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Updated: Nov 2, 2025
Electroretinogram Recording for Infants and Children under Anesthesia to Achieve Optimal Dark Adaptation and International Standards
Published on: September 3, 2020
An alternative electroretinography protocol for children: a study of diagnostic agreement and accuracy relative to
Oliver R Marmoy1,2,3, Mohammed Moinuddin2,4, Dorothy A Thompson1,2
1Tony Kriss Visual Electrophysiology Unit, Great Ormond Street Hospital for Children, London, UK.
Insights
The Great Ormond Street Hospital (GOSH-ERG) protocol accurately diagnoses paediatric retinal diseases, showing high agreement with the international standard (ISCEV-ERG) for children unable to complete the full test.
Area of Science:
- Ophthalmology
- Medical Imaging
- Pediatric Medicine
Background:
- Electroretinography (ERG) is crucial for diagnosing pediatric retinal diseases.
- The International Society for Clinical Electrophysiology of Vision (ISCEV) standard protocol (ISCEV-ERG) is the gold standard but can be challenging for children.
- A modified protocol at Great Ormond Street Hospital (GOSH-ERG) was developed to improve compliance.
Purpose of the Study:
- To evaluate the diagnostic accuracy and agreement between the GOSH-ERG and the ISCEV-ERG protocols.
- To compare the performance of both protocols in identifying retinal abnormalities in children.
Main Methods:
- Retrospective analysis of patient databases (2010-2020).
- Inclusion criteria: children with both GOSH-ERG and ISCEV-ERG recorded within four years.
- Analysis of ERG component peak times and amplitudes, assessing absolute abnormality and proportional deviation from reference ranges.
Main Results:
- 59 children included (38 with retinal disease, 21 normal).
- GOSH-ERG demonstrated excellent overall sensitivity (95%) and accuracy (86%) compared to ISCEV-ERG.
- High sensitivity and specificity observed for individual retinal systems and ERG components, with comparable waveform morphology across various retinal diseases.
Conclusions:
- The GOSH-ERG protocol exhibits high diagnostic accuracy comparable to the ISCEV-ERG standard.
- The findings support the use of GOSH-ERG for screening, diagnosing, and monitoring pediatric retinal diseases, especially in children with compliance difficulties.
- The GOSH-ERG offers a viable alternative for pediatric electroretinography when the ISCEV-ERG protocol is not feasible.
Purpose:
To assess the diagnostic accuracy and agreement between a paediatric electroretinography protocol used at Great Ormond Street Hospital (GOSH-ERG) and the 'gold standard' international protocol (ISCEV-ERG) in health and disease.
Methods:
Patient databases between 2010 and 2020 were screened to identify children with an ISCEV-ERG recorded within four years of a GOSH-ERG. Electroretinogram (ERG) component peak times and amplitudes were re-measured, and data were analysed in terms of absolute abnormality and proportional deviation from respective reference ranges. Abnormality was defined by the retinal system affected and by individual ERG a- and b-wave component analysis.
Results:
A total of 59 patients were included: 38 patients had retinal disease defined by an abnormal ISCEV-ERG and 21 had normal ISCEV-ERGs. When absolute abnormality was defined by combined retinal systems, the GOSH-ERG showed an excellent overall sensitivity of 95% (accuracy 86%). Individual retinal systems showed good-excellent sensitivity (67%-100%) and specificity (68%-97%). Electroretinogram (ERG) component sensitivities ranged between 60% and 97% and specificities between 79% and 97% dependent upon the protocol step. The proportional relationship appeared mostly linear between protocols. Electroretinogram (ERG) morphology was comparable for both protocols in a range of retinal diseases including those with pathognomonic ERGs.
Conclusion:
We demonstrate the high diagnostic accuracy of a paediatric ERG protocol (GOSH-ERG) relative to ISCEV standard ERGs. The close proportional deviation and similar waveform morphology indicate ERGs from each protocol are similarly affected in disease. This encourages the use of the GOSH-ERG protocol in the screening, diagnosis and monitoring of retinal disease in children who are unable to comply with the rigorous ISCEV-ERG protocol.
