Sporadic aniridia and Wilms' tumor: visual function evaluation of three cases.
The visual function of three infants with sporadic aniridia associated with Wilms' tumor and a deletion of the short arm of chromosome 11 was evaluated with electrophysiological tests. The patients presented nystagmus and photophobia. The electroretinograms (ERGs) were normal, as in other sporadic cases, but at variance with the familial cases. The latency of the flash visual evoked potentials (FVEPs) became shorter with time but remained longer than in age-matched controls, suggesting a delay in maturation of the nervous system. Poor visual function in our cases did not appear to be the result of gross retinal anomalies, as shown by the normal ERG, nor of an hypoplasia of the optic nerve. Contact lenses that provide an artificial pupil decreased photophobia and nystagmus and are therefore highly recommended to increase patient comfort.
Harnois
C
C
Unité de Recherche en Ophthalmologie, Centre Hospitalier de l'Université Laval, Québec, Canada.
Boisjoly
H M
HM
Jotterand
V
V
D002872:Chromosome Deletion; D002880:Chromosomes, Human, Pair 11; D003261:Contact Lenses; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000150:complications; D007498:Iris / Q000002:abnormalities*; D007680:Kidney Neoplasms / Q000150:complications* / Q000503:physiopathology; D008297:Male; D009759:Nystagmus, Pathologic / Q000150:complications; D014787:Vision Tests; D014785:Vision, Ocular; D009396:Wilms Tumor / Q000150:complications* / Q000503:physiopathology