Conservative ocular salvage approaches

Conservative ocular salvage approaches, such as systemic chemotherapy and local-control treatments, may be offered in an attempt to save the eye and preserve vision.[9] Ocular salvage rates correlate with intraocular grouping. The possibility of saving the eye without the use of external-beam radiation therapy (EBRT) exceeds 80% for children with early intraocular disease. However, the ocular outcomes for children with advanced intraocular disease are poor using systemic chemotherapy and local treatments, with less than 40% ocular salvage rates, even after the use of EBRT.[10] Plaque brachytherapy has been used as a salvage therapy for patients with unilateral retinoblastoma. In one series, 12 eyes in 12 children were treated with ruthenium plaque brachytherapy. Globe salvage was achieved in 75% of the patients. The ultimate local control rate was 66%.[11]

Caution must be used when delaying enucleation by extending treatment with systemic chemotherapy when tumor control does not appear to be possible, particularly for Group E eyes. Pre-enucleation chemotherapy for eyes with advanced intraocular disease may result in downstaging and underestimate the pathological evidence of extraretinal and extraocular disease, thus increasing the risk of dissemination.[12]

The delivery of chemotherapy via ophthalmic artery cannulation as initial treatment for advanced unilateral retinoblastoma appears to be more effective than systemic chemotherapy for chemoreduction, particularly for Group D eyes.[13,14]; [15][Level of evidence C3] In a multidisciplinary state-of-the-art center, intra-arterial chemotherapy to treat patients with advanced intraocular unilateral retinoblastoma may result in ocular salvage rates of approximately 70% to 90%.[14-18] For more information, see the Intra-Arterial Chemotherapy (Ophthalmic Artery Infusion of Chemotherapy) section.

Electroretinography, a technique that measures the electrical responses of various cell types in the retina, including the photoreceptors, can be used to assess retinal function during and after treatment with intra-arterial chemotherapy. In one study, pretreatment electroretinography correlated with final visual acuity after treatment with intra-arterial chemotherapy, suggesting that this technique may potentially be used to help with treatment strategy decisions and prioritization of interventions.[19]