Therapy-associated oculo-visual dysfunction affects vision-targeted health-related quality of life (HRQOL) and functional status among adult survivors of retinoblastoma, according to a study published in JAMA Ophthalmology.

Retinoblastoma is a very common malignancy in childhood and has an excellent prognosis; the survival rate is more than 95% for this patient population in developed countries. The effect of treatment-related adverse effects on vision-targeted HRQOL among adult survivors, however, is still unknown.

For the retrospective, self-reported Retinoblastoma Survivor Study, researchers assessed the outcomes of 470 adult retinoblastoma survivors who were treated between 1932 and 1994. Vision-related HRQOL was evaluated using the 25-item National Eye Institute Visual Field Questionnaire (NEI-VFQ-25). Of study participants, 86% had at least 1 eye removed, 56.5% were treated with radiotherapy, 61.3% rated their eyesight as good or excellent, and 16.2% reported being completely blind.

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The overall mean NEI-VFQ-25 composite score for all survivors was 81.1. The mean score was 91.4 and 72.3 among patients who had unilateral and bilateral retinoblastoma, respectively, with significant differences observed in measures of visual status and laterality (eg, general health, vision, near and distance activities, driving).

Patients who received radiotherapy did not have any significant reductions in NEI-VFQ-25 scores compared with patients who did not, but exposure was associated with worse scores on certain subscales. Patients with unilateral disease had lower peripheral vision subscale and dependency subscale scores, and survivors of bilateral disease had significantly lower scores on the near vision subscale.

The authors concluded that there is a “need for continued development of vision-preserving and eye-sparing procedures in the treatment of retinoblastoma, when possible, and suggests long-term survivors should be assessed for specific areas of need to strive for optimization of supportive services.”


Friedman DN, Chou JF, Francis JH, et al. Vision-targeted health-related quality of life in adult survivors of retinoblastoma[published online April 26, 2018]. JAMA Ophthalmol. doi: 10.1001/jamaophthalmol.2018.1082