified. Dose details only available for 104/143 Comparison group: 256 with IS and not treated with VBN | Retrospective chart review from February 2007 to February 2014 | Assessed vision with visual field exam, ERG and considered visual field defect to be vigabatrin related if both peripheral and bilateral. | 91/104 (87.5%) had a clinical evaluation by ophthalmologist | Limited by reliance of vision loss in records. No baseline data and rarely included serial ERG. No consistency in VF examination. No formal perimetry conducted % minimal ERG. Missing exposure details for 27% 39/143 of exposed patients: potential for incomplete dose–response assessment. Confounded by comorbid visual disorders. High prevalence of other vision-impairing diagnoses that can mask or explain VF defects |
| Developed VAVFD: 0/104 (0%) | |||||
| Visual loss identified in | |||||
| 31% (45/143) exposed to VBN | |||||
| 32% (31/256) not exposed to VBN | |||||
| 91/104 (87.5%) had a clinical evaluation by ophthalmologist | |||||
| Developed VAVFD: 0/104 (0%) | |||||
| Visual loss identified in | |||||
| 31% (45/143) exposed to VBN | |||||
| 32% (31/256) not exposed to VBN | |||||
| 91/104 (87.5%) had a clinical evaluation by ophthalmologist | |||||
| Developed VAVFD: 0/104 (0%) | |||||
| Visual loss identified in | |||||
| 31% (45/143) exposed to VBN | |||||
| 32% (31/256) not exposed to VBN | |||||
| Vigabatrin retinal toxicity in children with infantile spasms: an observational cohort study. Westall, Carol A. 2014 Canada | 146 children with infantile epileptic spasms treated with vigabatrin | Observation cohort study from September 1998 to December 2012. Assessed vision with ERG at baseline and >2 follow ups. | Developed VAVFD: 30/146 (21%) | No significant effect of cumulative exposure. | Limitations: Single centre specialist service Industry funding Loss to follow up |
| Duration of treatment: Developed VAVFD (%) | |||||
| 6 mths: 5.3 % | |||||
| 12 mths: 13.3% | |||||
| Does vigabatrin treatment for infantile spasms cause visual field defects? An international multicentre study. Riikonen, R., Carmant, L., Dorofeeva, M., Hollody, K., Krajnc, B., Rener, A., Primec, Z., Szabo, I., Wohlrab, G., & Sorri, I. 2013 Montreal, Moscow, Ljubljana, Kuopio, Pecs & Zurich | 32 children prescribed vigabatrin in infancy for infantile epileptic spasms | Observational cohort study. The children were examined using perimetry after the age of 9. | Developed VAVFD: 11/32 (34%) | Associations: | Small final sample for analysis No regression modelling for confounders No confidence intervals → poor precision Selection bias - Exclusion of children unable to perform perimetry |
| Rate of VAVFD increased from 11% (2 yrs) as duration of treatment increased. | |||||
| Duration of treatment: Developed VAVFD | Cumulative dose associated with VAVFD: 720g - 1440g | ||||
| < 1yr: 1/11(11%) (unconfirmed) | |||||
| 1-2 yrs: : 3/10 (30%) | |||||
| > 2 yrs: : 7 /11 (64%) | |||||
| Visual fields at school-age in children treated with vigabatrin in infancy. Gaily, E., Jonsson, H., & Lappi, M. 2009 Finland | 16 children treated with vigabatrin for infantile spasms were examined at ages 6-12. | Observational cohort group. Took place from 1993 to 2001. Patients were examined with visual field exam or goldman kinetic perimetry. |
Developed VAVFD: 1/16 (6.25%) | No association with vigabatrin and visual field impairment. | No multivariable analysis Small sample size Selection bias – only children who could cooperate with perimetry. Incomplete exam data for several children No serial VF data |
| VBN prescribed for 19 mths & received a cumulative dose of 572g. Had improved results on repeat testing | |||||
| VBN started at a mean age of 7.6 mths & duration 21 mths. | |||||
| Cumulative average dose: 655g. | |||||
| Visual impairment in children with epilepsy treated with vigabatrin. Gross-Tsur, V., Banin, E., Shahar, E., Shalev, R. S., & Lahat, E. 2000 Israel | 24 children were prescribed vigabatrin for over 6 months. Only 3 infantile epileptic spasms syndromes | Retrospective chart review. Children were examined with fundoscopy +/- ERG, VEP & perimetry. | Developed VAVFD: 0/3 (0%) | No association amongst the 3 children who had infantile spasms treated with vigabatrin and visual field impairment. | Small sample size Unable to perform VF testing in majority. No statistical adjustment for confounders Absence of pretreatment ocular examinations. |
Author Commentary:
The evidence is inconsistent regarding the association of visual field defects in infants with EISS treated with vigabatrin, ranging from 0-69%. (2-8) The prevalence varies depending on the study, length of follow-up, the method of testing and the duration of Vigabatrin exposure.
The incidence is much lower than the estimated prevalence of visual field defects associated with vigabatrin in the adult population of 45%. (9) This is consistent in all studies.
A number of papers demonstrated no cases or low frequency of VAVFD (3, 4, 8). The risk appears to stratify depending on the length of treatment, with exposure ≤12 months associated with a very low risk (12 months rising to 6-63%. (6% in y et al 2009, 13% in Westall et al 2015, 31% in Jonsson et al 2022, up to 63% in Riikonen et al 2013) (2, 5-7). The cumulative dose is a less consistent predictor of visual field defect in multiple cohorts (2, 5-7). Therefore, the duration of vigabatrin exposure appears to be the strongest consistent predictor of visual field loss.
Within this comparison, the majority of studies had small sample sizes (3-124) with VF testing inconsistent across centres and studies. The majority of children cannot complete perimetry tests, the gold standard for identifying visual field defects. Therefore, the true prevalence may be underestimated. In addition, there is a paucity of data studying the rate of VF loss/defects in children with epilepsy/genetic disorders not prescribed vigabatrin. Schwarz and colleagues showed a similar rates among children treated and not treated with VBN. (4) A number of studies focused on ERG abnormalities or subjective exams, which do not necessarily manifest as a clinical visual field defect (3, 4, 5). In the last 10 years, there are a paucity of new studies studying vigabatrin in IESS.
Future visual surveillance in this population may increasingly move from reliance on conventional perimetry toward handheld optical coherence tomography (OCT), as Rufai et al. demonstrated that handheld OCT can successfully obtain high-quality retinal and optic nerve imaging in awake infants and young children without sedation, overcoming major barriers of age, cooperation, and clinic accessibility that limit formal visual field testing.
The incidence is much lower than the estimated prevalence of visual field defects associated with vigabatrin in the adult population of 45%. (9) This is consistent in all studies.
A number of papers demonstrated no cases or low frequency of VAVFD (3, 4, 8). The risk appears to stratify depending on the length of treatment, with exposure ≤12 months associated with a very low risk (12 months rising to 6-63%. (6% in y et al 2009, 13% in Westall et al 2015, 31% in Jonsson et al 2022, up to 63% in Riikonen et al 2013) (2, 5-7). The cumulative dose is a less consistent predictor of visual field defect in multiple cohorts (2, 5-7). Therefore, the duration of vigabatrin exposure appears to be the strongest consistent predictor of visual field loss.
Within this comparison, the majority of studies had small sample sizes (3-124) with VF testing inconsistent across centres and studies. The majority of children cannot complete perimetry tests, the gold standard for identifying visual field defects. Therefore, the true prevalence may be underestimated. In addition, there is a paucity of data studying the rate of VF loss/defects in children with epilepsy/genetic disorders not prescribed vigabatrin. Schwarz and colleagues showed a similar rates among children treated and not treated with VBN. (4) A number of studies focused on ERG abnormalities or subjective exams, which do not necessarily manifest as a clinical visual field defect (3, 4, 5). In the last 10 years, there are a paucity of new studies studying vigabatrin in IESS.
Future visual surveillance in this population may increasingly move from reliance on conventional perimetry toward handheld optical coherence tomography (OCT), as Rufai et al. demonstrated that handheld OCT can successfully obtain high-quality retinal and optic nerve imaging in awake infants and young children without sedation, overcoming major barriers of age, cooperation, and clinic accessibility that limit formal visual field testing.
Bottom Line:
● Small but measurable risk of visual field constriction associated with vigabatrin use for IESS
● Very low risk in infants (0-14%) treated for less than 12 months
● Absolute risk in infancy (47/340 – 0.14%) is lower than expected when compared to older children and adults.
● VAVFD associated with cumulative exposure over time to vigabatrin rather than total cumulative dose
● Clinicians should limit exposure to the shortest effective duration, monitor vision when feasible, and discuss potential risks with caregivers. Consideration must be carried out for the high rates of comorbid conditions that may limit visual potential in this population and the clear clinical benefit of seizure control from vigabatrin.
References:
1. O'Callaghan, F. J., Edwards, S. W., Alber, F. D., Hancock, E., Johnson, A. L., Kennedy, C. R., Likeman, M., Lux, A. L., Mackay, M., Mallick, A. A., Newton, R. W., Nolan, M., Pressler, R., Rating, D., Schmitt, B., Verity, C. M., Osborne, J. P., & participating investigators (2017). Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial. The Lancet. Neurology, 16(1), 33–42. https://doi.org/10.1016/S1474-4422(16)30294-0
2. Jonsson, H., Lehto, M., Vanhatalo, S., Gaily, E., & Linnankivi, T. (2022). Visual field defects after vigabatrin treatment during infancy: retrospective population-based study. Developmental Medicine and Child Neurology, 64(5), 641–648. https://doi.org/https://dx.doi.org/10.1111/dmcn.15099
3. Ohtsuka, Y. (2018). Efficacy and safety of vigabatrin in Japanese patients with infantile spasms: Primary short-term study and extension study. Epilepsy and Behavior, 78, 134–141. https://doi.org/https://dx.doi.org/10.1016/j.yebeh.2017.09.010
4. Schwarz, M. D., Li, M., Tsao, J., Zhou, R., Wu, Y. W., Sankar, R., Wu, J. Y., & Hussain, S. A. (2016). A lack of clinically apparent vision loss among patients treated with vigabatrin with infantile spasms: The UCLA experience. Epilepsy & Behavior : E&B, 57(Pt A), 29–33. https://doi.org/10.1016/j.yebeh.2016.01.012
5. Westall, Carol A., et al. "Vigabatrin retinal toxicity in children with infantile spasms: an observational cohort study." Neurology 83.24 (2014): 2262-2268.https://doi.org/10.1212/WNL.0000000000001069
6. Riikonen, R., Carmant, L., Dorofeeva, M., Hollody, K., Krajnc, B., Rener, A., Primec, Z., Szabo, I., Wohlrab, G., & Sorri, I. (2013). Does vigabatrin treatment for infantile spasms cause visual field defects? An international multicentre study. European Journal of Paediatric Neurology, 17(SUPPL. 1), S49. https://doi.org/https://dx.doi.org/10.1016/S1090-37981370166-1
7. Gaily, E., Jonsson, H., & Lappi, M. (2009). Visual fields at school-age in children treated with vigabatrin in infancy. Epilepsia, 50(2), 206–216. https://doi.org/https://dx.doi.org/10.1111/j.1528-1167.2008.01961.x
8. Gross-Tsur, V., Banin, E., Shahar, E., Shalev, R. S., & Lahat, E. (2000). Visual impairment in children with epilepsy treated with vigabatrin. Annals of Neurology, 48(1), 60–64. https://search.ebscohost.com/login.aspx?direct=true&db=mdl&AN=10894216&site=ehost-live
9. Maguire, M.J., Hemming, K., Wild, J.M., Hutton, J.L. and Marson, A.G. (2010), Prevalence of visual field loss following exposure to vigabatrin therapy: A systematic review. Epilepsia, 51: 2423-2431. https://doi.org/10.1111/j.1528-1167.2010.02772.x
10. Rufai SR. Handheld optical coherence tomography removes barriers to imaging the eyes of young children. Eye (Lond). 2022;36(5):907-908. doi:10.1038/s41433-021-01884-5
● Very low risk in infants (0-14%) treated for less than 12 months
● Absolute risk in infancy (47/340 – 0.14%) is lower than expected when compared to older children and adults.
● VAVFD associated with cumulative exposure over time to vigabatrin rather than total cumulative dose
● Clinicians should limit exposure to the shortest effective duration, monitor vision when feasible, and discuss potential risks with caregivers. Consideration must be carried out for the high rates of comorbid conditions that may limit visual potential in this population and the clear clinical benefit of seizure control from vigabatrin.
References:
1. O'Callaghan, F. J., Edwards, S. W., Alber, F. D., Hancock, E., Johnson, A. L., Kennedy, C. R., Likeman, M., Lux, A. L., Mackay, M., Mallick, A. A., Newton, R. W., Nolan, M., Pressler, R., Rating, D., Schmitt, B., Verity, C. M., Osborne, J. P., & participating investigators (2017). Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial. The Lancet. Neurology, 16(1), 33–42. https://doi.org/10.1016/S1474-4422(16)30294-0
2. Jonsson, H., Lehto, M., Vanhatalo, S., Gaily, E., & Linnankivi, T. (2022). Visual field defects after vigabatrin treatment during infancy: retrospective population-based study. Developmental Medicine and Child Neurology, 64(5), 641–648. https://doi.org/https://dx.doi.org/10.1111/dmcn.15099
3. Ohtsuka, Y. (2018). Efficacy and safety of vigabatrin in Japanese patients with infantile spasms: Primary short-term study and extension study. Epilepsy and Behavior, 78, 134–141. https://doi.org/https://dx.doi.org/10.1016/j.yebeh.2017.09.010
4. Schwarz, M. D., Li, M., Tsao, J., Zhou, R., Wu, Y. W., Sankar, R., Wu, J. Y., & Hussain, S. A. (2016). A lack of clinically apparent vision loss among patients treated with vigabatrin with infantile spasms: The UCLA experience. Epilepsy & Behavior : E&B, 57(Pt A), 29–33. https://doi.org/10.1016/j.yebeh.2016.01.012
5. Westall, Carol A., et al. "Vigabatrin retinal toxicity in children with infantile spasms: an observational cohort study." Neurology 83.24 (2014): 2262-2268.https://doi.org/10.1212/WNL.0000000000001069
6. Riikonen, R., Carmant, L., Dorofeeva, M., Hollody, K., Krajnc, B., Rener, A., Primec, Z., Szabo, I., Wohlrab, G., & Sorri, I. (2013). Does vigabatrin treatment for infantile spasms cause visual field defects? An international multicentre study. European Journal of Paediatric Neurology, 17(SUPPL. 1), S49. https://doi.org/https://dx.doi.org/10.1016/S1090-37981370166-1
7. Gaily, E., Jonsson, H., & Lappi, M. (2009). Visual fields at school-age in children treated with vigabatrin in infancy. Epilepsia, 50(2), 206–216. https://doi.org/https://dx.doi.org/10.1111/j.1528-1167.2008.01961.x
8. Gross-Tsur, V., Banin, E., Shahar, E., Shalev, R. S., & Lahat, E. (2000). Visual impairment in children with epilepsy treated with vigabatrin. Annals of Neurology, 48(1), 60–64. https://search.ebscohost.com/login.aspx?direct=true&db=mdl&AN=10894216&site=ehost-live
9. Maguire, M.J., Hemming, K., Wild, J.M., Hutton, J.L. and Marson, A.G. (2010), Prevalence of visual field loss following exposure to vigabatrin therapy: A systematic review. Epilepsia, 51: 2423-2431. https://doi.org/10.1111/j.1528-1167.2010.02772.x
10. Rufai SR. Handheld optical coherence tomography removes barriers to imaging the eyes of young children. Eye (Lond). 2022;36(5):907-908. doi:10.1038/s41433-021-01884-5
Level of Evidence:
Level 2: Studies considered were neither 1 or 3
References:
- Jonsson, H., Lehto, M., Vanhatalo, S., Gaily, E., & Linnankivi, T.. Visual field defects after vigabatrin treatment during infancy: retrospective population-based study.
- Ohtsuka, Y.. Efficacy and safety of vigabatrin in Japanese patients with infantile spasms: Primary short-term study and extension study.
- Schwarz, M. D., Li, M., Tsao, J., Zhou, R., Wu, Y. W., Sankar, R., Wu, J. Y., & Hussain, S. A.. A lack of clinically apparent vision loss among patients treated with vigabatrin with infantile spasms: The UCLA experience
- Westall, Carol A.. Vigabatrin retinal toxicity in children with infantile spasms: an observational cohort study.
- Riikonen, R., Carmant, L., Dorofeeva, M., Hollody, K., Krajnc, B., Rener, A., Primec, Z., Szabo, I., Wohlrab, G., & Sorri, I.. Does vigabatrin treatment for infantile spasms cause visual field defects? An international multicentre study.
- Gaily, E., Jonsson, H., & Lappi, M.. Visual fields at school-age in children treated with vigabatrin in infancy.
- Gross-Tsur, V., Banin, E., Shahar, E., Shalev, R. S., & Lahat, E.. Visual impairment in children with epilepsy treated with vigabatrin.