Clinical Presentation and Outcome of Retinoblastoma among Children Treated at the National Cancer Institute (NCI) in Gezira,Sudan: A single institution experience

Background: Retinoblastoma (RB) is a rare and unique cancer that affects the eyes of very young children. There are few reports on RB in Sudan.

Materials and Methods: We performed a retrospective study of data from patients diagnosed with retinoblastoma between January 1999 and December 2009 at the National Cancer Institute in Gezira (NCI-Gezira).

Results: Of the 519 cases of childhood cancer treated at NCI-Gezira during the study period, 25 (4.8%) were retinoblastoma. Of these 25 patients with retinoblastoma, there were 13 boys and 19 cases were unilateral. The median age at diagnosis was 36 months (range, 8–60 months). The disease was localized in 9 patients, regional in 5 patients, and metastatic in 11 patients. The most frequent symptoms were enlarged eye (n?=?14) and leukocoria (n?=?8). Nine patients (36%) have been lost to follow-up; 9 were alive at last follow-up (7 in remission, 2 progressed); and 7 have died (5 from disease and 2 from unrelated causes). Twenty-two eyes were enucleated in 16 patients (6 bilateral and 10 unilateral). Pathologic examination of the enucleated eyes could only be completed in 11 patients. Diagnostic imaging in the form of computerized tomography scans or ultrasonography of the brain and orbit was done for 10 patients (40%).

Conclusions: Although these findings are not surprising, and similar to reports from other developing countries, we hope our work will provide a foundation for strategies to improve outcome for retinoblastoma in our center such as proper training, public awareness, team approach, and twinning.     

Clinical presentation and outcome of retinoblastoma based on age at presentation: a review of 1450 children

To compare the clinical presentation and outcomes of retinoblastoma (RB) based on age at presentation. Retrospective comparative study of 1940 eyes of 1450 children with RB. Presentation of RB with enlarged eyeball and eyelid swelling (2% and < 1% in ≤ 1 year, 4% and 2% in > 1–2 years, 7% and 2% in > 2–3 years, and 12% and 4% in > 3 years; p < 0.0001 and p = 0.05, respectively) is more common with increasing age. Based on the 8th edition of American Joint Committee Classification, T1 is more common in children younger than 1 year (27%), while T4 is more common in children > 3 years of age (20%) (p < 0.0001). Kaplan–Meier (KM) estimate at 1 and 5 years for globe salvage was 64% and 58% in children ≤ 1 year of age versus 30% and 20% in children > 3 years, respectively [Hazard ratio (HR) = 2.48; p < 0.0001], and KM estimate at 1 and 5 years for life salvage was 99% and 97% in children ≤ 1 year of age versus 89% and 78% in children older than 3 years, respectively (HR = 7.65; p < 0.0001). Uncommon clinical features of RB including enlarged eyeball and eyelid swelling are more common with increasing age. Younger age at presentation with RB is associated with better prognosis including higher chances of life and globe salvage.     

Enucleation for retinoblastoma: the experience of a single center in Jordan

Despite multiple advances in the management of retinoblastoma, enucleation remains an essential therapeutic modality. We studied patients who underwent enucleation at the King Hussein Cancer Center in Jordan. We retrospectively reviewed medical records of children with retinoblastoma who were treated at our center from June 2002 to February 2008. Twenty-eight eyes from 27 patients were enucleated. Median age at diagnosis was 1.1 years (range, 0.3–6.3 years). Twenty-six eyes (93%) had advanced disease (RE groups IV and V). Seventeen patients (61%) had unilateral retinoblastoma, and 11 (39%) had bilateral retinoblastoma. The median time from diagnosis to enucleation was 0.45 months (range, 0–45 months; mean, 4.4 months) and was longer for patients with bilateral retinoblastoma (median, 2.2 vs. 0.2 months; P = 0.034). Twenty enucleated eyes (71%) did not show high-risk pathologic features. Seventeen eyes with advanced intraocular disease were enucleated at the time of presentation, whereas chemoreduction was attempted for the other 19 eyes with advanced intraocular disease. Enucleation was then recommended for nine (47%) of those eyes. Enucleation at the time of diagnosis was feasible for most patients with advanced disease. Attempted salvage of eyes with advanced disease is justified, particularly in patients with bilateral disease. We were able to salvage almost half of these eyes. We hope our study provides new insights for counseling patients.     

Stage of presentation and visual outcome of patients screened for familial retinoblastoma: nationwide registration in the Netherlands

BACKGROUND: In the Netherlands a comprehensive programme for screening just after birth for familial retinoblastoma is taking place. In this report the stage of the disease at the time of detection, by way of screening, and the long term visual outcome in these patients was evaluated. METHODS: A nationwide, retrospective study. From January 1992-July 2004, patients at risk for familial retinoblastoma were screened 1-2 weeks after birth, and investigated for laterality, Reese-Ellsworth classification/International Classification of Retinoblastoma, macular involvement, age of primary retinoblastoma, initial therapy, and visual outcome. RESULTS: 17 patients were diagnosed with familial retinoblastoma. 88.3% developed bilateral, 11.7% unilateral retinoblastoma. Of the 34 eyes, 56% were R-E group I, 16% were group II A-B, 16% were group III A-B, 9% were group IV, 3% were group V. Using the International Classification of Retinoblastoma, 72% were group A, 19% were group B, 6% were group C, 3% were group E. The visual outcome revealed 73.5% of eyes with 20/20-20/40, 26.5% eyes with < or = 20/100-no light perception; 5.9% of eyes were enucleated, all other eyes were treated with local or conservative treatment methods. Of all eyes, 59% had extramacular retinoblastoma, 98% of patients had at least one eye with extramacular retinoblastoma. CONCLUSION: Most familial retinoblastoma patients present as a R-E group I or group A when screened within 2 weeks after birth. Nearly 90% of patients had a long term visual acuity of 20/20-20/40. Despite the common occurrence of macula involvement, bilateral macula involvement was infrequent, and since most eyes were salvaged, good vision was obtained in the majority of patients.     

Fluorine-18 fluorodeoxyglucose positron emission tomography (PET) to detect vital retinoblastoma in the eye: preliminary experience

BACKGROUND/AIMS: To report our first experience with FDG-PET in the detection of vital retinoblastoma. METHODS: Four newly diagnosed retinoblastoma patients, two treated retinoblastoma patients, and four control patients were enrolled in this pilot study. F18-FDG uptake was assessed in the light of clinical and histopathological features. RESULTS: PET discriminated between new patients and controls, although tumor uptake varied widely. PET added no useful information with regard to possible vital tissue in tumor scars in the eye of the two treated retinoblastoma patients. Moreover, PET findings did not correlate with clinical or histopathological features. CONCLUSION: Based on this small pilot study, F18-PET shows little promise in the detection of retinoblastoma. More research on other radiofarmacons is recommended.     

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

Background/objectivesEarly 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.MethodsIn 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.ResultsThe 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.ConclusionComparing 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.Subject terms: Outcomes research, Eye cancer     

Characteristics,management, and outcome of patients with uveal melanoma treated by Iodine-125 radioactive plaque therapy in a single tertiary cancer center in Jordan

Objective

To evaluate King Hussein Cancer Center experience in using Iodine-125 COMS radioactive plaque for treatment of Uveal Melanoma in Jordan.

Clinical outcome after in situ laser keratomileusis (LASIK) for myopia: a series of 390 eyes

PURPOSE: We report our personal experience with LASIK for myopia to study its efficacy, safety, predictability and stability for low, moderate and high myopia. PATIENTS AND METHODS: Between January 1996 and December 1997, 390 eyes were treated. Follow-up was 6 months for 254 and one year for 136. Initial myopia ranged from -1.5 to -16 diopters with a spherical equivalent of -8.49D. The surgical procedure with topical anesthesia used the Chiron ALK-E automated corneal shaper for the initial flap of 160 microns thick, and the Chiron Excimer Laser Keracor 117 Technolas with an active eye tracking system for the stromal photoablation. Preoperative refraction, uncorrected (UVA) and best corrected visual acuity (BCVA) were compared to postoperative results. RESULTS: They are reported overall and for sub-groups of myopia. (A<=-5 D, -5 D=20/40 at 6 months. Average UVA was 0.65 D at one year and was as better as preoperative myopia was lower (group A 0.65D, group B0.5 at 6 months). Mean spherical equivalent was +0.08 D at J1 and -1.02 D at one year (group A -0.8 D, group B -0.67 D, and group C -3.92 D). Safety was correct and only a few per and postoperative complications were observed with only two patients losing more than two lines of BCVA. Mean BCVA was 0.72 at one year. Predictability demonstrated that 90% of group A eyes were at +/-1 D from emmetropia and 74% in group B at 6 months. More than 95% of eyes were stable at 6 months and 90% at one year. CONCLUSION: LASIK is a safe and efficient surgical treatment for low, moderate and high myopia, and should be improved with new software and new corneal shapers.     

Iris melanocytic tumours in New Zealand/Aotearoa: presentation,management and outcome in a high UV exposure environment

Background/ObjectivesIris melanoma, a rare intraocular malignancy, represents the smallest subgroup of uveal melanoma. This first, comprehensive study of iris melanocytic lesions in the high ultraviolet environment in New Zealand/ Aotearoa (NZ) examines diagnosis, management and outcomes.Subjects/MethodsRetrospective study of iris melanocytic tumours referred to tertiary referral centres in Auckland, NZ, over 20 years (1999-2018). Data analysed include demographics, tumour characteristics, histology, genetic analyses, treatment modalities, recurrence, metastasis, 5-year and overall survival.ResultsCohort (N = 51) was predominantly NZ European (98.0%) with no indigenous Māori, or Pasifika. Median age at presentation was 58 years. Tumours involved a median of two clock hours of iris. The posterior tumour margin extended to the anterior chamber angle in 22 patients (45.8%). Management included initial observation 54.9%, iridectomy/excision biopsy 29.4%, irido-cyclectomy 7.8%, plaque radiotherapy 7.8%, proton beam radiotherapy 7.8%, and ultimately enucleation 17.6%. Histology was performed in 19 cases (37%) with 16 confirmed melanomas (84%). Mean follow-up 4.2 years with median visual acuity of 6/7.5 two years post intervention. Melanoma-related metastasis and mortality occurred in two cases with five-year melanoma-related mortality of 2.0%.ConclusionIn a climate with high ultraviolet exposure iris melanocytic tumours occurred almost exclusively in NZ Europeans, however, the majority of cases were category T1, possibly reflecting early diagnosis in the NZ health system. Nonetheless, >50% underwent surgery or radiotherapy, often utilising more than one modality. A high index of suspicion and early referral of iris melanocytic lesions should be considered in regions with high UV exposure.Subject terms: Uveal diseases, Outcomes research     

Baseline characteristics, the 25-Item National Eye Institute Visual Functioning Questionnaire, and their associations in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT)

OBJECTIVE: To describe characteristics of participants in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT) at baseline and to investigate associations among visual function, fundus features, and vision-related quality of life. DESIGN: Cross-sectional study. PARTICIPANTS: The 1052 participants in CAPT, a multicenter, randomized clinical trial. Eligibility criteria for CAPT included > or =10 large drusen and visual acuity > or =20/40 in each eye. METHODS: At baseline, the visual acuity, contrast sensitivity, and critical print size for each eye were measured, color stereo photographs of the disc and macula of each eye were taken, and the 25-item National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) was self-administered. Graders from a central photograph reading center evaluated the photographs for drusen characteristics and focal hyperpigmentation. All procedures were performed using standardized protocols. Associations among characteristics were assessed by Spearman correlation coefficients and multiple linear regression. RESULTS: Among CAPT participants at baseline, the mean age was 71.0 years, 60.6% were women, and 99.3% were white. The median visual acuity of the better eye was 20/20 and of the worse eye 20/25. In approximately one third of eyes, drusen covered > or =10% of the retina within 3000 microm of the foveal center, and 67.7% of eyes had focal hyperpigmentation. Drusen area and focal hyperpigmentation were weakly correlated (r = -0.08 to -0.18) with the measures of visual function. The measures of visual function were weakly associated with the NEI-VFQ-25 scores. An association of fundus features with NEI-VFQ-25 scores was not found. CONCLUSION: At baseline, CAPT participants had good visual function and several risk factors for progression to neovascular age-related macular degeneration. Scores on the NEI-VFQ-25 indicated that participants perceived some problems with their vision. Within this relatively homogeneous group of participants, measures of visual function were only weakly associated with the measures of vision-related quality of life.     

The Smith-Kettlewell Institute (SKI) longitudinal study of vision function and its impact among the elderly: an overview.

We present an overview of a multifaceted longitudinal study of vision function and its interaction with daily activities, health, and well-being among 900 persons aged 58 to 102 years at the first visit. Standard vision measures as well as nonconventional tests designed to assess visual performance under the nonideal conditions encountered in everyday life were used. Here we summarize a few of the findings to date, with an emphasis on a direct comparison of declines in different aspects of vision function with age. The rates of declines with advancing age vary widely for the different vision functions. Also described is the reading performance of the sample and its association with some of the vision measures. Furthermore, we describe some of the associations between vision test scores and extensive longitudinal health and functioning data collected by the Buck Center for Research in Aging. Findings show that many older people with good acuity are effectively visually impaired in performing everyday tasks involving low and changing light levels, stereopsis, glare, and low contrast. We also found that vision under nonideal conditions cannot be predicted from standard acuity on an individual basis.     

Clinical evaluation of corneal epithelialization after photorefractive keratectomy in patients treated with polydeoxyribonucleotide (PDRN) eye drops: a randomized, double-blind, placebo-controlled trial

PURPOSE: The effect of polydeoxyribonucleotide (PDRN) eye drops vs placebo on corneal epithelial healing after photorefractive keratectomy (PRK) for correction of myopic and myopic-astigmatic defects was evaluated in a randomized, double-blind clinical trial. Primary endpoint for efficacy was the evolution of corneal re-epithelialization. Secondary endpoint was the evaluation of PDRN eye drops tolerability. METHODS: Sixty eyes were enrolled in the study, randomly allocated into standard therapy plus placebo eye drops (30 eyes), or standard therapy plus PDRN eye drops (30 eyes). Checks were carried out preoperatively and at days 1, 2, 3, and 7 of the follow-up. Six eyes dropped out (four in PDRN group, two in placebo group) for reasons unrelated to the study. RESULTS: On day 2, the disepithelialized area was 8.4 mm2+/-9.2 (mean+/-SD) in controls and 6.0 mm2+/-6.8 in PDRN group. On day 3 a complete corneal re-epithelialization was found in 20 out of 26 (77%) eyes of PDRN group and in 17 out of 28 (61%) eyes of placebo group (p<0.05 in percentage terms). On day 7 of follow-up, all eyes appeared to be completely re-epithelialized. The mean score of corneal evaluation on day 3 was 2.9 in PDRN group and 3.75 in control group (p<0.05 between groups). No adverse events occurred during the study. CONCLUSIONS: The data of the study have shown that after PRK, PDRN stimulates corneal epithelium regeneration. PDRN eye drops administration four times a day is well tolerated by patients during the re-epithelialization stage. A much larger clinical study should be performed in order to prove the results obtained in this pilot study.     

Characteristics, management, and outcome of squamous carcinoma of the conjunctiva in a single tertiary cancer center in Jordan

AIM: To evaluate the features and outcome of management of malignant conjunctival squamous tumors in King Hussein Cancer Center (a referral tertiary cancer center in the Middle East). METHODS: Retrospective case series of 31 eyes for 31 patients with conjunctival squamous neoplasia. Main outcome measures included: age, gender, laterality, tumor location, pathological features, tumor stage, treatment modality, and outcome. RESULTS: Twenty (65%) patients were males and median age was 58y. Twenty-two (71%) eyes had the tumor in the nasal quadrant. Tumor invasion to nearby structures was seen in 19 (61%) eyes, including the cornea, fornix, eyelid, and orbit in 17 (55%), 1 (3%), 2 (6%), and 3 (10%) eyes, respectively. Eye salvage was achieved by surgical excision with cryotherapy followed by topical chemotherapy in 28 (90%) eyes, and orbital exenteration was necessary in 3 (10%) eyes due to orbital tumor invasion. Tumor recurrence was seen in 7 (23%) eyes, and the significant predictive factors for recurrence were tumor extension onto the nearby structures (P=0.04), tumor invasiveness (P=0.038), and tumor TNM stage (P=0.031). No significant change in visual acuity was seen, and disease related mortality was 6% (2 patients, both had orbital invasion by invasive squamous carcinoma). CONCLUSION: Conjunctival squamous carcinoma is more common in males. Advanced American Joint Committee on Cancer (AJCC) T-stage, tumor local invasion, more pathologically aggressive tumors, and surgical treatment alone (without adjuvant therapy) are associated with higher risk for recurrence, and orbital invasion is the most important poor prognostic factor for metastasis and death. Treatment strategies should be affected by tumor characteristics at presentation.     

年龄相关性黄斑变性诊断与治疗规范——2018年英国专家共识解读

年龄相关性黄斑变性为黄斑区结构的衰老性改变,主要由视网膜色素上皮细胞和视网膜退行性病变引起,患者出现不可逆性中心视力下降或丧失。英国国家卫生和临床优化研究所发布了最新的年龄相关性黄斑变性分类、诊断与治疗指南,本文即对年龄相关性黄斑变性的新指南进行解读。     

Clinical Experiences with Silflex (I): Presented at the International Contact Lens Conference,Fiji, July 1978

The Wohlk Siflex Silicone Contact lens is discussed with particular attention to the lens surface, the effects of tear chemistry, and other data which may be helpful to fellow practitioners. A definite relationship between tear chemistry and the lens is demonstrated, and methods by which the rate of mucus deposition on the lens surface can be reduced is suggested. In conclusion, the lens is found to have better properties than other types of lenses available.