Retinoblastoma, the most common eye cancer of childhood, is fatal if left untreated. Prognosis of patients with retinoblastoma in high-income countries (HICs) has improved over the past 50 years, now reaching a near 100% disease-free survival rate.^1-3 This is attributed to several factors, including (1) creation of specialized referral centers, (2) decoding of the genetic basis of the disease, (3) formation of screening programs, and (4) the introduction of chemotherapy.⁴ In HICs, retinoblastoma is a curable disease, and attention has now shifted to eye salvage^5,6 and improvement of quality of life.⁷ In low- and middle-income countries (LMICs), where more than 80% of global retinoblastoma cases arise, the prognosis is poor, and it is assumed that this is because of delayed diagnosis and treatment.^8-10 Publications from LMICs are scarce, and many countries do not report their retinoblastoma data.¹¹ The stage of retinoblastoma at the time of diagnosis in low-income, middle-income, and high-income countries has not been surveyed globally. This information is important for policy and health care planning at national and international levels.

Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. The cohort included 4064 children from 149 countries. The median age at diagnosis was 23·2 months (IQR 11·0–36·5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0·8%) of 636 children from high-income countries, 55 (5·4%) of 1027 children from upper-middle-income countries, 342 (19·7%) of 1738 children from lower-middle-income countries, and 196 (42·9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98·8%) of 4064 children. The 3-year survival rate was 99·5% (95% CI 98·8–100·0) for children from high-income countries, 91·2% (89·5–93·0) for children from upper-middle-income countries, 80·3% (78·3–82·3) for children from lower-middle-income countries, and 57·3% (52·1-63·0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16·67; 95% CI 4·76–50·00), cT4 advanced tumour compared to cT1 (8·98; 4·44–18·18), and older age at diagnosis in children up to 3 years (1·38 per year; 1·23–1·56). For children aged 3–7 years, the mortality risk decreased slightly (p=0·0104 for the change in slope). This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes. Queen Elizabeth Diamond Jubilee Trust.

To report a series of full-thickness macular holes (MH) that developed after pars plana vitrectomy for rhegmatogenous retinal detachment (RRD). A retrospective review of the demographic and medical data, including optical coherence tomography studies of 6 patients (7 eyes) who underwent pars plana vitrectomy for RRD and subsequently developed MH. There were 4 women and 2 men with a mean age of 60 ± 10 years (range, 50-79 years). The mean interval between RRD repair and MH formation was 20 ± 18 months (range, 1-48 years). Five eyes underwent surgery to repair the MHs, four of which were closed at the last follow-up. Two eyes were not operated, one because of unfavorable prognosis, and in the other, which presented with an epiretinal membrane before the development of the MH, the hole closed spontaneously. In all operated cases, the inner limiting membrane was peeled during MH surgery. The mean logarithm of the minimal angle of resolution was 0.73, 0.53, 0.77, and 0.57 at RRD presentation, after surgical repair of RRD and before MH diagnosis, at MH presentation, and at last follow-up, respectively. The study subjects accounted for 1.1% of our 609 patients who underwent pars plana vitrectomy for primary RRD during the study period. Macular hole may develop after pars plana vitrectomy for RRD, and these cases can be surgically repaired. The pathogenesis of the formation of these holes remains unclear.