title: Electroretinographic assessment and diagnostic reappraisal of children with visual dysfunction: a prospective study.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Prospective observational case series.
  
  
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Retina-Vitreous Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India. drvasumathy@yahoo.com
authors.forename: Vasumathy
authors.initials: V
authors.lastname: Vedantham
authors.forename: Jithendra
authors.initials: J
authors.lastname: Jethani
authors.forename: Perumalsamy
authors.initials: P
authors.lastname: Vijayalakshmi
mesh_terms: D002675:Child, Preschool; D003117:Color Vision Defects / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D003937:Diagnosis, Differential; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009755:Night Blindness / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D029242:Optic Atrophy, Hereditary, Leber / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D011379:Prognosis; D011446:Prospective Studies; D012160:Retina / Q000503:physiopathology*; D015354:Vision, Low / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D014792:Visual Acuity