Global Retinoblastoma Presentation and Analysis by National Income Level.

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 Participants: A 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 Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The 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, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This 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 global retinoblastoma outcome study : a prospective, cluster-based analysis of 4064 patients from 149 countries

DATA SHARING : The study data will become available online once all analyses are complete.BACKGROUND : 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. METHODS : 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. FINDINGS : 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). INTERPRETATION : 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.The Queen Elizabeth Diamond Jubilee Trust and the Wellcome Trust.https://www.thelancet.com/journals/langlo/homeam2023Paediatrics and Child Healt

Fundus imaging with a nasal endoscope

Wide field fundus imaging is needed to diagnose, treat, and follow-up patients with retinal pathology. This is more applicable for pediatric patients as repeated evaluation is a challenge. The presently available imaging machines though provide high definition images, but carry the obvious disadvantages of either being costly or bulky or sometimes both, which limits its usage only to large centers. We hereby report a technique of fundus imaging using a nasal endoscope coupled with viscoelastic. A regular nasal endoscope with viscoelastic coupling was placed on the cornea to image the fundus of infants under general anesthesia. Wide angle fundus images of various fundus pathologies in infants could be obtained easily with readily available instruments and without the much financial investment for the institutes

Brachytherapy, A viable option of globe salvage in treatment of large ciliary body melanocytoma

We report a case of large histopathologically proven melanocytoma of the ciliary body in a 15-year-old male, presented with rapid extraocular growth following incisional biopsy with scleral patch graft. We chose brachytherapy with Ruthenium 106 plaque over enucleation as the later was refused by the parents. The initial apical height of the tumor was 14.2 mm on ultrasonography. Two weeks after brachytherapy, the mass regressed to a size of 8.1 mm and 1 year later to 6.7 mm. This is the first case report showing the response of brachytherapy to ciliary body melanocytoma, which results in ocular and visual acuity salvation with considerable decreased in size of the tumor. The authors conclude that brachytherapy is an option in the management of non-resectable melanocytoma of the ciliary body

Creatively tiding over the crisis

Background: A patient with a retained intraocular metallic foreign body post-trauma was taken up for vitrectomy and intraocular foreign body removal. Unfortunately, the intraocular magnet was not available at the moment on the table! How a little bit of creativity and innovative thought helped us tide over this crisis is the content of this video. Purpose: To demonstrate magnetization of a metallic surgical instrument for temporary use in the event of unavailability of the intraocular magnet for intraocular foreign body removal. Synopsis: A ferromagnetic substance can be magnetized temporarily using an existing magnet. We obtained a general-purpose magnet and wrapped it in sterile plastic, using which we magnetized normal intraocular forceps and a Micro Vitreo Retinal (MVR) blade by giving about 20–30 strokes over the magnet in a single direction. This aligned the magnetic domains in the metal in a parallel fashion. These Do It Yourself (DIY)- magnetic instruments were then effectively utilized to remove the metallic intraocular foreign body. Highlights: The video showcases effectively harnessing the available resources and tiding over the dearth of a necessary instrument, with the right use of an innovative idea and some creativity!. Video link: https://youtu.be/QtRC-AK5FLU

Novel techniques in scleral buckling

Scleral buckling is a surgical technique that is employed successfully to treat rhegmatogenous retinal detachments (RRD) for more than 60 years. With the introduction of pars plana vitrectomy (PPV), there is a growing trend towards the use of PPV for treatment of retinal detachment. There is a reluctance to perform scleral buckling (SB) in RRD due to the perceived steep learning curve, declining mastery over indirect ophthalmoscopy, and poor ergonomics associated with SB. In this article, we discuss the surgical challenges and tips to overcome these in four headings: localization of the break, retinopexy, SB, and subretinal fluid (SRF) drainage. Localization of the break can be performed by the use of forceps or illuminated scleral depressor. It can be facilitated by prior drainage of SRF in cases with bullous RRD. Chandelier with wide-angle viewing system can be used for easier localization of break and cryopexy. Sutureless buckling and suprachoroidal buckling are easier and faster alternatives to the conventional technique. Reshaping the silicone segment helps in accommodating the wider circumferential band. Modified needle drainage, laser choroidotomy, and infusion-assisted drainage can make SRF drainage easier and safer. The above techniques and other practical tips have been explained in detail with the illustrations to make the process of learning the art of SB easier

Therapeutic surprise! Photodynamic therapy for cavernous haemangioma of the disc

Purpose: To report the response of cavernous haemangioma of the disc to PDT. Methods and Patients: A 32 years old lady presented to us with complaints of sudden onset of blurry vision in her right eye. What seemed initially as polypoidal choroidal vasculopathy turned out as cavernous haemangioma of the disc after pneumatic displacement of the sub-retinal haeme. She was treated with PDT as she was prone to recurrent haemorrhage from the lesion due to her low platelet count. Results: Complete regression of the cavernous haemangioma was noted as early as the second week itself and remained regressed for 4 months. Conclusion: PDT can be safe option for patients presenting with symptomatic cavernous haemangioma of the disc or retina

Sutureless scleral buckle in the management of rhegmatogenous retinal detachment

Purpose: To evaluate the anatomical and functional outcomes of sutureless scleral buckling for the repair of rhegmatogenous retinal detachment (RD). Design: Retrospective interventional case series. Materials and Methods: Retrospective analysis of 50 eyes of 49 patients with rhegmatogenous RD, who underwent sutureless scleral buckling from January 2009 to March 2013. Results: Primary retinal re-attachment rate of 86% was achieved with single surgery, but final anatomical success was 94% with additional interventions in the form of intravitreal gas, buckle revision, and/or pars plana vitrectomy. Best corrected logarithm of minimum angle of resolution visual acuity improved from 1.44 ± 1.01 preoperatively to 0.50 ± 0.40 at a mean follow-up of 6.7 months.XS Conclusion: Sutureless scleral buckling achieves excellent anatomical and functional success in majority of the patients with rhegmatogenous RD