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An 8-year-old girl presented with painless decreased vision and leukocoria in the right eye since 6 days. There was no antecedent history of trauma. Visual acuity in the right eye was counting fingers 1 m. Ocular examination revealed right exotropia, conjunctival congestion, anterior chamber cells

Background Red reflex test (RRT) is a simple, non-invasive method that can be performed easily by pediatricians during the clinical examination in neonatal period, infancy and childhood. Abnormal reflexes can lead to prompt diagnosis of several ocular disorders, with potentially severe consequences on patient's vision, cognitive function and even life. Aim To underline the contribution of pediatricians to early detection of vision and life threatening diseases by using RRT effectively. Methods For the present systematic review, PubMed searches were performed using the key words "red reflex and newborn"; "red reflex and neonate"; "red reflex and complications"; "red reflex and necessity"; "red reflex and retinoblastoma"; "red reflex and congenital cataract"; "red reflex and glaucoma"; "red reflex and prematurity"; "red reflex and leukocoria"; "red reflex and blindness"; "red reflex sensitivity and specificity"; "red reflex and differential diagnosis"; "red reflex and guidelines". The relevant articles were selected without language restrictions. When a full-text publication was not available, their English abstracts were used. In some cases, studies from the reference lists of the selected articles provided useful information. The research took place in September 2020, in the Ophthalmology Department of University Hospital of Alexandroupolis. Results A total of 45 articles were selected according to the used key words. After reviewing data from these articles, it is supported that red reflex remains an effective tool of undeniable importance for early detection of severe eye conditions, such as cataract, retinoblastoma, retinopathy of prematurity and glaucoma. Although literature reports some limitations of RRT, including a notable percentage of false positive tests, the inability to detect small, peripheral retinoblastomas and the lower sensitivity for posterior segment pathology, it is widely accepted that the benefits from the regular evaluation of the test on public health are significant. Therefore, RRT has been established by international guidelines and should be an essential component of pediatricians clinical practice. Red reflex implementation should be incorporated in pediatricians educational programs, so that they would be able to provide quality services and safe diagnoses. Conclusion The implementation of RRT should be encouraged in all neonatal/pediatric departments. Prompt education of pediatricians should be empowered in order to achieve careful vision screening, according to current guidelines.

Untreated congenital toxoplasmosis remains an important cause of neurologic and ocular disease worldwide. However, congenitally infected infants may not have signs and symptoms their physicians recognize, leading to delayed diagnosis and missed opportunities for treatment. We describe a pair of twins diagnosed with congenital toxoplasmosis at 11 months of age following incidental detection of leukocoria in one twin.

Visual development begins at birth and continues throughout childhood. Ocular pathology can lead to permanent visual impairment and subsequent problems in overall development and school performance as well as signify serious systemic disease or even life-threatening malignancies. Prompt identification of congenital or early acquired ocular pathology via regular bedside ocular examinations and assessments of visual behaviors by neonatal clinicians is a critical component of newborn care. The goal of this article is to review the components of the newborn eye examination, describe key ocular findings and diagnoses that are common or critical to identify in the newborn period, and offer guidance on necessity and timing of ophthalmology referral.

This case series highlights the possibility of retinoblastoma in children with a history of trauma. Retinoblastoma commonly presents with leukocoria. In our series, the history of blunt trauma led to a misdiagnosis. The delay in correctly diagnosing retinoblastoma was made more difficult with hyphaema and vitreous haemorrhage obscuring the fundus view. Hyperdensities in imaging tests were mistaken for intraocular foreign bodies and post-trauma insult rather than calcification of an intraocular tumour. Both patients underwent anterior chamber washout. The patients were referred to our centre when their condition worsened. Retinoblastoma was highly suspected and confirmed from histopathological examination after enucleation. An accurate diagnosis can only be achieved by exercising a high index of suspicion. Misdiagnosis and mismanagement will lead to poor prognosis.

Background Retinoblastoma (RB) outcomes in Thailand are unfavorable compared to those of developed countries. This study aims to determine whether the clinical outcomes of patients with RB significantly improved after the implementation of new therapeutic approaches and which clinical factors affect survival and globe-saving outcomes. Methods The medical records of patients newly diagnosed with RB and treated at Siriraj Hospital between January 2005 and December 2018 were reviewed retrospectively. Clinical data, treatments, and outcomes were collected and analyzed. Results In 194 eyes (144 patients), leukocoria was the most common presenting feature (76.8%); 129 (66.5%) eyes were staged in group E of the International Classification of Intraocular Retinoblastoma. Of the 149 enucleated eyes, 35 had high-risk histopathological features, mostly choroidal invasion; 45 eyes (23.2%) could be salvaged. The 5-year overall survival rate was 90.3%, an improvement compared to the previous study. The 5-year enucleation-free survival rates of Groups A and B, C, D and E were 100%, 83.1%, 36.7% and 16.6% respectively. Factors associated with a lower survival rate were interval from symptom onset to diagnosis >3 months (hazard ratio (HR): 5.8: 95% confidence interval (CI): 1.637, 20.579) and buphthalmos (HR: 12.57: 95% CI: 3.936, 40.153). Factors associated with high-risk features were secondary glaucoma (HR: 11.016: 95% CI: 1.24, 98.10) and pseudohypopyon (HR: 14.110: 95% CI: 2.16, 92.05). Conclusions Survival rates and globe-saving rates appear to have improved; however, advanced-stage presentation remains the major hindrance. Further studies with a larger cohort and longer follow-up are warranted.

A 3-hour-old full-term female neonate presents with white pupils noted at the time of NICU admission. ### Prenatal and Birth Histories The infant was limp and cyanotic at delivery and required positive pressure ventilation for 5 minutes. No risk factors for sepsis were noted and there was no evidence of placental abruption. In addition, the fetal heart rate tracing was normal before delivery. The reasons for the perinatal depression at birth were unclear. Subsequently, the infant started breathing spontaneously and was transitioned to room air. Neurologic examination findings obtained at the outside facility were significant for wide-eyed appearance with staring and generalized low muscle tone. ### Presentation The infant was transferred to the NICU at 3 hours of age for therapeutic hypothermia evaluation. Initial physical examination findings were remarkable for the presence of leukocoria bilaterally. This was visualized easily with both ambient lighting in the patient room as well as direct examination with an ophthalmoscope (Fig 1). Red reflex was absent bilaterally. Figure 1. Bilateral leukocoria. The infant’s neurologic assessment findings at admission were consistent with moderate hypoxic-ischemic encephalopathy. Therapeutic whole-body cooling was initiated. An umbilical venous catheter was placed and continuous electroencephalogram obtained. The infant also received ampicillin and ceftazidime, intravenous fluids, and continuous fentanyl infusion. ### Vital Signs (3 Hours of Age)

A 4-year-old girl presented with leukocoria, vision loss, and painless left eye redness. Posterior segment examination revealed a total retinal detachment and abnormal, tortuous retinal vessels in the left eye. No visible mass, vitreous seeding, or hemorrhage were observed. B-mode ultrasound showed a detached retina in a "cinnamon-roll" configuration. Due to poor visual prognosis and high clinical suspicion of malignancy, enucleation was performed. Further histopathological analysis confirmed the diagnosis of diffuse infiltrative retinoblastoma, with an atypical presentation.

Midwives should receive appropriate training to enable them to reliably detect an abnormality in the red reflex, so as to detect potentially sight- (and possibly life-) threatening visual anomalies in newborns.Midwives who had completed the relevant Newborn and Infant Physical Examination (NIPE) training in the UK, were tested to determine whether they could reliably detect an abnormality in the red reflex of a model eye. Their results were compared to a group of undergraduate optometry students in their final year, who had considerably more experience with ophthalmoscopy.Two groups of adult participants, 27 post-graduate midwives and 10 undergraduate optometry students with different levels of experience in ophthalmoscopy were recruited. Participants were required to perform the red reflex test repeatedly on a set of 10 model eyes with interchangeable simulated retinas, that produced both normal and abnormal 'red' reflexes, under controlled conditions.A total of 1,810 observations were recorded across both groups of participants. Both the sensitivity and the specificity for distinguishing between normal and abnormal reflexes were higher for the optometry students (sensitivity 77.5 per cent and specificity 96.7 per cent) than the midwives (sensitivity 53.9 per cent and specificity 75.4 per cent). The positive predictive values were 21.7 per cent and 67.4 per cent for the midwives and optometry students, respectively. The negative predictive values were 92.8 per cent for the midwives and 98.0 per cent for the optometry students.Despite completion of required training on the red reflex test, optometry students outperformed midwives in the detection of anomalous red reflex test simulations from a model eye. This result is likely due to differences in training in ophthalmoscopy between the two groups of participants. Additional training in the conduct of the red reflex test for midwives is therefore recommended.

Purpose: Diffuse infiltrating retinoblastoma (DIR) is characterized by absence of intraocular mass, lack of calcification. It may mimic inflammatory uveitis or exudative retinopathy. Observations: An eight-years-old boy presented with progressive loss of vision in left eye. Clinical evaluation revealed neovascular glaucoma with a yellow–gray fundal glow, exudative retinal detachment, subretinal exudation and telangiectatic vessels. The presentation was consistent with exudative retinopathy (Coat’s disease) but for the presence of a family history of retinoblastoma in the younger sibling. Despite the absence of an intraocular mass or calcification on multimodal imaging, the enucleation was done on the basis of clinical suspicion of retinoblastoma. Histopathology confirmed a diagnosis of DIR. Conclusions : DIR can pose a diagnostic challenge due to its non-characteristic clinical and imaging features and atypical presentation. A high index of suspicion along with a positive family history was key to diagnosis in our case; histopathology was confirmatory. Keywords: Retinoblastoma, Diffuse infiltrating, Leukocoria, TNM staging, Hereditary retinoblastoma

Importance: Educating parents to recognize signs of eye disease and consult a healthcare professional is critical to timely diagnosis, intervention and outcomes. Background: We evaluate the effectiveness of an eye-health information pamphlet on parents' level of concern and help-seeking intention should they hypothetically observe leukocoria or strabismus in their child. Design: Double-blind, randomized controlled trial conducted at a metropolitan antenatal outpatient clinic. Participants: In total, 518 pregnant women were enrolled in the study. Methods: After completing a study-specific, pre-test survey describing hypothetical clinical scenarios at baseline, participants were randomly assigned to receive a pamphlet on either paediatric eye health (intervention) or infant play (control). The post-test survey was sent by email 2 weeks after baseline. Main outcome measures: A change in the parents' level of concern if they observed leukocoria or strabismus and a change in their help-seeking intention if they hypothetically observed leukocoria or strabismus in their child. Results: Of the 518 women, 382 (73.7%) completed the post-test survey. At follow-up, women who received the intervention were more likely to report a higher level of concern if they observed leukocoria (OR 1.711 [CI: 1.176-2.497] P = .005]) and were less likely to delay help-seeking (OR 0.560 [CI 0.382-0.817] P = .003). No change in the level of concern for strabismus was identified between the groups; however, at follow-up, women who received the intervention were less likely to delay help-seeking (OR 0.318 [CI 0.125-0.806] P = .016). Conclusion and relevance: Providing parents with relevant, evidence-based information can significantly improve their knowledge and positively influence help-seeking intentions if leukocoria or strabismus are observed.

Coats’ disease was first described by George Coats, as a unilateral retinal vascular abnormality.Coats’ disease is a nonhereditary, idiopathic disease presenting with vascular telangiectasia withintraretinal and subretinal exudation with no racial preponderance and systemic associations. Coats’disease presents with a wide ambit of clinical features- vision loss, strabismus, leukocoria, ornystagmus. The three classical features that are pathognomonic of Coats’ are exudative retinaldetachment, telangiectatic vessels, and peripheral retinal ischemia. The modalities for treatment ofCoats disease that can be used are laser photocoagulation, anti-VEGF agents, or a combination ofboth and cryotherapy. This article describes a case report of a 10-year-old male child withcomplaints of painless loss of vision, his ophthalmological evaluation, and the treatment isundertaken.

To report the epidemiology, clinical characteristics, treatment and outcomes of patients with ocular toxocariasis.Retrospective review of patients diagnosed with ocular toxocariasis at a pediatric ...

The most important part of digital image analysis is object classification. Nowadays, deep learning makes an enormous achievement in computer vision problems. So there has been a lot of interests in applying features learned by convolutional neural networks (CNNs) on general image recognition to more tasks such as object detection, segmentation and face recognition. Leukocoria detection is one of the serious challenges in infant retinal treatment. Leukocoria is represented as an abnormal white reflection appearing in the eyes of an infant suffering from retinoblastoma. This research proposes a deep Visual Geometry Group-net CNN classifier for automatic detection of leukocoria. The proposed classifier comprises pre-processing, feature extraction and classification. The deep CNN classifier contains convolution layer, pooling layer and fully connected layer with weights are developed on each image. Experimental results based on several eye images consist of ordinary and leukocoric from flicker, and it demonstrates that the proposed classifier provides better results with the accuracy of 98.5% and the error rate is below 2% which exceeds the current results.

Neonatal retinoblastoma is more widely reported in developed countries than in developing countries. We have conducted a retrospective case series to understand the nature of neonatal retinoblastoma in Malaysia due to lack of data from developing countries. A retrospective record review of 295 patients was conducted from the Retinoblastoma Registry on the National Eye Database (NED) between January 2005 and December 2019. Six out of 295 patients (2.03%) were identified as neonatal retinoblastoma in Malaysia from 2005 until 2019 compared to developed countries, in which the detection rate were 3.5 to 10 times higher. Lack of awareness among survivor parents about the risk of their newborns developing retinoblastoma in may be a contributing factor. Neonatal retinoblastoma is difficult to manage, thus close and continuous monitoring is needed in centres dedicated to retinoblastoma management.

Children who present with an abnormal red reflex (ARR) are often referred to ophthalmology due to concern for retinoblastoma. However, an ARR can indicate a wide variety of pathologies, all of which have the potential to develop amblyopia and irreversible vision loss. In this retrospective cohort study, we demonstrate that children who presented with an ARR had a mean age of 22.0 ± 32.5 months and were more frequently referred by their pediatricians (74.5%). The majority of these patients (61.8%) had a normal examination on further evaluation, followed by refractive error (20.4%). Amblyopia was diagnosed in 83.9% of patients with refractive error, with a mean age of 50.3 ± 49.2 months. Because many ARR-associated pathologies require time-sensitive treatment to prevent vision loss, proper screening is critical for diagnosis. Pediatricians play a key role in screening, so education on more common ARR pathologies can better facilitate referrals and improve outcomes.

Background Paediatric head and neck cancers represent an important group of childhood cancers that require maximum attention at large. They are often diagnosed at advanced clinical stages at Muhimbili National Hospital (MNH). Objective To describe the clinical profile of paediatric head and neck cancers at a tertiary hospital in Tanzania Method A hospital based descriptive cross-sectional study was conducted involving 180 paediatric patients. Data were analyzed using SPSS program version 21. Results A total of 180 paediatric patients were recruited where 61.1% were males and 38.9% were females. Majority belonged to the age group 0-5 years (53.9%). A total of 10 primary anatomical sites were found with the neck (cervical lymph node) predominating (36.1%), followed by the orbit/eye (34.4%) and the least anatomical sites were oral cavity (1.1%) and parapharyngeal space (0.5%). Leukocoria (93.9%), red eye (93.9%) and orbital cellulitis (77.6%) predominated in retinoblastoma but in patients with lymphomas, predominant features were night sweats (100%), weight loss (100%) and fevers (95.8%). Conclusion The clinical profile depicted in this study appears to correlate with advanced clinical stages. Keywords: Paediatric; Head; Neck Cancers; Muhimbili; Tanzania

Background: Retinoblastoma is the most common intraoc-ular tumor in children. There are two forms heritable and non-heritable. Leukocoria is the most common presenting sign, accounting for about 56.1% of cases. Strabismus is the second most common mode of presentation. Early retinoblastoma patients (group A and B ICRB) treated by systemic chemo-therapy in conjunction with local control have the higher rate of eye salvage. Aim of Study: To classify retinoblastoma patients presented to Oncology Unit of Ain Shams Department of Ophthalmology to international groups and study presentation, modalities of treatment and treatment outcome of group A and B patients. To see to what extent in Ain Shams Department of Ophthal-mology we succeed achieving eye salvage in early retinoblas-toma patients group A and B. Patients and Methods: This study involved patients have been treated at Oncology Unit of Ain Shams Department of Ophthalmology. Data was extracted from recorded files of patients in the period from January 2004 till January 2012. All patients data were subjected to refinement of: Age, sex, date of first visit, complaint, family history, fundus examina-tion, diagnosis, classification to international groups, treatment, follow-up. Results: Complete tumor regression had occurred in 90% of all group A and B patients following treatment with systemic chemotherapy plus focal laser thermotherapy. Enucleation was needed in 6.7% of all patients. Group A only patients had a 90% percentage of complete tumor regression, with only one patient died from pneumonia (one out of 30). Group B only patients had a 90.5% percentage of complete tumor regression, with only two patients had needed enucleation (2 out of 30). EBR was needed in 6.7% of all patients. Age at presentation mean was 1.09 year with range (0.1-3). Regarding presenting symptoms, 80% of patients presented with leuko-coria and 13.3% with squint. Family history was negative in 86.7% and positive in 13.3%. Conclusion: Our conclusion is that early retinoblastoma patients (group A and B ICRB) treated by systemic chemo-therapy in conjunction with local control have resulted in ocular salvage rates exceeding 90% for early intraocular disease.

Introduction: Retinoblastoma is one of the pediatric malignancies and its treatment is still challenging. The objective of this study was to highlight the sociodemographic, clinical, and therapeutic aspects of retinoblastoma at the Donka Paediatric Haematology/Oncology unit in Donka university hospital, Guinea, and thereby highlight the conditions of this malignancy in this country. Methods: This was a retrospective study over 3 years (January 1, 2017 to December 31, 2019). The study population was all retinoblastoma patients who were treated in this unit. Socio-demographic, clinical, and therapeutic data were analyzed. Results: Out of 210 pediatric patients followed up for cancer in this unit during the period, retinoblastoma accounted for 46 (22%). The patient age at diagnosis was 35.17 months (range: 3 - 132 months). At admission, metastases were observed in 16 patients (35%): 14 with brain metastases and 2 with lymph node metastases. Preoperative chemotherapy was employed in 38 (83%), tumor resection was performed in 20 patients (43%), and postoperative chemotherapy was performed in 19 patients. As for the prognosis, remission in 9, death in 14, and treatment discontinuation in 15. Only one patient had health insurance (2%). Conclusion: In this region of Guinea, retinoblastoma is the most common ocular tumor in children. Its diagnosis is easy: strabismus and leukocoria are the early signs of retinoblastoma. The prognosis depends on how early the diagnosis is made. Physicians, care-givers, and family members should be aware of the nature of this malignancy and social education/message as such is necessary.

Background: Pediatric cataracts cause significant visual disability worldwide. The objectives of this study were to determine the distribution of pediatric cataracts by sex, age groups, laterality, type and presentation in population of D.I.Khan District, Pakistan. Materials & Methods: This descriptive study was carried out at Department of Ophthalmology, Gomal Medical College, D.I.Khan, Pakistan from January 2015 to December 2015. Sample was collected from Eye unit, DHQ Teaching Hospital, D.I.Khan. Pediatric cataract patients under 14 years of age were included in the study. Demographic variables were sex and age groups. Research variables were laterality, type and presentation. All variables being nominal were analyzed through frequency and percentages. The observed and expected distribution of pediatric cataract by sex, age groups, laterality, type and presentation were respectively tested by chi-square goodness of fit test. Results: Out of a sample of 47 patients, 29 (61.7%) were boys and 18 (38.3%) girls, 22 (46.81%) were

Purpose: To report the presenting signs of retinoblastoma in a large cohort of patients who underwent orthoptic assessment at presentation. Methods: A retrospective medical chart review was conducted on 131 patients with retinoblastoma who presented consecutively to a single institution during a 6-year period. The main outcome measure was the presenting sign(s) of the disease. Results: Of 131 patients with retinoblastoma, 88 presented with unilateral disease and 43 presented with bilateral disease (mean ages: 22.7 and 14.8 months, respectively). Leukocoria was the presenting sign in 56% of patients, leukocoria and strabismus in 18%, strabismus in 13%, inflammation in 8%, and “other” signs in 5%. The fovea was affected by the retinoblastoma tumor or its sequelae in 75% of patients. Patients who presented with strabismus were significantly more likely to have foveal involvement than patients who presented with leukocoria alone ( P = .001). Thirty-one percent of patients had strabismus as a component of their presentation; 63% had exotropia, 23% had esotropia, and 14% had variable strabismus. The percentage of patients with strabismus increased to 66% when small angle and variable strabismus were also considered. Patients with inflammation had worse ocular survival ( P < .05). Conclusions: This study assessed the combination of leukocoria and strabismus as presenting features of retinoblastoma. Foveal involvement is common in patients who have strabismus and may influence decision-making regarding globe salvage. The authors confirmed that exotropia is more common than esotropia in retinoblastoma in the largest cohort to have undergone an orthoptic assessment. [ J Pediatr Ophthalmol Strabismus . 2021;58(5):324–330.]

Background: Retinoblastoma is the most common primary intraocular tumor in children. Prune belly syndrome is a rare congenital disease affecting newborns. The combination of the two diseases has never been reported before. In this article, we present a case of a male infant diagnosed with prune belly syndrome (PBS) at birth. Case report: Patient underwent many surgeries to manage the resulting consequences of PBS. At the age of 7 months, he presented with left eye leukocoria. He was examined and diagnosed with retinoblastoma. The eye was enucleated, and the diagnosis was confirmed histopathologically. This is the first case to be described in the literature of such an association to the best of our knowledge. Key words: prune belly syndrome; retinoblastoma; Eagle-Barrett syndrome

PURPOSE: To study clinical characteristics of Retinoblastoma and analyze the occurrences of patient survival and globe salvation. METHODS: This is a prospective and non-randomized study involving retinoblastoma patients at a tertiary institute from May, 2017 to May, 2019. All patients underwent comprehensive medical assessment and illness management investigations. The given treatment was recorded, including focal therapy, enucleation and chemotherapy. RESULTS: Leukocoria was the foremost common presentation (60.68%) followed by proptosis (12.3%). The patient survival rate was 87.7% whereas globe salvation was at 23.2% (19 eyes). CONCLUSION: Most patients came for clinical examination for rst time at a later stage of disease, having high risk clinical features such as positive family history, hyphema, and large tumor volume. More awareness and early stage diagnosis are of prime importance in order to achieve globe salvation in retinoblastoma.

Lymphatic malformations are relatively uncommon orbital lesions which are usually diagnosed in early childhood. Their exact etiology is unknown. Persistent Hyperplastic Primary Vitreous (PHPV) results from the failure of the embryological primary vitreous and hyaloid vasculature to regress. We report a rare case of a 9-year old male child who presented to our clinic with unilateral coexisting superficial orbital lymphatic malformation with persistent fetal vasculature manifesting as visual compromise and leukocoria. Keywords: Persistent fetal vasculature, Microphthalmia, Cataract, Lymphangioma, Leukocoria.

We sought to evaluate the role and diagnostic potential of ocular B-scan ultrasonography in childhood eye disease in an observational cross-sectional study; 1091 patients with a total of 1445 eyes examined were studied. Cataract was the single most common indication for ultrasound followed by corneal pathology, ocular trauma, posterior segment pathology, primary congenital glaucoma, leukocoria, orbital pathology and other disease. Ultrasonography resulted in a change in diagnosis in 198 cases (18%). We conclude that B-scan ultrasonography plays an important adjunctive role in the management of childhood eye disease.

Retinoblastoma is a childhood retinal malignancy affecting approximately 9,000 new patients globally per year (1). Leukocoria and strabismus are the most common presenting signs. However, retinobla...

Purpose: Retinoblastoma though a rare disease is the most common intraocular malignancy in the paediatric population. The age of presentation, clinical picture and modes of treatment has been changing with time in developing countries. The purpose of this study is to evaluate the changing trend and know our current status on retinoblastoma. Materials and Methods: Five years retrospective study of retinoblastoma patients at a tertiary eye centre in Kathmandu. Results: Seventy-four eyes of 62 patients fulfilled the inclusion criteria. There was equal gender distribution. Eighty percent had unilateral retinoblastoma while twenty percent had bilateral retinoblastoma. Unilateral cases presented late (32.23 months) while bilateral cases presented earlier (19 months). 71% patients presented with leukocoria while only 11% presented with proptosis. Sixty-four eyes underwent destructive procedure (60 enucleation and 4 exenteration) and 10 eyes were managed with LASER. Conclusion: Retinoblastoma patients are presenting earlier with leukocoria as common mode of presentation. Eye saving management of retinoblastoma are in rising trend in recent years. Keywords: Retinoblastoma, Leukocoria, Enucleation, Nepal.

Background: Early diagnosis and timely management of Retinoblastoma (RB) patients are essential to improve eye salvage and survival rates. The objective of this study is to evaluate the lev...

Purpose To study the clinical presentation, treatment, and outcomes of patients with bilateral advanced intraocular retinoblastoma. Methods Retrospective case series of 72 patients. Results The mean age at presentation was 19 months. Leukocoria (n = 49, 68%) was the most common presenting complaint. The tumors were classified as groups D (n = 60, 42%) or E (n = 84, 58%) based on the Philadelphia version of International Classification of Retinoblastoma (ICRB); groups D (n = 84, 58%) or E (n = 60, 42%) based on Children's Hospital Los Angeles version of International Classification of Intraocular Retinoblastoma (ICIoR); T2 (n = 116, 81%) or T3 (n = 28, 19%) based on 8th edition American Joint Committee Classification (AJCC). Systemic chemotherapy (n = 138, 96%) was the most common primary treatment modality. The chance of globe salvage was higher for group D based on ICRB (83%; odds ratio (OR) 7.73; 95% confidence interval (CI) 3.45-17.33) or ICIoR (81%; OR 12.75; 95% CI 5.74-28.34) and T2b (73%; OR 5.19; 95% CI 2.51-10.73) based on AJCC. Over a mean follow-up period of 59 months, tumor recurrence was noted in 42 (29%) eyes and globe salvage was achieved in 83 (58%) eyes. Of the 50 eyes where vision was recorded, vision of 20/200 or better was achieved in 24 (48%) eyes. There were events of leukemia (n = 1, 1%), pinealoblastoma (n = 1, 1%), systemic metastasis (n = 3, 4%), and death (n = 4, 6%) during the follow-up period. Conclusion Multimodality treatment allows globe salvage (58%) and vision salvage (48%) in eyes with advanced group D and E intraocular retinoblastoma.

espanolLa retinopatia del prematuro sigue siendo la principal causa de ceguera infantil que afecta principalmente a los bebes prematuros y de bajo peso al nacer. La prevencion del parto prematuro es la estrategia mas efectiva para reducir la incidencia de retinopatia del prematuro. La identificacion y el tratamiento oportunos son esenciales para prevenir la perdida innecesaria de la vision causada por retinopatia del prematuro. Se ha asociado con multiples factores de riesgo entre los principales el bajo peso al nacer, edad gestacional y utilizacion de oxigeno suplementario, entre otros. Su cuadro clinico se caracteriza por una membrana retrolental que origina leucocoria y eventualmente sin tratamiento con complicaciones potenciales como glaucoma, catarata, miopia y ceguera, siendo esta ultima la mas grave. El estandar de tratamiento actual sigue siendo la terapia ablativa a traves de la fotocoagulacion con laser de la retina isquemica avascular periferica y actualmente se estan realizando estudios con anti VEGF los cuales han demostrado beneficios significativos, sin embargo aun no han sido aprobado por la FDA para su utilizacion clinica. EnglishRetinopathy of prematurity continues to be the leading cause of childhood blindness that primarily affects premature and low birth weight babies. The prevention of preterm birth is the most effective strategy to reduce the incidence of retinopathy of prematurity. Timely identification and treatment are essential to prevent the unnecessary loss of vision caused by retinopathy of prematurity. It has been associated with multiple risk factors among the main low birth weight, gestational age and use of supplemental oxygen, among others. Its clinical picture is characterized by a retrolental membrane that causes leukocoria and eventually without treatment with potential complications such as glaucoma, cataract, myopia and blindness, the latter being the most serious. The current standard of treatment continues to be ablative therapy through laser photocoagulation of the peripheral avascular ischemic retina and studies are currently being conducted with anti VEGF which have shown significant benefits, however, they have not yet been approved by the FDA for its clinical use.

To study the clinical presentation, treatment, and outcome of patients with retinoblastoma (RB) in India.Retrospective study of 1,457 patients with RB (2,074 eyes).The mean age at presentation of RB was 29 months (median, 24 months; range,1-370 months). There were 812 (56%) men and 645 (44%) women with unilateral presentation of RB in 57% (n = 834) and bilateral in 43% (n = 623). Familial RB was present in 4% (n = 55). The most common presenting complaints included leukocoria (n = 1,100; 75%), proptosis (n = 91; 6%), strabismus (n = 77; 5%), and red eye (n = 68; 5%). Most (n = 1,889; 91%) tumors were intraocular in location, and 185 (n = 185; 9%) had extraocular tumor extension at presentation. The most common modalities of primary treatment-included systemic chemotherapy (n = 1,171; 60%) and enucleation (n = 674; 35%). At a mean follow-up period of 44 months (median, 30 months; range, 3-234 months), 92% (n = 1,206) were alive, and 108 (8%) patients died because of RB. Based on Kaplan-Meier analysis, the survival at 1, 3, 5, and 10 years was 94%, 91%, 90%, and 89%, respectively.The most common presenting signs of RB in Asian Indian population are leukocoria and proptosis. With appropriate treatment, the survival rate is favorable at 92%.

PURPOSE: To improve and validate the smartphone-based leukocoria detection application so that non-ophthalmologists could make use of the smartphone for early detection of Retinoblastoma (RB) in young children without anesthesia and pharmacological dilatation of the pupil. METHODS: Two apps, MDEyeCare and CRADLE, developed for red reflex based leukocoria detection were used in iPhone 6s. MDEyeCare methodology was modified with respect to ambient lighting, the distance between camera and eye and different gazes for better performance. We analyzed totally 34 eyes of 23 RB patients and four normal children. Each of the RB patients was confirmed with clinical examination and radiological investigations. RESULTS: Modification in the methodology of MDEyeCare app could detect the leukocoria in early stages of RB (50% of Group B, 83% of Group C). In late stages (Group D and E), 100% of tumors were detected. The CRADLE app failed to provide adequate leukocoria detection except four late stage RB eyes. Among the 14 normal eyes (6 from unilateral RB and eight from normal children), pseudo-leukocoria was observed in three eyes only at lateral gaze even with MDEyeCare app. CONCLUSION: Improved methodology in smartphone-based app enhanced the detection of RB and this may translate into better outcome after treatment with respect to vision salvage.

Retinoblastoma is a common ocular malignancy (Eye cancer) of children. It gets developed in the eyes of children born due to mutation in the RB1 gene. Retinoblastoma is also called leukocoria which develops from the immature cells of a retina. The proposed idea is to develop a web-based application to detect eye cancer and its response to the treatment. Deep learning algorithms like Convolution Neural Network (CNN) can be used for classifying eye images to detect the presence or absence of Retinoblastoma. The proposed system applies pre-processing techniques such as smoothening and resizing to the fundus images of the eye for better results. Image processing techniques such as Histogram equalization, Morphological Operations, Unsharp Masking, Image Intensity Adjustment, and Segmentation are used for tumor extraction. After extraction, the tumor regression is calculated by comparing the area of consecutive stages of the tumor. Thus the proposed system serves as a helping aid to health professionals to track the response of their treatment to such patients.

The case involves a five-year-old female patient with a myelinated retinal nerve fiber (MRNF) layer of the right optic disc. Although this is a rare, benign, and often asymptomatic condition, it is sometimes associated with ocular findings which require early detection and treatment. In this case, the patient presented with strabismus, high myopia, and amblyopia. She was found to have myelinated retinal fiber layer lesions of the superotemporal and inferotemporal retina of her right eye. This case report aims to demonstrate the importance of performing a thorough evaluation of MRNF in the pediatric patient as well as to increase awareness of this entity to avoid misdiagnosis.

Purpose To assess the general knowledge of and attitude to various common eye conditions in children among pediatricians in north Jordan. Methods This was a descriptive cross-sectional study. An exploratory questionnaire was developed by the research team to collect the necessary qualitative information. Results In total, 48 pediatricians participated in the study. Around two-thirds performed eye examinations in children; however, only 10.4% (n = 5) carried out eye exams routinely as part of every child's health visit. The most common test done was assessment of red reflex, which was performed by only 60.4% (n = 29) of responders. Almost all participants (95.8%, n = 46) recognized the risk factors for retinopathy of prematurity (ROP). The majority of pediatricians (n = 46, 95.8%) would immediately refer a child with suspected glaucoma to an ophthalmologist. In regard to leukocoria, the majority recognized cataract (n = 38, 79.2%) and retinoblastoma (n = 40, 83.3%) as major causes of this condition. Almost all pediatricians (n = 46, 95.8%) would immediately refer a child with leukocoria to an ophthalmologist. On the subject of refractive errors, only 62.5% (n = 30) of physicians confirmed that refractive errors could occur in children of any age and that they may need glasses. Nonetheless, 70.8% (n = 34) stated that refractive errors could be a cause of squint in children. Amblyopia and underlying central causes of squint were a major concern for most responders with 68.8% (n = 33) and 75% (n = 36), respectively. Conclusion Although the knowledge of and attitude to eye disease among pediatricians were at a satisfactory level, the degree of collaboration with ophthalmologists is limited. More workshops need to be held for pediatricians., Highlights • Knowledge of eye disease among pediatricians were at a satisfactory level. • The degree of collaboration with ophthalmologists is limited. • More workshops need to be held for pediatricians.

Pediatric patients are commonly referred to imaging following abnormal ophthalmological examinations. Common indications include papilledema, altered vision, strabismus, nystagmus, anisocoria, proptosis, coloboma, and leukocoria. Magnetic resonance imaging (MRI) of the brain and orbits (with or without contrast material administration) is typically the imaging modality of choice. However, a cranial CT scan is sometimes initially performed, particularly when MRI is not readily available. Familiarity with the various ophthalmological conditions may assist the radiologist in formulating differential diagnoses and proper MRI protocols afterward. Although MRI of the brain and orbits usually suffices, further refinements are sometimes warranted to enable suitable assessment and accurate diagnosis. For example, the assessment of children with sudden onset anisocoria associated with Horner syndrome will require imaging of the entire oculosympathetic pathway, including the brain, orbits, neck, and chest. Dedicated orbital scans should cover the area between the hard palate and approximately 1 cm above the orbits in the axial plane and extend from the lens to the midpons in the coronal plane. Fat-suppressed T2-weighted fast spin echo sequences should enable proper assessment of the globes, optic nerves, and perioptic subarachnoid spaces. Contrast material should be given judiciously, ideally according to clinical circumstances and precontrast scans. In this review, we discuss the major indications for imaging following abnormal ophthalmological examinations.

There are no substitutes to a thorough history and careful clinical examination while planning for any surgery and pediatric cataract is no different. Preoperative evaluation should be performed keeping the differential diagnoses in mind. Questions like the cause of the cataract, whether to operate, when to operate and how to operate should be answered by preoperative examination. Performing biometry and deciding the particulars of the intraocular lens implant including the post-operative target refraction are all equally challenging but essential prerequisites. With numerous uncertainties, a good preoperative planning is the key to successful management of pediatric cataract.

Congenital cytomegalovirus (CMV) infection (cCMV) is challenging to differentiate from congenital rubella syndrome (CRS) clinically. Virus detection and serological tests are needed. However, they are often not readily available or are expensive. This is a case of a five-month-old male with bilateral cataracts. He was jaundiced at birth and started having seizure episodes at one month of age. He was also diagnosed with right inguinal hernia and had abnormal bilateral hearing tests. Both eyes were noted to have leukocoria at two months of age. There was dazzle on both eyes and sclerae were anicteric. Examination revealed dense cataracts on both eyes, but their ocular ultrasound results were essentially normal. Due to the bilateral hearing loss and bilateral cataracts, CRS was initially considered despite the absence of heart abnormality since there were reported CRS cases without the complete triad. However, possible coinfection or another disease was considered due to the presence of jaundice, seizures, and hernia, which were never seen in our previous CRS patients nor were reported in the literature. The patient underwent cataract extraction on both eyes without intraocular lens implantation (IOL) as recommended for CRS cataracts to prevent severe inflammation. TORCH (TOxoplasmosis, Rubella, Cytomegalovirus, Herpes simplex) test was negative for rubella but positive for CMV. As such, the patient would have benefitted from early IOL implantation. The patient was then referred to a national medical center for possible treatment. However, since the patient already tested negative for CMV polymerase chain reaction (PCR) there, systemic antiviral therapy was no longer initiated. This case presented the challenge of clinically differentiating cCMV and CRS.

Leukocoria, known as 'white pupil', can be present in several eye diseases, including retinoblastoma, a rare malignant eye tumour. It can be detected in photographs and is called photoleukocoria. The red reflex test is the best tool to detect leukocoria, and the American Academy of Paediatrics (AAP) recommends that it should be performed in all children's routine medical appointments in the first five years of live.

A nine-day-old male infant presents with his mother to the pediatric clinic with a concern of a white spot in his right eye. There was no history of antenatal or perinatal complications and the mother's serology was negative. Physical examination was remarkable only for leukocoria in the right eye. He was evaluated to have a Mittendorf dot. This is a benign clinical finding. Pupillary reflex check is an important part of a physical examination which can recognise pathological conditions such as cataract, retinoblastoma, metabolic errors with ocular manifestations and lead to early treatment before the child loses vision.

Introduction Retinoblastoma (Rb) is the most common intraocular malignant tumor of childhood. The different modes of Rb presentation comprise proptosis, anterior chamber inflammatory signs, spontaneous hyphema, secondary glaucoma, and strabismus. The primary aim of this study was to investigate the different clinical presentations and stages of Rb that may help in early detection and timely diagnosis to prevent the advancement of the disease and increase rates of survival in children. Materials and methods This was a descriptive cross-sectional study conducted between December 2019 and May 2020 over a period of six months at a tertiary care hospital in Karachi, Pakistan. The sample size included 68 eyes of children with lesions of Rb at the time of presentation to the hospital. Brightness scans (B-scans), computed tomography (CT) scans, and magnetic resonance imaging (MRI) were performed. The International Intraocular Retinoblastoma Classification (IIRC) was used to stage each eye. In case of enucleation (if necessary) of the eye, the biopsy was performed to evaluate the histological features of cancer. All statistical analysis was performed using Statistical Package for Social Sciences version 17.0 (IBM Corp., Armonk, New York). Results The mean age of the children was 3.21 ± 1.75 years. Leukocoria was the most common clinical presentation observed in more than half (n = 35, 51.47%) of the sample population followed by proptosis reported in nearly two-fifths (n = 25, 36.76%), strabismus and phthisis bulbi observed in equal proportions (n = 3, 4.41%), and hypopyon documented in a minor proportion (n = 2, 2.94%) of patients. Regarding stages of the Rb disease, the most common stages were observed to be stage C and stage E. Conclusions This study concludes that the early detection of Rb is possible through a better understanding of presenting features of the disease. It can prevent the progression of the disease to the advanced stages and decrease morbidity and mortality. The early detection of Rb can be made possible through the examination of red reflex on the regular check-ups of children as leukocoria is the most common clinical presentation.

Myelinated retinal nerve fiber layer is a benign retinal condition that is usually an incidental finding in a healthy patient. Visual acuity is typically not compromised, but some cases have other associated ocular conditions that can affect vision. Diagnosis in challenging atypical cases requires imaging studies to rule out other pathologies. The authors describe the case of a 2-year-old girl who presented with atypical optic nerve fiber myelination. [ J Pediatr Ophthalmol Strabismus . 2020;57:e56–e58.]

Background Homozygous protein C (PC) deficiency is a potentially fatal disease with ocular blinding presentation or sequela. Case presentation A 5 month-old boy was presented for evaluation of leukocoria. He had a history of frequent bruises and PC deficiency, treated with warfarin. His intraocular pressure was normal. In the left eye leukoma with anterior segment dysgenesis, shallow anterior chamber, and cataract were observed. Fundus was not visible. B-scan revealed a closed funnel retinal detachment. His right eye had a normal anterior segment and a thin retina with anomalous retinal vascular branching at equator and peripheral retina. A fibrovascular tuft on the optic nerve head with induced traction on superior arcade was visible. Total loss of a and b wave of both were appreciated in electroretinography (ERG). Fluorescein angiography (FA) showed very severe leakage at the junction of the vascularized and non-vascularized retina and optic nerve head. Favorable outcome was achieved with lasering of avascular retina in the right eye. Conclusion The potential for protein C deficiency should be assessed in all infants with leukocoria, anterior segment dysgenesis, retinal detachment and retinal dysplasia. Early diagnosis could save the child’s life and vision.

Introduction: A masquerade syndrome is an atypical presentation of a neoplastic process that mimics an inflammatory condition. In this paper, we focus on orbital pseudocellulitis. Case Series: Our case series includes 5 retinoblastoma patients with orbital pseudocellulitis at presentation. In 3 patients the disease was bilateral, in 1 trilateral, and in 1 unilateral. The eyes with pseudocellulitis were enucleated, while the fellow eyes were treated conservatively, when affected. Four patients responded well to the therapy and showed remission of the tumor. The patient with trilateral retinoblastoma did not respond to therapy and died of disease. Discussion: Differential diagnosis with infectious orbital cellulitis is extremely important. Patients with orbital cellulitis present with fever, sinusitis, leukocytosis, and raised inflammatory markers, while ophthalmoscopic examination is negative and imaging studies show sinus involvement. On the contrary, patients with retinoblastoma do not show systemic inflammation, while ophthalmoscopic examination reveals leukocoria, buphthalmos, and an intraocular tumor mass associated with retinal detachment. Magnetic resonance imaging shows intralesional calcifications and soft tissue edema without sinus involvement. Histology confirms the diagnosis. Conclusions: Medical history, physical examination, and imaging studies are crucial in the diagnosis of retinoblastoma-associated orbital pseudocellulitis. Retinoblastoma should be excluded in all patients with signs of pre-septal orbital cellulitis through fundoscopy and/or imaging studies

To report on the characteristics and outcome of management of Coats’ disease, and to describe a novel surgical technique for management of stage 3B with total retinal detachment (RD) by scleral external drainage with anterior chamber (AC-maintainer) placement before the drainage without pars plana vitrectomy. A retrospective study of 26 eyes from 25 Coats’ patients. Outcome measures included: demographics, presentation, laterality, stage, treatment, and outcome. The median age at diagnosis was 3.5 years. Twenty patients (80%) were males, and all except 1 girl had unilateral disease. The presenting complaint was impaired vision in 13 (50%) eyes, leukocoria in 6 (23%) eyes, and strabismus in 7 (27%) eyes. Based on the Shields classification; 3(12%) eyes were stage 1, 9 (35%) eyes were stage 2, 10 (38%) eyes were stage 3, 2 (8%) eyes were stag e4, and 2 (8%) eyes were stage 5. Primary management included cryotherapy (54%), laser photocoagulation (27%), intravitreal anti-vascular endothelial growth factor (23%), intravitreal steroids (23%), and surgical drainage (12%). The 3 eyes in stage 3B (with total exudative RD) underwent subretinal fluid drainage with AC maintainer, and all had the retina reattached completely for 6 months follow up after the surgery. At mean follow up 21 months, 4 (15%) eyes were enucleated, 19 (73%) eyes had improvement or stabilization in visual acuity. Coats’ disease usually presents at advanced stage with poor visual prognosis, individualized management with close follow up are mandatory to save the eye. Subretinal fluid drainage with AC maintainer is a safe and useful technique for repairing total RD in eyes with stage 3B Coats’ disease.

Retinoblastoma is the most common primary ocular malignancy(Eye cancer) of children. It gets developed in the eyes of children born due to mutation in the RB1 gene. Retinoblastoma is also called leukocoria which develops from the immature cells of a retina. The proposed idea is to develop a web-based application to detect eye cancer and its response to the treatment. Deep learning algorithms like Convolution Neural Network(CNN) can be used for classifying eye images to detect the presence or absence of Retinoblastoma. The proposed system applies pre-processing techniques such as smoothening and resizing to the fundus images of the eye for better results. Image processing techniques such as Histogram equalization, Morphological Operations, Unsharp Masking, Image Intensity Adjustment, and Segmentation are used for tumor extraction. After extraction, the tumor regression is calculated by comparing the area of consecutive stages of the tumor. Thus the proposed system serves as a helping aid to health professionals to track the response of their treatment to such patients.

Congenital tumors, in particular orbital teratomas, develop in the fetus and may be recognized prenatally during ultrasound examinations or within the first few years after birth. Retinoblastoma is the most frequent intraocular malignant tumor in children. Extremely rare in fetal eyes, it may develop soon after birth and in most cases is recognized within the first 3 years of life, owing to leukocoria or screening of children with a positive family history. Medulloepithelioma of the eye is a rare tumor of the ciliary body that usually presents after 2 years of age, but it can be seen in newborns or adults.

PURPOSE To determine the age distribution of children with Coats disease and the impact of age at diagnosis on the visual prognosis. METHODS Consecutive Coats disease cases aged 18 years or younger at diagnosis were retrospectively included. Clinical and imaging parameters were analyzed by comparative, correlation, survival, univariate, and multivariate statistics. RESULTS Ninety-eight patients were included. At diagnosis, mean age was 5.4 years ± 4.3 years (1 month-18 years). Younger age at diagnosis was correlated with more severe disease stage (P < 0.0001, r = -0.52), which was confirmed by survival analysis (P < 0.0001). Comparative analysis was performed between patients younger and older than 4 years at diagnosis. Leukocoria or strabismus was more frequent at presentation in patients younger than 4 years (P < 0.0001). Areas of peripheral nonperfusion and peripheral telangiectasia were more extensive at presentation in younger than older patients (P = 0.0003 and P = 0.039). Foveal sparing at diagnosis was less frequent in younger than older patients (2% vs. 23%, P = 0.002). The incidence of structural complications or enucleation during follow-up (mean duration: 5.9 years ± 4.5 years) was higher, and last-recorded visual acuity was lower in younger than older patients (P = 0.001 and P = 0.0009). Final logarithm of the minimal angle of resolution visual acuity was negatively correlated with age at diagnosis (P = 0.001, Spearman r = -0.42). Multivariate analysis indicated that disease stage (P < 0.0001), but not age at diagnosis (P = 0.07), independently influenced the last-recorded visual acuity. CONCLUSION Onset of Coats disease in children of younger age is associated with more severe manifestations, more advanced stage, and worse visual outcome. Age, correlated with disease stage, should be considered a prognostic marker in Coats disease.