Electroretinographic assessment and diagnostic reappraisal of children with visual dysfunction: a prospective study

Vedantham, Vasumathy; Jethani, Jithendra; Vijayalakshmi, Perumalsamy
March 2007
Indian Journal of Ophthalmology;Mar2007, Vol. 55 Issue 2, p113
Academic Journal
journal article
Purpose: To assess the presence or absence of a retinal cause of visual impairment using electroretinography (ERG) in children with no obvious discernable cause on ocular examination.Design: Prospective observational case series.Materials and Methods: A prospective study was carried out involving 120 children with the mean age 4.4+/-3.2 years with visual dysfunction. All children underwent ERG under general anesthesia using a special handheld mini-Ganzfeld (Kurbisfeld) dome.Results: Fifty-two (43.3%) children were male and 68 (56.7%) were female. The clinical diagnosis was as follows: Leber's congenital amaurosis (LCA) (n=47), achromatopsia (n=25), congenital stationary night blindness (CSNB) (n=9) and others (unclassifiable, n=39). The visual acuity ranged from perception of light (PL) to PL with projection in children with LCA. In the rest (n=73), some sort of visually guided behavior was discernable. Following ERG, a diagnostic reappraisal resulted as follows: LCA (n=49), achromatopsia (n=28), CSNB (n=4), cone-rod dystrophy (n=22), rod-cone degeneration (n=7), normal (n=8) and others (unclassifiable, n=2). Except for the two unclassifiable cases, ERG was successful in the diagnosis or exclusion of retinal dysfunction in the rest. By Pearson Chi-square test, there was a statistically significant association between the clinical and ERG diagnosis (P < 0.001).Conclusion: LCA was the commonest cause of visual dysfunction in our series. A statistically significant correlation between clinical and electrophysiological diagnosis was seen. ERG helped in firmly establishing the presence or absence of global retinal dysfunction in the majority (118/120) of pediatric patients with visual dysfunction.


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