Health-related quality of life and visual function in retinoblastoma survivors with ocular prostheses: a...

Retinoblastoma (RB) survivors who have undergone enucleation and use ocular prostheses may face long-term challenges related to visual function and overall well-being. This study aimed to evaluate health-related quality of life (HR-QoL) and visual function in this population, addressing a gap in knowledge regarding their lived experiences and functional vision. This cross-sectional study included 12 unilateral and 3 bilateral RB survivors (10 girls, 5 boys; mean age 15.5 years, range 6.8-26.5), all treated with unilaterally enucleation and an ocular prosthesis at Sahlgrenska University Hospital, Sweden (2000-2019). HR-QoL was assessed using the Paediatric Quality of Life Inventory (PedsQL), both self- and parent-reports. Functional vision was evaluated by best-corrected visual acuity (BCVA) and structured history-taking of perceptual visual dysfunctions (PVDs). Results were compared with age-matched healthy controls. Self-reported PedsQL total score (82.5) equalled normative data (83.0) and parents' report (82.5). Parents' reports were comparable to published parental norms (82.5 vs. 87.6). Survivors with subnormal BCVA (≥ 0.2 LogMAR) had significantly lower self-reported HR-QoL scores than those with normal vision (64.1 vs. 88.0, p = 0.014), a difference not reflected in parent-reports. Survivors reported more PVDs ≥ 1 area (median 1, range 1-4) than controls (9/15 vs. 1/15, p = 0.005). The number of affected PVD areas correlated with age (r = 0.59, p = 0.022), but not with BCVA or PedsQL score. RB survivors with ocular prostheses require holistic care that includes assessment of HR-QoL and functional vision, beyond visual acuity, to identify unmet needs and guide interventions that support overall well-being and optimal long-term outcomes.