Publications by authors named "Ido Didi Fabian"

60 Publications

Sex, gender, and retinoblastoma: analysis of 4351 patients from 153 countries.

Eye (Lond) 2021 Jul 16. Epub 2021 Jul 16.

International Centre for Eye Helath, London School of Hygiene & Tropical Medicine, London, UK.

Objective: To investigate in a large global sample of patients with retinoblastoma whether sex predilection exists for this childhood eye cancer.

Methods: A cross-sectional analysis including 4351 treatment-naive retinoblastoma patients from 153 countries who presented to 278 treatment centers across the world in 2017. The sex ratio (male/female) in the sample was compared to the sex ratio at birth by means of a two-sided proportions test at global level, country economic grouping, continent, and for selected countries.

Results: For the entire sample, the mean retinoblastoma sex ratio, 1.20, was higher than the weighted global sex ratio at birth, 1.07 (p < 0.001). Analysis at economic grouping, continent, and country-level demonstrated differences in the sex ratio in the sample compared to the ratio at birth in lower-middle-income countries (n = 1940), 1.23 vs. 1.07 (p = 0.019); Asia (n = 2276), 1.28 vs. 1.06 (p < 0.001); and India (n = 558), 1.52 vs. 1.11 (p = 0.008). Sensitivity analysis, excluding data from India, showed that differences remained significant for the remaining sample (χ = 6.925, corrected p = 0.025) and for Asia (χ = 5.084, corrected p = 0.036). Excluding data from Asia, differences for the remaining sample were nonsignificant (χ = 2.205, p = 0.14).

Conclusions: No proof of sex predilection in retinoblastoma was found in the present study, which is estimated to include over half of new retinoblastoma patients worldwide in 2017. A high male to female ratio in Asian countries, India in specific, which may have had an impact on global-level analysis, is likely due to gender discrimination in access to care in these countries, rather than a biological difference between sexes.
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http://dx.doi.org/10.1038/s41433-021-01675-yDOI Listing
July 2021

Lag Time between Onset of First Symptom and Treatment of Retinoblastoma: An International Collaborative Study of 692 Patients from 10 Countries.

Cancers (Basel) 2021 Apr 19;13(8). Epub 2021 Apr 19.

Department of Ophthalmology, University of Washington, Seattle, WA 98195, USA.

Background: The relationship between lag time and outcomes in retinoblastoma (RB) is unclear. In this study, we aimed to study the effect of lag time between onset of symptoms and diagnosis of retinoblastoma (RB) in countries based on their national-income and analyse its effect on the outcomes.

Methods: We performed a prospective study of 692 patients from 11 RB centres in 10 countries from 1 January 2019 to 31 December 2019.

Results: The following factors were significantly different among different countries based on national-income level: age at diagnosis of RB ( = 0.001), distance from home to nearest primary healthcare centre ( = 0.03) and mean lag time between detection of first symptom to visit to RB treatment centre ( = 0.0007). After adjusting for country income, increased lag time between onset of symptoms and diagnosis of RB was associated with higher chances of an advanced tumour at presentation ( < 0.001), higher chances of high-risk histopathology features ( = 0.003), regional lymph node metastasis ( < 0.001), systemic metastasis ( < 0.001) and death ( < 0.001).

Conclusions: There is a significant difference in the lag time between onset of signs and symptoms and referral to an RB treatment centre among countries based on national income resulting in significant differences in the presenting features and clinical outcomes.
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http://dx.doi.org/10.3390/cancers13081956DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8073369PMC
April 2021

Effect of polysaccharide on human retinoblastoma Y79 cell proliferation and apoptosis.

Int J Ophthalmol 2021 18;14(4):497-503. Epub 2021 Apr 18.

Department of Ophthalmology, Fuling Central Hospital, Chongqing 408000, China.

Aim: To explore the effect of the () polysaccharide on the proliferation and apoptosis of human retinoblastoma (RB) Y79 cells and its mechanism.

Methods: The refined polysaccharide was obtained using techniques such as water extraction, ethanol precipitation, and decompression concentration. The inhibition effect of the polysaccharide on the proliferation of Y79 cells was detected by cell proliferation assay. Flow cytometry was used for the detection of cell apoptosis rate and cycle change. Real-time qunatitative polymerase chain reaction (RT qPCR)and Western blotting were used to detect the expression of cell apoptosis signal pathway-related factors (caspase-3, caspase-8, and caspase-9) and cell cycle signal pathway-related factors (CDK1 and cyclinB1) at the transcriptional and translational levels.

Results: Infrared and ultraviolet spectrum scanning showed that the extracted drug was a polysaccharide with high purity. After being treated with different concentrations of polysaccharide for different periods of time, the Y79 cells showed different degrees of proliferation inhibition. Flow cytometric observations showed that the cell apoptosis rate and the proportion of cells blocked in the G2/M phase were significantly increased after polysaccharide treatment. Further analysis revealed that the mRNA and protein expression of caspase-3, caspase-8, and caspase-9 in the polysaccharide treatment groups increased significantly compared with that in the control groups, while the expression of CDK1 and cyclinB1 decreased significantly.

Conclusion: The polysaccharide could inhibit the proliferation and induce apoptosis of Y79 cells. Its possible mechanism is the upregulation of caspase-3, caspase-8, and caspase-9 expression in the cell apoptotic signaling pathway and the downregulation of CDK1 and cyclinB1 expression in the cell cycle signaling pathway.
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http://dx.doi.org/10.18240/ijo.2021.04.03DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8025161PMC
April 2021

Knowledge, attitude and practice of Ethiopian pediatricians concerning childhood eye diseases.

BMC Ophthalmol 2021 Feb 17;21(1):91. Epub 2021 Feb 17.

Jimma University, Jimma, Ethiopia.

Background: Eye examination and vision assessment are vital for the detection of conditions that result in blindness. Childhood blindness seriously impacts the development, education, and future employment opportunities of affected children. Pediatricians' knowledge of eye diseases is critical for the prevention of blindness through early diagnosis, allowing proper treatment and identification of conditions requiring referral to an ophthalmologist to preserve or restore vision. This study aimed to assess the knowledge, attitude, and practice of Ethiopian pediatricians concerning childhood eye diseases.

Methods: We carried out a cross-sectional descriptive study of pediatricians working in various hospitals and clinics in Ethiopia. Participants were selected via a convenient sampling technique. Data were collected using both closed and open-ended semi-structured questionnaires. Responses were entered into EpiData 3.1 and transferred to SPSS version 21.0 software for analysis.

Results: A total of 79 pediatricians participated in the study. Our findings showed that the attitude of all but 2 participants towards improving the management of childhood eye diseases was positive, even though this was not reflected in actual knowledge or practice. Even though attitudes were positive, knowledge was often poor and practice inadequate owing to barriers such as inadequate undergraduate training, lack of ophthalmology options during pediatric residency, and unavailability of ophthalmic equipment.

Conclusions: Participants' attitudes towards improving treatment for childhood eye diseases are positive, but their insufficient knowledge of eye diseases makes their practice poor in this respect.
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http://dx.doi.org/10.1186/s12886-021-01842-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7890964PMC
February 2021

Non-Invasive Low Pulsed Electrical Fields for Inducing BBB Disruption in Mice-Feasibility Demonstration.

Pharmaceutics 2021 Jan 27;13(2). Epub 2021 Jan 27.

The Advanced Technology Center, Sheba Medical Center, Tel Hashomer, Ramat-Gan 5262000, Israel.

The blood-brain barrier (BBB) is a major hurdle for the treatment of central nervous system disorders, limiting passage of both small and large therapeutic agents from the blood stream into the brain. Thus, means for inducing BBB disruption (BBBd) are urgently needed. Here, we studied the application of low pulsed electrical fields (PEFs) for inducing BBBd in mice. Mice were treated by low PEFs using electrodes pressed against both sides of the skull (100-400 square 50 µs pulses at 4 Hz with different voltages). BBBd as a function of treatment parameters was evaluated using MRI-based treatment response assessment maps (TRAMs) and Evans blue extravasation. A 3D numerical model of the mouse brain and electrodes was constructed using finite element software, simulating the electric fields distribution in the brain and ensuring no significant temperature elevation. BBBd was demonstrated immediately after treatment and significant linear regressions were found between treatment parameters and the extent of BBBd. The maximal induced electric field in the mice brains, calculated by the numerical model, ranged between 62.4 and 187.2 V/cm for the minimal and maximal applied voltages. These results demonstrate the feasibility of inducing significant BBBd using non-invasive low PEFs, well below the threshold for electroporation.
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http://dx.doi.org/10.3390/pharmaceutics13020169DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7911365PMC
January 2021

Incidence of Retinoblastoma Has Increased: Results from 40 European Countries.

Ophthalmology 2021 Jan 26. Epub 2021 Jan 26.

International Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United Kingdom; Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Tel-Aviv University, Tel-Aviv, Israel.

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http://dx.doi.org/10.1016/j.ophtha.2021.01.024DOI Listing
January 2021

International travel to obtain medical treatment for primary retinoblastoma: A global cohort study.

Int J Cancer 2021 04 31;148(8):1858-1866. Epub 2020 Oct 31.

International Centre for Eye Health, London School of Hygiene and Tropical Medicine, London, UK.

Early diagnosis and treatment of retinoblastoma (Rb), the most common intraocular malignancy, can save both the child's life and vision. However, access to services and hence chances for survival and preserving the eye and its vision vary widely across the globe. Some families have to, or make a choice to, leave their home country to seek planned medical treatment abroad. We aimed to investigate how frequently this cross-border travel occurs and the factors associated with it. A total of 278 Rb centres in 153 countries were recruited to participate in a global cross-sectional analysis of newly diagnosed Rb patients in 2017. Number and proportions of children who travelled from their home country for treatment were analysed by country, continent, socioeconomic stratum and clinical and demographic features. The cohort included 4351 new patients of whom 223 [5.1%, 95% confidence interval 4.5-5.8] were taken across country borders for planned medical treatment. Independently significant predictors of travelling across borders included: being from a country with a smaller population, being from a country classified as low socioeconomic status, having bilateral Rb and having intraocular disease without extraocular spread. The factors that determine international travel for Rb treatment are complex and deserve further investigation. We may need to rethink the way services are delivered in the light of the threat of severe curtailment of international travel from pandemics like corona virus disease 2019.
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http://dx.doi.org/10.1002/ijc.33350DOI Listing
April 2021

The distribution of relaxin receptors in the anterior segment of primary open-angle glaucoma patients.

Indian J Ophthalmol 2020 10;68(10):2117-2120

The Sam Rothberg Glaucoma Center, Goldschleger Eye Institute, Sheba Medical, Ramat Gan; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Purpose: Relaxin is a polypeptide hormone produced by the corpus luteum and the decidua in females and by the prostate in males. It has vasodilatory and antifibrotic effects. In the past, it has been reported that injection of relaxin hormone has caused a reduction in the intraocular pressure although its mechanism has not yet been fully understood and the expression of relaxin receptors has not yet been evident in the human eye. Therefore, the purpose of this current study was to examine the presence of relaxin receptors in the human eye anterior segment.

Methods: This is an interventional non-randomized study. Patients with primary open-angle glaucoma who underwent trabeculectomy were recruited. During the surgery, a punch biopsy (including the cornea, sclera, trabecular meshwork, and Schlemm's canal) was taken and stained with hematoxylin-eosin, and immunohistochemistry staining for relaxin/insulin-like family peptide receptor 1 (RXFP1).

Results: A positive staining was noted for RXFP1 in the inner uveal, corneoscleral, and cribriform meshwork and Schlemm's canal's endothelium. Negative staining for RXFP1 was noted in the cornea and sclera.

Conclusion: RXFP1 is present in the anterior segment drainage system of the human eye. Therefore, this receptor may have an active role in regulating the outflow facility and in the reduction of intraocular pressure.
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http://dx.doi.org/10.4103/ijo.IJO_2008_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7728022PMC
October 2020

Adjuvant use of laser in eyes with macular retinoblastoma treated with primary intravenous chemotherapy.

Br J Ophthalmol 2020 Sep 15. Epub 2020 Sep 15.

Royal London Hospital, London, London, UK.

Background: Adjuvant use of laser with systemic chemotherapy for treatment of retinoblastoma may reduce recurrence rates while also causing local side effects. Information is lacking on the effect of laser on visual outcomes.

Methods: A retrospective review of two retinoblastoma centres in the United Kingdom was conducted. Patients were included if there was a macular tumour in at least one eye. Eyes that received chemotherapy alone were compared with eyes that received chemotherapy plus adjuvant laser.

Results: A total of 76 patients and 91 eyes were included in the study. Systemic chemotherapy alone was used in 71 eyes while chemotherapy plus laser was used in 20 eyes. Demographic characteristics of both groups were similar. Macular relapse rates were similar between groups: 22/71 (31%) eyes in chemotherapy group and 9/20 (45%) eyes in laser group (p=0.29). There was no increase in vitreous relapses in the laser group (2/20 eyes), compared with the chemotherapy group 10/71 eyes (p=0.99). Survival analysis demonstrated similar time to first relapse between groups. Final visual acuity was equal between groups with 6/15 or better present in 31.1% of eyes in the chemotherapy group and 37.5% of eyes in the laser group (p=0.76). Presence of tumour at the fovea was predictive of final visual acuity, regardless of treatment group.

Conclusion: Adjuvant laser in the treatment of retinoblastoma is safe and does not lead to increased rate of vitreous recurrence. Final visual acuity is determined by the presence of tumour at the fovea and not the use of laser.
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http://dx.doi.org/10.1136/bjophthalmol-2020-316862DOI Listing
September 2020

Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries.

Br J Ophthalmol 2020 Sep 15. Epub 2020 Sep 15.

Pediatric Oncology Unit, Hospital Universitario y Politécnico La Fe, Valencia, Spain.

Background: The 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.

Methods: A 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.

Results: Capture 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.

Conclusions: Fewer 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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http://dx.doi.org/10.1136/bjophthalmol-2020-316613DOI Listing
September 2020

Long-term Outcomes of Small Pigmented Choroidal Melanoma Treated with Primary Photodynamic Therapy.

Ophthalmol Retina 2021 May 2;5(5):468-478. Epub 2020 Sep 2.

Ocular Oncology Service, Moorfields Eye Hospital, London, United Kingdom; NIHR Biomedical Research Centre for Ophthalmology at Moorfields Eye Hospital and University College London Institute of Ophthalmology, London, United Kingdom. Electronic address:

Purpose: To report the long-term outcomes of patients with small, pigmented, posteriorly located choroidal melanoma undergoing primary treatment using photodynamic therapy (PDT) with verteporfin at the London Ocular Oncology Service.

Design: Retrospective, interventional, consecutive case series.

Participants: All patients undergoing primary treatment using PDT with verteporfin from April 2014 to December 2015 and followed until December 2019.

Methods: This is a long-term follow-up study of the same cohort of patients previously reported by our group in 2017 and 2018.

Main Outcome Measures: Local tumor control, visual outcomes, and metastasis-free survival.

Results: Twenty-six patients were included with a mean (± standard deviation) age and tumor thickness of 62 ± 14 years and 1.3 ± 0.5 mm, respectively. Tumors were posteriorly located (mean distance to optic nerve and fovea = 2.0 ± 2.2 mm and 1.6 ± 1.5 mm, respectively), and the majority were fully pigmented (73%). Overall, patients were followed for a median (interquartile range [IQR], range) of 49.5 (15.3, 7.0-66.0) months from first PDT to last follow-up. Over the course of this study, 14 of 26 (54%) have developed a local recurrence at a median of 20.0 months (20.5, 4.7-60.9 months). The most common pattern of recurrence was an isolated increase in basal dimensions (9/14; 64%). Median (IQR) final logarithm of the minimum angle of resolution visual acuity of the whole cohort was 0.2 (0.5). The only statistically significant difference in baseline and outcome characteristics between treatment failures and nonfailures was the distance to the fovea (median [IQR], 0.5 [1.3] vs. 2.5 [2.8]; P = 0.002) and final logarithm of the minimum angle of resolution visual acuity (median [IQR], 0.50 [0.80] vs. 0.00 [0.14]; P = 0.002), respectively.

Conclusions: Although treatment of small pigmented posterior choroidal melanoma with PDT effectively preserves visual acuity, 5-year treatment-success calculated by Kaplan-Meier analysis was only 38.4%. Recurrences after PDT tend to occur along the tumor edges, often with minimal increase in thickness. Given the substantial risk of treatment failure, primary PDT with vertepofrin is recommended in exceptional cases of choroidal melanoma, for which other treatments with greater tumor control are not a feasible option.
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http://dx.doi.org/10.1016/j.oret.2020.08.019DOI Listing
May 2021

Retinal Microvascular Signs as Screening and Prognostic Factors for Cardiac Disease: A Systematic Review of Current Evidence.

Am J Med 2021 01 27;134(1):36-47.e7. Epub 2020 Aug 27.

Department of Ophthalmology, Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel; Sacker Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

The substantial burden of heart disease promotes an interest in new ways of screening for early disease diagnosis, especially by means of noninvasive imaging. Increasing evidence for association between retinal microvascular signs and heart disease prompted us to systematically investigate the relevant current literature on the subject. We scrutinized the current literature by searching PubMed and Embase databases from 2000 to 2020 for clinical studies of the association between retinal microvascular signs and prevalent or incident heart disease in humans. Following exclusions, we extracted the relevant data from 42 publications (comprising 14 prospective, 26 cross-sectional, and 2 retrospective studies). Our search yielded significant associations between retinal vascular changes, including diameter, tortuosity, and branching, and various cardiac diseases, including acute coronary syndrome, coronary artery disease, heart failure, and conduction abnormalities. The findings of our research suggest that the retinal microvasculature can provide essential data about concurrent cardiac disease status and predict future risk of cardiac-related events.
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http://dx.doi.org/10.1016/j.amjmed.2020.07.013DOI Listing
January 2021

Role of ethnicity and socioeconomic status (SES) in the presentation of retinoblastoma: findings from the UK.

BMJ Open Ophthalmol 2020 15;5(1):e000415. Epub 2020 May 15.

Moorfields Eye Hospital NHS Foundation Trust, London, UK.

Background: The relationship between the ethnic background or socioeconomic status (SES) and late retinoblastoma (Rb) presentation in the UK is unclear. We aimed to investigate if such correlations exist in a cohort of non-familial Rb cases.

Methods: A cross-sectional study based at the two centres providing Rb care in the UK. Included were non-familial Rb cases that presented from January 2006 to December 2011. Epidemiological and clinical data were retrieved from medical charts, as well as patients' postcodes used to obtain the Index of Multiple Deprivation (IMD) score. A postal questionnaire was sent to participants' parents to collect further, person-level, information on languages spoken and household socioeconomic position. Statistical correlations to advanced Rb at presentation as well as to treatment by enucleation and need for adjuvant chemotherapy were investigated.

Results: The cohort included 189 cases, 98 (51.8%) of which were males. The median age at diagnosis was 16 months (IQR 8-34 months). Of the study patients, 153 (81%) presented with advanced Rb; 78 (41%) with group D and 75 (40%) with group E Rb. A total of 134 (72%) patients were treated with enucleation. South Asian ethnicity and being in the most deprived IMD quintile were associated with a higher likelihood of presentation with advanced disease, but these estimates did not reach statistical significance. Older age at presentation was associated with enucleation and bilateral disease with adjuvant chemotherapy.

Conclusions: In this national UK study of patients with non-familial Rb, there was no evidence of an association of ethnicity or SES and the risk of presenting with advanced disease. These findings may reflect equality in access of healthcare in the UK.
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http://dx.doi.org/10.1136/bmjophth-2019-000415DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232618PMC
May 2020

Retinoblastoma presentation, treatment and outcome in a large referral centre in Tehran: a 10-year retrospective analysis.

Eye (Lond) 2021 02 4;35(2):575-583. Epub 2020 May 4.

MAHAK Hematology Oncology Research Center (MAHAK-HORC), MAHAK Hospital, Tehran, Iran.

Background/objectives: Early diagnosis, care and treatment of retinoblastoma is a challengeable issue for Iranian health system. This study was designed and conducted in a referral multidisciplinary centre in the capital city of Iran to evaluate management, care, prognosis and survival rates of paediatric patients with retinoblastoma.

Methods: In this retrospective study, a total number of 309 patients younger than 15 years, diagnosed with retinoblastoma, who referred for diagnosis and treatment to MAHAK's Pediatric Cancer Treatment and Research Center (MPCTRC) from 2007 to 2017 were evaluated. All data were analyzed via SPSS version 22 software in regard of parametric and non-parametric data. Survival rates were analyzed using the Kaplan-Meyer method.

Results: The mean age of patients was 20 months and the majority of patients (77%) had leukocoria as a common clinical symptom at the time of diagnosis. Primary treatment methods were systemic chemotherapy (94%), laser (35%) and primary enucleation (28%). Relapses occurred in nearly 42% of cases, and the median time from diagnosis to the first relapse was 9 months. At the time analyzing the data, 11% of patients died. Patients' 5-year OS and RFS rates were 79.6% and 41.5%, respectively.

Conclusion: Comparing results with other conducted studies identifies that the recurrence rate was high in our considered patients. Also, OS and RFS rates in our study were not as considerable as other reports. Screening methods, updating protocols and follow-up of patients may lead to improvements in survival rates of patients with retinoblastoma.
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http://dx.doi.org/10.1038/s41433-020-0907-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8027402PMC
February 2021

Naevus of Ota: clinical characteristics and proposal for a new ocular classification and grading system.

Br J Ophthalmol 2021 01 30;105(1):42-47. Epub 2020 Mar 30.

Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Hashomer, Israel.

Introduction: Naevus of Ota is a congenital condition that may involve the skin, eyeball and even intracranial structures usually in the distribution of the ophthalmic and maxillary divisions of the trigeminal cranial nerve. The purpose of this study was to summarise our experience with the ocular clinical presentation, imaging, outcome, treatment of complications and to offer a new classification of patients with naevus of Ota.

Methods: We retrospectively reviewed the patients' medical records and the following parameters were retrieved and analysed: demographics, clinical presentation complications and treatment of complications. Imaging characteristics of patients with naevus of Ota were compared with images from the same period of time of 57 age-matched and gender-matched patients without naevus of Ota (control group).

Results: The series was composed of 40 patients (18 males, 22 females) whose mean age at diagnosis was 35.27 years (range 0.5-77 years). Thirty-three patients (82.5%) were type I naevus of Ota according to the Tanino classification, three patients (7.5%) were type II, one patient (2.5%) was type III and three patient (7.5%) were type IV (bilateral naevus of Ota). We further classified all cases in according to the ocular involvement extent. Three patients developed malignant transformation to choroidal melanoma and four patients developed glaucoma.

Conclusions: In this study, a new clinical classification based on the involved ocular component and extent of the involvement (in quadrants) of the globe is suggested first. Further studies are needed to assess whether our clinical ocular classification can assist in identifying patients at risk for developing glaucoma and malignant transformation.
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http://dx.doi.org/10.1136/bjophthalmol-2019-313984DOI Listing
January 2021

Global Retinoblastoma Presentation and Analysis by National Income Level.

JAMA Oncol 2020 05;6(5):685-695

Imam Hussein Cancer Center, Karbala, Iraq.

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.
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http://dx.doi.org/10.1001/jamaoncol.2019.6716DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047856PMC
May 2020

Ocular Manifestations of Leukemia and Results of Treatment with Intravitreal Methotrexate.

Sci Rep 2020 02 6;10(1):1994. Epub 2020 Feb 6.

Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Israel.

Ocular involvement in leukemia is considered rare. Ocular symptoms can be the presenting signs of leukemia, they can appear after diagnosis has been established, or they can be the first manifestation of a relapse after remission. We report, to the best of our knowledge for the first time, the ocular manifestation of a series of patients with ocular leukemia and the result of their treatment with intravitreal methotrexate (MTX) injections. This is a retrospective cohort study. The medical records of 12 consecutive patients with ocular leukemia (24 eyes, 11 eyes treated with MTX) treated at the Sheba Medical Center from January 2010 to December 2017 were retrospectively reviewed. Details on ocular inflammatory reaction and tumor cell infiltration at presentation and the end of follow-up were recorded as main outcome measures. The 12 patients included 7 women and 5 men (mean age ± standard deviation at diagnosis 25.92 ± 23.91 years, range 2-82 years). Eleven eyes of 6 patients were treated with intravitreal MTX injections. The indication for treatment was biopsy proven, tumor cell infiltration. The mean number of MTX injections was 3.37 ± 5.35 (range 1-18). The mean follow-up was 27.08 ± 36.79 months (range 1-93). All treated eyes showed improvement in the inflammatory reaction and tumor cell infiltration. In conclusion we found that Intravitreal MTX injections may be an effective therapeutic approach for eyes with intraocular leukemic tumor cell infiltration.
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http://dx.doi.org/10.1038/s41598-020-58654-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7005017PMC
February 2020

Number, frequency and time interval of examinations under anesthesia in bilateral retinoblastoma.

Graefes Arch Clin Exp Ophthalmol 2020 Apr 3;258(4):879-886. Epub 2020 Jan 3.

Retinoblastoma Service, Royal London Hospital, London, UK.

Purpose: Current practice in retinoblastoma (Rb) has transformed this malignancy into a curable disease. More attention should therefore be given to quality of life considerations, including measures related to examinations under anesthesia (EUAs). We aimed to investigate EUA measures in bilateral Rb patients and compare the findings to EUAs in unilateral Rb.

Methods: A retrospective analysis of bilateral Rb patients that presented to the London Rb service from 2006 to 2013, were treated and had long-term follow-up.

Results: A total of 62 Rb patients, 15 (24.2%) of which had International Intraocular Retinoblastoma Classification (IIRC) group A/B/no Rb at presentation, 26 (41.9%) C/D, and 21 (33.9%) were E in at least one eye. The mean number of EUAs was 35.8 ± 21.5, mean time from first to last EUA was 50.6 ± 19.9 months, and mean EUA frequency was 0.715 ± 0.293 EUAs/month. IIRC group was found not to correlate with any of the EUA measures. Age at presentation inversely correlated with time interval from first to last EUA and to EUA frequency (p ≤ 0.029). Rb family history correlated with the latter measure (p = 0.005) and intraophthalmic artery chemotherapy and brachytherapy correlated with all EUA measures (p ≤ 0.029). Mean follow-up time was 80.1 ± 24.3 months. When compared with a previously reported cohort of unilateral Rb, the present group underwent 3× more EUAs (p < 0.001) over nearly double the time (p < 0.001).

Conclusions: Families should be counselled on anticipated EUA burden associated with bilateral Rb. In this respect, age at presentation and family history were found to have a predictive role, whereas IIRC group did not.
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http://dx.doi.org/10.1007/s00417-019-04589-4DOI Listing
April 2020

Response to "Two Uveal Melanomas in One Eye: A Choroidal Nevus Giving Rise to a Melanoma in an Eye with a Separate Large Choroidal Melanoma".

Ocul Oncol Pathol 2019 Jun 29;5(4):303. Epub 2019 Mar 29.

Ocular Oncology Service, Moorfields Eye Hospital, London, United Kingdom.

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http://dx.doi.org/10.1159/000497615DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6615318PMC
June 2019

Development of conjunctival intraepithelial neoplasia following phototherapy for mycosis fungoides.

Am J Ophthalmol Case Rep 2019 Jun 3;14:98-100. Epub 2019 Apr 3.

Goldschleger Eye Institute, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Israel.

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http://dx.doi.org/10.1016/j.ajoc.2019.03.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6461563PMC
June 2019

Examinations under anaesthesia as a measure of disease burden in unilateral retinoblastoma: the London experience.

Br J Ophthalmol 2020 01 12;104(1):17-22. Epub 2019 Mar 12.

Moorfields Eye Hospital, London, UK.

Background: Early diagnosis strategies and advances in retinoblastoma (Rb) management have resulted in nearly 100% survival. More attention should, therefore, be given to quality of life considerations. We aimed to quantify the number of examinations under anaesthesia (EUAs) in a cohort of patients with Rb, as a measure of disease burden.

Methods: A retrospective analysis of patients with unilateral Rb that presented to the London Rb service from 2006 to 2013, were treated and had long-term follow-up. Correlations of clinical variables to number of EUAs were investigated.

Results: A total of 107 patients with Rb were included that presented at a mean age of 26.51 ± 22.68 months. The International Intraocular Retinoblastoma Classification (IIRC) was group B in 5 (5%), C in 13 (12%), D in 48 (45%) and E in 41 (38%) of the cases. Primary treatment was intravenous chemotherapy in 36 (34%) and enucleation in 71 (66%) of the cases. Mean number of EUAs was 20.67 ± 6.62, 12.52 ± 6.23 and 11.15 ± 6.91 for combined groups B/C, group D and group E patients (p < 0.001), respectively. On analysis, early age atpresentation and conservative treatments were found to significantly correlate with increased number of EUAs (p < 0.001). Mean follow-up time was 74.42 ± 25.16 months and no metastasis or death were reported.

Conclusion: Families should be counselled regarding the number of EUAs associated with the patient's IIRC group, with B/C eyes undergoing twice the number as compared with D/E eyes. For group D cases, where both enucleation and conservative therapy are valid options, treatment choice has a significant impact on the number of EUAs.
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http://dx.doi.org/10.1136/bjophthalmol-2018-313556DOI Listing
January 2020

[UNUSUAL VITREOUS DETACHMENT].

Harefuah 2019 Feb;158(2):109-111

Ocular Oncology Service - The Goldschleger Eye Institute, Sheba Medical Center, Tel-Aviv University, Israel.

Introduction: A 49 years old patient with a history of brachytherapy for choroidal melanoma was referred to the ocular emergency room for suspected recurrence due to pigment in the vitreous cavity. On examination, pigmented vitreous secondary to posterior vitreous detachment with traction from the atrophic tumor was observed with no evidence of recurrence.
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February 2019

The Incidence of Binocular Visual Impairment and Blindness in Children with Bilateral Retinoblastoma.

Ocul Oncol Pathol 2019 Jan 19;5(1):1-7. Epub 2018 Jun 19.

The Royal London Hospital, Barts Health NHS Trust, London, United Kingdom.

Purpose: The study aimed to assess the incidence of and risk factors leading to visual impairment and legal blindness in children with retinoblastoma.

Procedures: This is a single-center, retrospective case series of all patients with bilateral retinoblastoma presenting from 2010 to 2014.

Results: A total of 44 patients were included in the study. Visual impairment was present in 14 (38%) children, legal blindness was present in 7 (19%) children. Bilateral macular tumors (BMT) were associated with visual impairment (12 of 18 patients with BMT, 2 of 19 patients without BMT, = 0.0006) and legal blindness (7 of 18 patients with BMT, 0 of 19 patients without BMT, = 0.003). The International Intraocular Retinoblastoma Classification (IIRC) of the better eye also predicted visual impairment (16% in IIRC Group a, b, c, 75% in IIRC Group D, E, = 0.004) and blindness (3% eye in IIRC Group a, b, c, 50% in Group D, E, = 0.005). Various non-Snellen visual acuity measures were able to predict visual impairment in pre-verbal children, providing them with early assistance.

Conclusions: The rates of visual impairment and blindness reported in this paper can be used to counsel families regarding the risk of binocular visual impairment. Early detection and support for visually impaired infants are essential as development can be affected by severe visual impairment.
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http://dx.doi.org/10.1159/000489313DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341332PMC
January 2019

Tetrac Delayed the Onset of Ocular Melanoma in an Orthotopic Mouse Model.

Front Endocrinol (Lausanne) 2018 8;9:775. Epub 2019 Jan 8.

Goldschleger Eye Institute, Sheba Medical Center, Affiliated to The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Ocular melanoma research, the most common primary intraocular malignancy in adults, is hindered by limited models. In a series of experiments using melanoma cells injected intraocularly into mouse eyes, we developed a model for ocular melanoma. Inoculation of 5 × 10 B16F10 cells led to rapid tumor growth, extensive lung metastasis, and limited animal survival, while injection of 10 cells was sufficient for intraocular tumors to grow with extended survival. In order to improve tumor visualization, 10 melanoma cells (B16F10 or B16LS9) were inoculated into Balb/C albino mouse eyes. These mice developed intraocular tumors that did not metastasize and exhibited extended survival. Next, we studied the therapeutic potential of inhibitor of the thyroid hormones-αvβ3 integrin signaling pathway in ocular melanoma. By utilizing tetraiodothyroacetic acid (tetrac), a thyroid hormone derivative, a delay in tumor onset in the B16F10 (integrin+) arm was observed, compared to the untreated group, while in the B16LS9 cells (integrin-) a similar rate of tumor onset was noticed in both experimental and control groups. In summary, following an optimization process, the mouse ocular melanoma model was developed. The models exhibited an extended therapeutic window and can be utilized as a platform for investigating various drugs and other treatment modalities.
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http://dx.doi.org/10.3389/fendo.2018.00775DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331424PMC
January 2019

Epidemiological and genetic considerations in retinoblastoma.

Community Eye Health 2018 ;31(101):29-30

Retinoblastoma Service: Royal London Hospital; Ocular Oncology Service NIHR Biomedical Research Centre for Ophthalmology, Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, UK.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5998388PMC
January 2018

Classification and staging of retinoblastoma.

Community Eye Health 2018 ;31(101):11-13

Retinoblastoma Service: Royal London Hospital; Ocular Oncology Service NIHR Biomedical Research Centre for Ophthalmology, Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, UK.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5998397PMC
January 2018

Understanding retinoblastoma: epidemiology and genetics.

Community Eye Health 2018 ;31(101)

Retinoblastoma Service: Royal London Hospital; Ocular Oncology Service NIHR Biomedical Research Centre for Ophthalmology, Moorfields Eye Hospital and UCL Institute of Ophthalmology, London UK.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5998389PMC
January 2018

Do no harm: Observation only in a 98-year-old woman with a suspicious large intraocular tumour.

Am J Ophthalmol Case Rep 2018 Jun 9;10:206-207. Epub 2018 Mar 9.

Ocular Oncology Service, Moorfields Eye Hospital, London, UK.

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http://dx.doi.org/10.1016/j.ajoc.2018.03.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5854867PMC
June 2018

[DEVELOPMENT AND CHALLENGES IN TREATMENT OF UVEAL MELANOMA].

Harefuah 2018 Feb;157(2):95-98

Goldschleger Eye Institute, Sheba Medical Center, Sackler Faculty of Medicine Tel Aviv University, Tel Aviv, Israel.

Introduction: Uveal melanoma, the most common primary intraocular malignancy in adults, is potentially a lethal tumour. Since the development of local radiotherapy, tumour control is achieved in the majority of cases and most eyes are salvaged. Despite this, more than 50% of patients develop distant metastatic spread, mainly to the liver, and in the absence of efficient treatments to extend life, die soon after. A great deal of effort has been put into developing prognostic markers for metastatic spread and survival. Novel genetic prognostic tests, recently introduced, are now being clinically used in many ocular oncology centres worldwide. In addition, in most centres, patients are referred for systemic surveillance programs for early detection of liver metastasis. Novel treatment modalities to battle metastatic uveal melanoma are being developed and used in clinical trials. Their efficacy is yet to be proved. This review summarizes the recent developments and current challenges related to uveal melanoma management.
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February 2018
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