Evaluation of Visual Outcomes in Retinoblastoma Survivors and Determining Factors Predicting Failure of Vision Salvage

Theme: Paediatric ophthalmology and strabismus
What: Paediatric ophthalmology and strabismus
Part of: Paediatrics I: Pediatric Ophthalmology & Strabismus - Vision Screening to Retinoblastoma / Pédiatrie I: Ophtalmologie pédiatrique et strabisme - Dépistage du rétinoblastome
When: 6/1/2024, 04:15 PM - 05:45 PM
Where: Room | Salle 714 A

Abstract

Purpose: Improving survival rates of retinoblastoma (RB) has shifted the focus toward eye and vision salvage. We aim to identify factors responsible for suboptimal visual outcome and evaluate final vision and impairment in RB survivors. 

Study Design: Retrospective, Observational.

Methods: Clinical data of all RB patients treated from January 2000 to August 2021 at The Hospital for Sick Children was reviewed. Ocular characteristics, treatment, and final vision were documented. Salvaged eyes were required to have at least one year of quiescence to be included. In children with bilateral RB, visual impairment (VI) was categorized using ICD11. The visual acuity (VA) in logMAR for each salvaged eye was separately analyzed. Unsuccessful vision salvage was defined as eyes with vision potential that were primarily enucleated, that underwent secondary enucleation or were salvaged, but had visual outcomes worse than their potential. 

Results: 312 affected eyes of 222 children were included. 90 children (41%) had bilateral RB of which 11 (12%) had severe VI (5% of all children with RB). Diagnosis age of <6months was associated with lesser VI. None of them had severe VI or blindness when both eyes were salvaged. Overall, 187 eyes were enucleated of which 38 were secondary enucleations. Diagnosis age of <12 months was associated with higher chances of salvaged eye status. Of 125 eyes salvaged, one was excluded from analysis of visual outcome as there was no vision documented after treatment initiation. Mean VA was: 0.53 (n=124; SD: 0.69), and in each AJCC stage was: cT1: 0.28 (n= 80; SD: 0.45); cT2: 0.97 (n= 42; SD: 0.76); cT3: 1.45 (n= 2; SD: 1.91), with a mean follow-up of 9.51 years (SD: 5.43). A final VA of 6/60 or worse was found in 28 eyes (23%). As anticipated, a higher stage (p <.05) and involvement of the fovea (p<.05) were associated with poorer vision outcomes in the eyes that were salvaged. Eyes treated with external beam radiation (n=3) had an average VA of 1.59, as compared to 0.51 of eyes without. Of the 52 eyes with unsuccessful vision salvage, 4 eyes (all unilateral) with good vision potential underwent primary enucleation, 38 eyes underwent secondary enucleation, and 10 salvaged eyes were found to have worse vision compared to expected visual outcome.  

Conclusion: This study represents a large cohort and is the only one to report visual outcomes compared to different AJCC stages of disease. Most salvaged eyes had good vision after treatment and were associated with a lower stage at diagnosis and absence of foveal involvement. Of the eyes with vision unsuccessfully salvaged, most necessitated secondary enucleation due to disease progression. None of those with bilateral RB and both eyes salvaged had blindness or severe VI. The limitations include use of outdated treatment modalities over the long study period and not considering factors like amblyopia treatment contributing to suboptimal VA.

Presenter(s)

Presenting Author: Kanchan Sainani

Additional Author(s):

Helen Dimaras, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Omer Jamal, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Emilia Cirillo, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Andrew Malem, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Stephanie Kletke, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Furqan Shaikh, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Brenda Gallie, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

Ashwin Mallipatna, The Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.

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