The clinical and electrophysiologic data (electroretinograms and visual evoked potentials) were studied in 45 patients with optic nerve hypoplasia. The patients were divided into three fairly distinct groups on the basis of their electrophysiologic alterations. Group 1 consisted of 13 patients with almost extinguished visual evoked potentials and with mild electroretinographic alterations. These were the cases that are traditionally recognized as optic nerve hypoplasia. The serious visual impairment in these cases was accompanied by various developmental ophthalmologic and nonophthalmologic abnormalities. Group 2 included 26 patients without any significant visual evoked potential or electroretinographic alterations, but with overt funduscopic signs of optic nerve hypoplasia. These patients were consistently suffering from strabismus and/or amblyopia. The visual functions based on visual evoked potential and electroretinographic recordings could be fairly normal apart from a pathologic ophthalmoscopic picture characteristic of optic nerve hypoplasia. Group 3 included six patients with abnormal albeit well-recordable visual evoked potentials and subnormal or negative-type electroretinograms that suggested an accompanying retinal disease. This finding seems to prove that a subset of patients with optic nerve hypoplasia with nystagmus may have a primary retinal abnormality. Our study provides further evidence that optic nerve hypoplasia is not a uniform disease entity.
The results suggest highly variable central responses and groups of cones with preserved function in areas previously considered nonresponsive. The high variability of the central responses could be a result of variable foveal cone density, with differences in inheritance- and duration-related cone degeneration at the time of the examination. The authors stress the value of step-by-step analysis of the trace array of the mfERGs, which can reveal the still-functioning groups of cones.
Purpose: To asses the effect of strabismus surgery on visual evoked potential (VEP) amplitude after age 5 years in children with acquired esotropia.
Methods: Visual evoked potentials to binocular and monocular pattern reversal stimulation were recorded in 10 children aged 5 to 6 years before and 3 months after surgical correction of their esotropia. Visual function (fusion) was tested by synoptophore after strabismus surgery. Electrophysiological and clinical data were correlated following surgical intervention.
Results: A significant increase in pattern VEP amplitudes was noted following strabismus surgery. Visual evoked potential changes were most prominent on binocular stimulation. Binocuiar fusion showed similarly significant improvement after intervention. Postoperative VEP data, however, were not consistently correlated with functional improvement.
Conclusion: Surgical intervention in esotropia, even if performed years beyond the end of the classical critical period, can have a strong effect on binocular VEPs and a beneficial effect on the development of binocular fusion.
Journal of Pediatric Ophthalmology and Strabismus 2001 ;38:83-88.
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