Aims In optic nerve hypoplasia (ONH), the extent of functional loss of retinal ganglion cells cannot be determined by ophthalmoscopic examination. The prognostic value of visual electrodiagnostic tests in infants and toddlers with ONH was assessed by comparison with visual outcome.
Methods 85 participants with ONH had electroretinogram (ERG) and visual-evoked potential (VEP) testing to flash and to pattern-reversal checks and ocular fundus photography prior to 36 months of age. These initial measures were compared with visual acuity outcomes at 5 years of age in the better-seeing eye.
Results Visual outcomes ranged from normal to no light perception. Electrodiagnostic tests with prognostic value were: the amplitude of the flash VEP (Spearman's rank correlations, p<0.001), the threshold category of stimulus (flash or check size) that elicited a VEP (p<0.001) and the amplitude of the N95 component of the pattern ERG (PERG) to 4-degree checks (p<0.02). Optic nerve size and co-existing pallor were also significant correlates. Stepwise regression analysis composed a best prediction model from VEP threshold category, optic nerve size and optic disc pallor (R2=58%; p<0.001).
Conclusions Optic disc diameter, observation of disc pallor, VEP and PERG testing in infancy are useful for establishing the visual prognosis at 5 years of age in children with ONH. This is consistent with the notion that these parameters are related to the anatomic and functional preservation of retinal ganglion cells.
- Optic nerve
- visual pathway
- clinical trial
- child health (paediatrics)
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Funding This research was supported in part by One Small Voice Foundation, the Vision Research Trust (Scotland), the Carnegie Trust for the Universities of Scotland and by NIH General Clinical Research Center grant (M01 RR00043). The One Small Voice Foundation provided funds to support personnel for study oversight, data collection and management, statistical analyses and article preparation. Database support was provided by the General Clinical Research Center, and studies were performed in the General Clinical Research Center satellite at CHLA. The Vision Research Trust provided a PhD scholarship for CAC. The Carnegie Trust supported travel. Other funders: NIH.
Competing interests None.
Ethics approval This study was conducted with the approval of the Committee on Clinical Investigations at Children's Hospital Los Angeles.
Provenance and peer review Not commissioned; externally peer reviewed.