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BackgroundChemotherapy is increasingly used as primary treatment for group D retinoblastoma, whereas primary enucleation is considered to have a diminishing role. This study aimed to compare the management course, including number of examinations under anaesthesia (EUAs), of group D patients treated by enucleation versus chemotherapy.MethodsA retrospective analysis of 92 group D patients, of which 40 (37 unilateral) underwent primary enucleation and 52 (17 unilateral) were treated with intravenous chemotherapy. Number of EUAs was compared between the treatment groups with respect to the whole cohort, using univariate and multivariate analysis, and to unilateral cases only.ResultsPatients were followed up for a median of 61 months (mean: 66, range: 14–156), in which time primary enucleated patients had on average seven EUAs and chemotherapy-treated patients 21 EUAs (pConclusionGroup D patients’ families should be counselled regarding the significant difference in number of EUAs following primary enucleation versus chemotherapy when deciding on a treatment strategy. In this regard, primary enucleation would be most beneficial for older patients with unilateral disease.

Eye salvage rate for group D retinoblastoma using intravenous chemotherapy (IVC) as a primary modality is50%. To report on 13 years' experience with the use of primary IVC for group D retinoblastoma.A retrospective analysis of 64 group D eyes (52 patients) treated with primary IVC, from 2002 to 2014.The median age at presentation was 11.0 months (mean: 18.6, range: 0.6-144.0), 35 (67%) patients had bilateral disease, 38 (73%) germline disease and 8 (15%) cases were familial. In addition to IVC, patients received a median number of three treatments (mean: 6, range: 0-24), including thermotherapy/cryotherapy, plaque radiotherapy, intra-ophthalmic artery chemotherapy (IAC) and/or intravitreous chemotherapy. External beam radiotherapy (EBRT) was used in five eyes, all of which were eventually enucleated. In a median follow-up time of 55 months (mean: 64, range: 14-156), 63% of eyes were salvaged. By the Kaplan-Meier survival analysis, globe salvage rate was 83%, 70%, 59% and 45% at 1, 3, 5 and 10 years, respectively. There were no cases of metastatic spread from intraocular retinoblastoma and no deaths. IVC-related adverse events included febrile neutropenia in 21 (40%) patients and anaphylactic reaction to carboplatin in 2 (4%), all conservatively resolved. Of the patients receiving IAC, third and sixth nerve palsies were documented in two (10%) and one (5%) eyes, respectively.Primary IVC for group D eyes, with adjuvant treatments as required, was found to be a safe and efficient approach, achieving 63% eye salvage rate, no metastatic spread from intraocular retinoblastoma and no deaths. IAC has now replaced EBRT as a successful salvage treatment.

PurposeThe aim of this study was to investigate if there was a relationship between ethnicity or socioeconomic status and the presentation of advanced non-familial retinoblastoma in the UK.MethodsA cross sectional study based at the two centres providing retinoblastoma care in the UK. Non-familial cases of retinoblastoma (Rb) presenting between January 2006 and December 2011 were included. Data collected included: age at diagnosis, gender, child’s ethnicity, International Intraocular Retinoblastoma Classification (IIRC) stage with Groups D and E being considered advanced, laterality, treatment, and postcodes. Individual postcode (ZIP code) data was used to obtain the Index of Multiple Deprivation (IMD) score. A postal questionnaire was sent to participants’ parents to collect further, person-level, information on languages spoken and household socioeconomic position. Measures of severity of retinoblastoma also included: requirement for primary enucleation; the use of adjuvant chemotherapy; and mortality.Results189 cases were analyzed. 98 (52%) male and 91 (48%) female. Median age at diagnosis was 16 months [IQR 8 – 34 months]. 153/189 (81%) of cases presented with advanced retinoblastoma; 75 (40%) group E, 78 (41%) group D. 134 (72%) of cases were treated with enucleation.Multivariable analysis showed that older age at presentation was associated with enucleation and bilateral disease was associated with adjuvant chemotherapy. There was some indication that South Asian ethnicity and being in the most deprived IMD quintile were associated with a higher likelihood of presentation with advanced disease, but these estimates did not reach statistical significance.ConclusionsIn this first national UK study of patients with non-familial retinoblastoma, there was no evidence of an association of ethnicity or socio-economic status and the risk of presenting with advanced disease. This may reflect equality in access of health care in the UK. As a result, awareness campaigns should continue.

Background/aims To determine the frequency and cause of visual loss following intra-arterial melphalan (IAM) in patients with retinoblastoma with age appropriate vision. Methods Assessment of patients with refractory retinoblastoma that had undergone systemic chemotherapy, with or without local treatment, and were subsequently treated with IAM. Eyes of patients with a healthy foveola were assessed. The main outcome measures included visual, macular (including Pattern Visual Evoked Potentials and Fundus Fluorescein Angiography) and retinal functions (Electroretinograms). Results Five of twelve eyes (42%) demonstrated severe visual loss following IAM at last follow-up (median 21 months). This was due to either retinal detachment (1 eye, 20%) or choroidal ischaemia involving the foveola (4 eyes, 80%). All 3 eyes that had technical difficulties or vasospasm during catheterisation suffered visual loss. 8 out of 10 eyes that had a non-age adjusted dose of melphalan suffered visual loss. Electroretinograms post-IAM deteriorated in 4 of 8 eyes (50%) and Pattern Visual Evoked Potentials deteriorated in 3 (37%), though only one of these 3 showed concomitant visual acuity loss. Conclusions Structural and vascular damage to the foveola limited visual acuity. Complications associated with catheterisation and high doses of melphalan may be contributory factors to visual morbidity. Although visual loss is described, no patient developed metastases and most retained good vision.

Purpose To report the effect of intravitreal anti-vascular endothelial growth factor injections (IVI) on visual acuity in eyes with choroidal neovascularisation (CNVM) and co-existent vitreomacular traction (VMT) or when VMT has developed during the course of treatment. Methods Retrospective interventional case series of seven eyes in seven patients. VMT was monitored with serial optical coherence tomography scans. Results The mean age at presentation was 74 years (range 64–95 years). All patients presented with blurring of central vision, rather than distortion. The aetiology of CNVM was wet age-related macular degeneration in five eyes (72%), angioid streaks in one eye (14%) and pathological myopia in one eye (14%). Ranibizumab was used in four eyes (57%) and bevacizumab in three (43%) for the active CNVM component. The mean follow-up was 11 months (range 2–28 months). None of the eyes in this series required surgery for the VMT component, nor were there any cases of spontaneous resolution of VMT. Visual acuity was stabilised or improved in five of the seven eyes (71%) with IVI. Visual acuity results across the whole group were gain of three or more lines of Snellen visual acuity in two eyes (28%), gain of up to three lines in three eyes (42%), no change in visual acuity in one eye (14%) and loss of up to three lines in one eye (14%). There were no eyes losing more than three lines of Snellen visual acuity. In four eyes with pre-existing VMT, visual acuity improved in three with IVI. In three eyes that developed VMT after IVI, visual acuity improved in two with IVI. Delay from diagnosis of CNVM to treatment with IVI contributed to a poor response. Conclusions Most eyes improved visual acuity with IVI for combined CNVM and VMT. Despite the often dramatic features of VMT on optical coherence tomography, treatment of co-existing CNVM should be prompt. Vitreoretinal surgery was not required in this series, but is held in reserve if there is still potential for gain in vision following CNVM resolution.

Background: Temporalis fascia has been recommended for hydroxyapatite sphere exposure. The aim of this study was to identify potential risk factors for exposure of porous polyethylene (Medpor) sphere implants and evaluate the use of autogenous temporalis fascia as a patch graft for exposure. Methods: A retrospective review of consecutive cases of porous polyethylene sphere orbital implant exposure. Results: Five cases presented between May 2000 and October 2001 (three males, two females; mean age 44.5 years). Three had enucleation (two with primary implants) and two had evisceration (one with primary implant). Exposure occurred in one primary, two secondary, and two replacement implants. Orbital implant diameter was 20 mm in four cases and 16 mm in one case (contracted socket). The mean time from implantation to exposure was 23 months (range 0.7–42.6). Three patients had secondary motility peg placement before exposure. The average time from last procedure (sphere implant or peg insertion) to exposure was 3 months (range 0.7–12.6). Four patients required surgical intervention, of which three needed more than one procedure. Autogenous temporalis fascia grafting successfully closed the defect without re-exposure in three of these four patients. The grafts were left bare in three patients, with a mean time to conjunctivalise of 2.4 months (range 1.6–3.2). Conclusions: Exposed porous polyethylene sphere implants were treated successfully with autogenous temporalis fascia graft in three of four patients. This technique is useful, the graft easy to harvest, and did not lead to prolonged socket inflammation, infection, or extrusion.