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 aim was to report normative values of retinal nerve fiber layer (RNFL) and macular parameters in children using spectral domain optical coherence tomography (OCT) and to perform correlations with age, refractive error and axial length.MethodsThis was an observational cross-sectional study recruiting 113 healthy children aged 6 to 17 years with no ocular abnormality except refractive error. After a comprehensive eye examination and axial length measurement, RNFL and macular thickness measurements were performed using the Cirrus OCT machine. Main outcome measures were macular volume, macular thickness and RNFL thickness values as well as their correlations with age, refractive error and axial length. Right eyes of all subjects were selected for analysis.ResultsOne hundred and eight children were included in the study, 65 females and 43 males. Mean age was 10.7+/−3.1 years, average spherical equivalent refraction (SE) was −0.02+/−1.77(−4.25 to +5.00) diopters and average axial length was 23.5+/−1.0 (21.5 to 25.8)mm. Mean RNFL thickness was 95.6+/−8.7 μm, average macular thickness was 279.6+/−12.5 μm, central macular thickness was 249.1+/−20.2 μm, and mean macular volume was 10.1+/−0.5 mm3. Central macular thickness values were significantly higher in males (p < 0.001). RNFL measurements did not correlate with age but did show a positive correlation with SE. All macular parameters were consistently positively correlated with age and most of them were positively correlated with SE. When controlling for axial length, only the macular inner circle thickness was positively correlated with age.ConclusionsUsing Cirrus OCT, normative RNFL and macular parameters in healthy children below 18 years of age were established; measurements varied by age and gender.

Central macular thickness was significantly increased in anisometropic amblyopia using SD-OCT. Anisometropia alone did not produce such a difference, which points to a possible correlation between amblyopia and the development of the retinal layers.

Using enhanced high-definition SD-OCT, amblyopic eyes demonstrated qualitative and quantitative differences in macular features, possibly representing signs of immaturity compared with normal fellow eyes.

Normal variation in interocular symmetry exists. Our results can contribute to establishing a standard reference for interocular differences in OCT parameters in children aged 6-17 years using the Cirrus device.

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