June 2020
Volume 61, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2020
Survivorship, Vision and Eye Salvage Following Pars Plana Vitrectomy for Residual Active or Recurrent Retinoblastoma
Author Affiliations & Notes
  • Zhao Xun Feng
    University of Ottawa, Ottawa, Ontario, Canada
  • Junyang Zhao
    Pediatric Oncology Center, Beijing Children’s Hospital, Beijing, China
  • Qiyan Li
    Department of Ophthalmology, Beijing Tongren Hospital, Beijing, China
  • Songyi Wu
    Department of Ophthalmology, Quanzhou Children’s Hospital, Quanzhou, China
  • Liwen Jin
    Department of Ophthalmology, Quanzhou Children’s Hospital, Quanzhou, China
  • Brenda L Gallie
    Department of Ophthalmology and Vision Sciences, Hospital for Sick Children, Toronto, Ontario, Canada
    Department of Ophthalmology, University of Toronto, Toronto, Ontario, Canada
  • Footnotes
    Commercial Relationships   Zhao Xun Feng, None; Junyang Zhao, None; Qiyan Li, None; Songyi Wu, None; Liwen Jin, None; Brenda Gallie, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 2813. doi:
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      Zhao Xun Feng, Junyang Zhao, Qiyan Li, Songyi Wu, Liwen Jin, Brenda L Gallie; Survivorship, Vision and Eye Salvage Following Pars Plana Vitrectomy for Residual Active or Recurrent Retinoblastoma. Invest. Ophthalmol. Vis. Sci. 2020;61(7):2813.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Intraocular surgery has long been tabooed in eyes with retinoblastoma due to concern of extraocular spread. However, this contraindication largely came from reports of metastatic death following intraocular procedures in eyes with unsuspected retinoblastoma. Our group recently reported on 21 children who received pars plana vitrectomy (PPV) to remove tumor from their last remaining eye and showed no evidence of extraocular spread. To further investigate the safety and efficacy of PPV, we studied the survivorship, vision and eye salvage of a large cohort of retinoblastoma children treated with PPV.

Methods : Outcomes were reviewed for 225 children (245 eyes) treated with PPV for residual active tumor or recurrent tumor following primary chemotherapy at 3 Chinese centers between 2013 and 2014.

Results : At 5 years following PPV, 12 patients died, 8 related to the eye that had PPV. The 5-year overall survival rate was 94.1% (95% CI: 91.1% to 97.1%). The 5-year PPV eye-related survival rate (PPVSR) was 96.3% (95% CI: 93.8% to 98.9%). Children with PPV for residual active tumor had better survival than those with PPV for recurrent tumor (5-year PPVSR 98.1% vs 91.4%, P = .021). Children with adjuvant systemic chemotherapy following PPV had 100% (95% CI: 93.5% to 100%) 5-year PPVSR, not significantly higher than those without adjuvant chemotherapy (100% vs 94.8%, P = .102). Of 153 eyes with partial or no retinectomy, post-PPV visual acuity was 20/20–20/60 (23, 15%); 20/80–20/160 (6, 4%); 20/200–20/400 (9, 6%); counting fingers, hand motion or light perception (42, 28%); no light perception (NLP) (45, 29%); visual acuity unavailable (28, 18%). Complete retinectomy was performed on 92 eyes, which as expected, had NLP. The 5-year eye salvage rate following PPV was 80.6% (95% CI: 75.4% to 85.8%). PPV for residual active tumor and recurrent tumor had similar eye salvage rate (82.0% vs 76.7%, P = .379). Eyes with IIRC B or C staging had better salvage rate than eyes with IIRC D or E staging (100.0% vs 78.0%, P = .005).

Conclusions : PPV circumvents chemotherapy resistance by direct excision of tumor while also managing complications that limit vision such as cataract, vitreous hemorrhage, vitreous seeding and retinal detachment. We showed that PPV achieved 5-year eye salvage of 80.6% and eye-specific survival of 96.3%. PPV has potential to be part of the standard of care for retinoblastoma.

This is a 2020 ARVO Annual Meeting abstract.

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