A prospective study of children treated for retinoblastoma: Cognitive and visual outcomes in relation to treatment

Abstract

ABSTRACT.Purpose: To assess cognitive and visual outcomes in children treated for retinoblastoma. Patients and Methods: A population-based group of 22 children treated for retinoblastoma were followed in a longitudinal, prospective study. Eleven children had unilateral tumours, all of which had been enucleated. The remaining 11 had bilateral tumours. Seven of these had undergone unilateral enucleation and local or external beam radiation to the other eye. Four children had been treated with local or external beam radiation only. Cognitive outcome and visual function was assessed at 4 and 6 years of age. Results: We found no general delay in cognitive or motor development at 4 or 6 years of age. Better cognitive capacities were found in the bilateral/irradiated retinoblastoma patients than in unilateral/non-irradiated patients. However, two of the irradiated children, both of whom had been treated at 1 month of age, performed below group mean. None of the children was totally blind. Three children were visually impaired due to tumours affecting the macular area in the remaining eye; another three had subnormal visual acuity but no macular pathology. A total of 14 children had been enucleated and had normal vision in the remaining eye. Visual fields and dark adaptation were adversely affected in a few cases, but colour vision was normal in all. Conclusion: Bilateral retinoblastoma seems to be associated with superior cognitive capacities. Few children were visually impaired according to WHO criteria. We speculate that children treated during the first months of life may be at risk of adverse cognitive and visual outcomes. The immature brain may be affected by radiation treatment, causing both cognitive and visual deviations.

Developmental and Adaptive Functioning in Children With Retinoblastoma: A Longitudinal Investigation

“…5,9,10 This highlights the necessity of continuing to assess cognitive functioning in patients with retinoblastoma as they age. Early childhood is a time of considerable cognitive growth and variability.…”

“…To date, the available studies have relied on observations of children who were several years post-treatment 5,9 or consisted of cross sectional assessments at only one point in time, 9,10 thus limiting the conclusions that can be made regarding potential change in functioning over time. Thus, the early developmental trajectory of these children, who were diagnosed and treated during infancy and toddlerhood, remains unknown.…”

“…In contrast, more recent reports have suggested that cognitive functioning after diagnosis and treatment of retinoblastoma is within the average to above-average range and similar to that for same-age peers. 5,9,10 Notably, there are variations in cognitive performance, particularly with regard to age at diagnosis, disease location (unilateral v bilateral), and treatment modality. 9 Children treated before the age of 3 months, those with unilateral disease, and those treated with radiation therapy at a young age performed significantly worse than their counterparts.…”

“…9 Children treated before the age of 3 months, those with unilateral disease, and those treated with radiation therapy at a young age performed significantly worse than their counterparts. 5 In addition, an estimated 5% to 10% of patients with retinoblastoma will have an interstitial 13q chromosomal deletion. 11 These patients have been noted to…”

“…4,5 Early studies of intellectual functioning in children with retinoblastoma suggested that survivors demonstrated above-average intellectual functioning when compared with the general population or unaffected siblings. [6][7][8] Such findings were particularly true of children whose disease was associated with blindness.…”