Pediatric Cataract Surgery Following Treatment for Retinoblastoma: A Case Series and Systematic Review

Purpose: To determine the visual and refractive outcomes and the ocular and systemic complications of cataract surgery in eyes treated for retinoblastoma.

Design: Retrospective consecutive case series and systematic review.

Methods: Children <18 years of age with retinoblastoma who underwent surgery for secondary cataract between 2000 and 2020 were reviewed. Medline (OVID), Embase, Web of Science, and the Cochrane database were searched from inception to August 2020.

Results: A total of 15 eyes of 15 children were included. The mean age at retinoblastoma diagnosis was 12 months (median, 14; interquartile range [IQR], 4-19). Cataract developed at a mean age of 39 months (median, 31; IQR, 20-52), secondary to multiple treatments (n = 7), pars-plana vitrectomy (n = 3), external-beam radiotherapy (n = 2), laser (n = 2), and retinal detachment (n = 1). The mean preoperative quiescent interval was 44 months (median, 28; IQR, 15-64). Primary intraocular lens implantation was performed in 93%, posterior capsulotomy in 40%, and anterior vitrectomy in 33% of participants. Postoperatively, 100% had improved fundus visibility and 73% had improved vision. Complications included visual axis opacification (11 of 15), capsular phimosis (5 of 15), and zonulopathy (3 of 15). No patient developed intraocular recurrence, extraocular extension, or metastasis at a mean of 76 months (median, 78; IQR, 29-128) follow-up. The systematic review identified 852 studies, with 18 meeting inclusion criteria. Across all studies (n = 220 children), intraocular recurrence occurred in 6%, globe salvage in 91%, and extraocular extension and metastasis in <1%.

Conclusions: Modern retinoblastoma therapies, including intravitreal chemotherapy and vitrectomy, cause secondary cataract. Following cataract surgery, intraocular recurrence risk is low and extraocular spread is rare. Although surgery improves tumor visualization, visual prognosis may be limited by several factors. Challenges include biometry limitations and a high incidence of zonulopathy.