| dc.description.abstract | Background: Retinoblastoma is a curable eye cancer with high survival rates (>90%) when
diagnosed and treated early. Visual prognosis is affected by tumour classification, location,
treatment and heredity. While newer treatment modalities enhance globe salvaging and
vision preservation, the long-term clinical outcomes of visual acuity (VA) and functional vision
are still understudied, leading to challenges for parents in understanding their child's vision.
Aim: To investigate the different factors influencing VA outcomes and functional vision in
patients diagnosed with retinoblastoma.
Methods: This retrospective observational cohort study included all patients with
retinoblastoma from a national centre between 1991-2015. Patients with missing data on
treatment modality and inaccurate VA measurements were excluded. VA outcomes were
measured at ages 7, 12, and 18 using the LogMAR scale with recognition acuity. Functional
vision was assessed using the international PEDIG scale for visual impairment (VI). Effects
of tumour classification, location, treatment modalities and heredity on VA outcomes were
analysed.
Results: This study included 271 patients with 379 retinoblastoma-diagnosed eyes. At age 7,
enucleation rate was 60% (229 eyes), with 18% experiencing VI. Tumour classification,
location, treatment, and heredity were strongly associated with VA outcomes (p<0.001).
Familial heredity showed the best VA outcomes (LogMAR 0.20), sporadic heredity had
severe VI prevalence (31%), and sporadic non-hereditary had high enucleation rates (93%).
Foveal tumours resulted in poor VA outcomes (LogMAR 1.80), while peripheral tumours had
better outcomes (LogMAR 0.00). Tumour classification, location and treatment are correlated
with each other (p<0.001). Therefore influencing VA and leading to increased VI in
advanced-staged tumours and more aggressive treatments.
Conclusion: Patients with retinoblastoma exhibited high rates of blindness due to
enucleation, but maintained high functional vision without VI (82%). Heredity played a vital
role, showing the importance of early screening. Tumour location significantly impacted VA
outcomes with localization closer to the macula resulting in poorer vision. Less severe
tumours received less aggressive treatments, leading to better VA outcomes.
Recommendations: The findings of this study provide valuable insights for personalized
support, highlighting the urgent need for a new classification system considering vision
preservation and further exploration of vision-related quality of life effects. | |
