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.

The incidence of syphilis and syphilitic uveitis in our community is increasing. The prevalence of associated neurosyphilis is unknown, and it remains unclear whether syphilitic uveitis should be treated as secondary syphilis with intramuscular penicillin or neurosyphilis with intravenous penicillin. The (English language) literature was reviewed for all unique cases of syphilitic uveitis reported from 1984 to June 2008. For each case the following data were recorded: the clinical features of the syphilis, the uveitis and any associated neurosyphilis, the human immunodeficiency virus (HIV) status, lumbar puncture findings, treatment and follow up. We identified 143 patients in 41 original reports of syphilitic uveitis (93 HIV-positive and 50 HIV-negative). Posterior uveitis was reported in 79 patients (55.2%); panuveitis was reported in 36 patients (25.2%); anterior/intermediate uveitis was reported in only 28 patients (19.6%). Lumbar puncture findings were abnormal in 82 patients (57%), and the majority of these patients (76%, 62 out of 82) were HIV-positive. One hundred and ten (77%) patients were treated with intravenous therapy, usually penicillin. Most recovered from the syphilis, however, a proportion did not recover full vision. There were 13 (9%) treatment failures, which tended to occur in patients who were HIV-positive (n = 11), had abnormal lumbar puncture findings (n = 8) and/or were treated (n = 11) intravenously. There is a high incidence of abnormal lumbar puncture findings in patients with syphilitic uveitis and a strong association with HIV infection. Most received appropriate therapy with a low relapse rate, which was not related to the type of therapy.

No abstract

Purpose To validate a table of amounts of three horizontal muscle surgery in patients with large-angle infantile esotropia (Z60 prism dioptres, PD). Methods A prospective interventional case series reporting the postoperative alignment of 51 patients (27 male, 24 female) over a 15-year period was conducted. Surgery amounts were according to a published table developed on a previous patient cohort (n ¼ 49), using bilateral medial rectus recession with graded unilateral lateral rectus resection. Kaplan-Meier lifetable survival curves were formulated for success to orthotropia ( ± 10 PD) after one and subsequent horizontal muscle surgeries for up to 8 years follow-up. Results The median preoperative deviation was 65 PD (range 60-80 PD) and median age at surgery was 11.8 months (range 5.1 months-3.6 years). Surgical success to orthotropia ( ± 10 PD) after one surgery was 100% at 2 months, 95.7% at 6 months, 91.3% at 12 months, 77.8% at 4 years, and 73.6% at 8 years. Postoperative failure requiring further horizontal surgery occurred in 17.6% (residual esotropia 4, consecutive exotropia 5). Conclusions Our second cohort has reproduced the success rate of the previous cohort (77.8% vs 77.1% at 4 years). If the published table of surgical amounts is used, three horizontal muscle surgery in large-angle infantile esotropia (Z60 PD) appears to have a good long-term success rate, and does not lead to the high rates of either residual esotropia or consecutive exotropia reported by others in the literature.

JIA may be associated with ongoing uveitis and complications in adulthood.