Global Retinoblastoma Study Group IMPORTANCE Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale.OBJECTIVES To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. DESIGN, SETTING, AND PARTICIPANTSA total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. MAIN OUTCOMES AND MEASURESAge at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. RESULTSThe cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low-and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI,, and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI,). CONCLUSIONS AND RELEVANCEThis study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs.

BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease.ConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

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Background Bilateral cataract is a significant cause of blindness in children in Ethiopia. This study aimed to identify the resources available for cataract surgery in children, and to assess current surgical practices, surgical output and factors affecting the outcome of surgery in Ethiopia. Methods A Google Forms mobile phone questionnaire was emailed to nine ophthalmologists known to perform cataract surgery in young children (0–5 years). Results All nine responded. All but one had received either 12- or 3–5-month’s training in pediatric ophthalmology with hands-on surgical training. The other surgeon had received informal training from an experienced colleague and visiting ophthalmologists. The surgeons were based in seven health facilities: five in the capital (Addis Ababa) and eight in six public referral hospitals and one private center. Over 12 months (2017–2018) 508 children (592 eyes) aged 0–18 years (most < 15 years) were operated by these surgeons. 84 (17%) had bilateral cataract, and 424 (83%) had unilateral cataract mainly following trauma. A mean of 66 (range 18–145) eyes were operated per surgeon. Seventy-one additional children aged > 5 years were operated by other surgeons. There were substantially fewer surgeons per million population (nine for 115 million population) than recommended by the World Health Organization and they were unevenly distributed across the country. Methylcellulose and rigid intraocular lenses were generally available but less than 50% of facilities had a sharp vitrectomy cutter and cohesive viscoelastic. Mean travel time outside Addis Ababa to a facility offering pediatric cataract surgery was 10 h. Conclusion Despite the high number of cases per surgeon, the output for bilateral cataracts was far lower than required. More well-equipped pediatric ophthalmology teams are urgently required, with deployment to under-served areas.

Background: Bilateral cataract is a significant cause of blindness in Ethiopia. This study aimed to identify the resources available for cataract surgery in children, and to assess current surgical practices, surgical output and factors affecting the outcome of surgery in Ethiopia. Methods: A Google Forms mobile phone questionnaire was emailed to nine ophthalmologists known to perform cataract surgery in young children.Results: All nine responded. All but one had received either 12- or 3-5-month’s training in pediatric ophthalmology with hands-on surgical training. The other surgeon had received informal training from an experienced colleague and visiting ophthalmologists. Five worked in the capital, Addis Ababa, and they worked in six public referral hospitals and one private center. Over 12 months (2017-2018) 508 children underwent surgery; 84 (17%) children had bilateral and 424 (83%) had unilateral cataract, mainly following trauma (mean 66 (range 18-145 standard deviation (SD) ±47) eyes/surgeon). There were substantially fewer surgeons per million population (nine for 115 million population) than recommended by the World Health Organization and they were unevenly distributed across the country.Methylcellulose and rigid intraocular lenses were generally available but less than 50% of facilities had a sharp vitrectomy cutter and cohesive viscoelastic. Mean travel time outside Addis Ababa to a facility offering pediatric cataract surgery was 10 hours. Conclusion: Despite the high number of cases per surgeon, the output for bilateral cataracts was far lower than required. More well-equipped ophthalmologists trained in pediatric cataract surgery are urgently required, with deployment to under-served areas.