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.

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Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP) of 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p<0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. All subtypes, including primary glaucoma, were sporadic. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.

Purpose: The aim was to report long-term surgical success of primary congenital glaucoma (PCG) patients in Thailand. Materials and Methods: PCG patients who underwent one of the following primary operations: trabeculotomy, goniotomy, trabeculectomy, combined trabeculotrabeculectomy (CTT) and diode transscleral cyclophotocoagulation (TSCPC) between January 1992 and January 2018 were reviewed. Surgical success was defined as intraocular pressure (IOP) between 5 and 21 mm Hg with or without antiglaucoma medications. Failure was defined as IOP ≤5 or ≥21 mm Hg for 2 consecutive visits, or when an additional glaucoma surgery was required to control IOP. Survival curves were analyzed using multilevel mixed-effect Weibull model. Results: A total of 81 eyes from 55 PCG patients were included. Surgical procedures involved 20 goniotomies, 15 trabeculotomies, 16 trabeculectomies, 15 CTT, and 15 TSCPC. Median follow-up time was 24 months (interquartile range: 9 to 60 mo). Overall success rates were 68.8% at 1 year, 63.8% at 3 years, and 53.7% at 5 years. All types of surgery except TSCPC had comparable cumulative 1 year success rates ranging from 78.5% to 83.3%. Cumulative success rates of trabeculotomy (80.05%) and CTT (79.4%) were maintained at 3 and 5 years and were the highest among all procedures at 5 years. TSCPC had a significantly lower success rate compared with other types of surgery (hazard ratio: 7.4 to 13.1, all P=0.01). All patients receiving primary TSCPC showed no success at 48 months. Conclusion: Primary trabeculotomy and primary CTT demonstrated the highest long-term success rates in PCG patients.

Purpose To evaluate the correlation between clinical presentations, radiological findings and high risk histopathological features of primary enucleated eyes in patients with advanced retinoblastoma at a tertiary child hospital in Thailand. Materials and methods We retrospectively reviewed the medical records of patients who were treated with primary enucleation of tumor eyes between 2015–2020. Demographic data, radiological assessment, and histopathological findings were collected. The association between clinical presentations and high-risk histopathological features in the primary enucleated eyes were evaluated. The radiological findings, which showed characteristic of high risk features, were compared with the histopathological findings. Results Thirty-three enucleated eyes were enrolled in this study. The mean age at diagnosis was 23.12 months. Most patients had unilateral group E retinoblastoma, with no difference in sex and laterality of the eye. Leukocoria was the most common presentation, followed by proptosis and uveitis. Older age at presentation were statistically associated with post laminar cribrosa optic nerve invasion (P-value 0.0027) and high-risk histopathological features in enucleated eyes (P-value 0.0032). Clinical presentations with proptosis were statistically associated with post laminar cribrosa optic nerve invasion, while leukocoria and uveitis were statistically associated with anterior segment invasion. Unifocal intraocular mass with necrosis was the most common histopathological finding. High-risk features were found in 45% of primary enucleated eye. The sensitivity and specificity of magnetic resonance imaging (MRI) for detected optic nerve invasion in retinoblastoma patients were 75% and 54%, respectively. Conclusion Patients with unilateral retinoblastoma who presented with older age related to high-risk features after enucleation. Ophthalmic examination with slitlamp is the best way for detection of anterior segment invasion. Choroidal invasion was unable to predict with clinical presentation. MRI was the better imaging for detection of post laminar cribrosa optic nerve invasion.

Baerveldt implantation is an effective procedure with high success rate. HP was common after Baerveldt implantation. Preoperative IOP and numbers of previous glaucoma surgery are risk factors for developing HP. Presence of HP can predict whether long-term anti-glaucoma medication(s) are needed or not.

Purpose To study the prevalence of the prescription glasses given to first-grade students due to the “Good Sight for Thai Children” (GSTC) policy. Methods This was a cross-sectional study that examined all prescription glasses given to first-grade students throughout Thailand, after visual screening due to the GSTC policy between 2016 and 2017. Trained class teachers screened their students’ visual acuity and referred children who had less than 20/40 visual acuity in either eye to a hospital for an eye examination and prescription glasses. Results A total of 786,729 students were screened. Of these, 20,401 (2.59%) students were referred to hospital. However, only 9867 (48.37%) students presented to a hospital. Glasses were prescribed for 5324 (53.96%) students following cycloplegic refraction by ophthalmologists or trained refractionists. The mean spherical equivalent was −1.08 (−19.00 to +10.00, SD 2.32) diopters. There were 1626 (30.54%) children at amblyopic risk without glasses. A 5.49% had high myopia (< −6 diopters), 5.22% had high hyperopia (> +5 diopters) and 27.82% had high astigmatism (>2 diopters). A cylindrical lens analysis showed that 81.53% had with-the-rule astigmatism, 4.07% had against-the-rule astigmatism, and 14.40% had oblique astigmatism. Conclusion Although the reliable prevalence of refractive error cannot be estimated, the prevalence of visual impairment may be estimated. There were a number of students who required glasses. The astigmatism was the most common refractive error on prescription glasses for first-grade children. With-the-rule astigmatism was the most prevalent. The visual screening program of school children proved to be valuable and should be continued and developed.

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