Macular retinoblastoma: Evaluation of tumor control, local complications, and visual outcomes for eyes treated with chemotherapy and repetitive foveal laser ablation

Objective: To determine tumor control rates, complication rates, and visual acuity (VA) for patients with macular retinoblastoma undergoing systemic chemotherapy and foveal diode laser ablation. Design: Noncomparative retrospective case series. Participants: All patients with retinoblastoma in the macula at the Bascom Palmer Eye Institute between 1991 and 2004 were evaluated. Patients included in the study were managed by the same clinician with a planned therapeutic strategy. Methods: Patients with Reese-Ellsworth groups I to IV disease underwent 4 to 9 cycles of systemic chemotherapy with vincristine, etoposide, and carboplatin, and patients with group V disease underwent 6 to 10 cycles with or without cyclosporine. All tumors underwent repetitive diode laser ablation (2-24 sessions) applied to the foveal and extrafoveal portions of tumors at each visit until tumors were deemed inactive for at least 6 months. Main Outcome Measures: Recurrence requiring external beam radiation therapy or enucleation; VA; and complications including iris atrophy, peripheral focal lens opacity, peripheral anterior synechiae, and foveal neovascular membrane. Results: Forty-four eyes of 33 patients were treated. Eyes were classified as Reese-Ellsworth group I (11 [2%]),11(6 [12%]),111(3 [7%]), IV (5 [9%]), or V (29 [67%]). Thirty-eight eyes (86%) had successful tumor control and avoided enucleation at a median follow-up of 36 months, At 3 years, tumor control rates were 100% for Reese-Ellsworth groups I to IV and 83% for group V. All eyes requiring enucleation were in Reese-Ellsworth group V. No eyes required external beam radiation. The most common complications were iris atrophy (61%) and focal lens opacity (14%). Strabismus was noted in 16% of eyes. Snellen VA measured 20/40 or better in 36% of eyes, 20/80 or better in 57%, and 20/400 or better in 86%. An increasing number of laser applications and chemotherapy cycles was not associated with decreased VA, strabismus, or development of an afferent pupillary defect but was associated with development of local complications. Conclusions: A unique approach to the application of diode laser therapy characterized by repetitive foveal treatments and aggressive chemotherapy resulted in tumor control rates that exceed those previously published. Furthermore, despite laser application directly to the fovea, 57% of patients retained 20/80 or better vision.