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Background/Purpose This study aims to describe the clinical characteristics and treatment outcome of retinoblastoma in Mongolian children. Methods Data of all children diagnosed with retinoblastoma at the National Center for Maternal and Child Health of Mongolia from 1987 to January 2014 were reviewed retrospectively. The ICRB classification was used. Survival characteristics of the cohort were analyzed. Results Retinoblastoma was diagnosed in 79 eyes of 64 cases during the study period. Median age of diagnosis was 24.5 ± 15.8 months. There were no differences in sex ratio, and 15 cases (23%) were bilateral. Forty-three (67%) patients were from rural areas. The more frequent clinical presentations were leukocoria in 50 (78%) patients, strabismus in 24 (38%) patients, and glaucoma in 21 (33%) patients. Sixty-one (95%) patients were diagnosed with Classification D or worse when presented to us. Due to late diagnosis in the majority of cases, unilateral and bilateral enucleations were performed in 48 (61%) eyes and 24 (30%) eyes, respectively; exenteration was done in three (4%) eyes. Fifty-two (81%) patients received chemotherapy and 13 (8.3%) patients underwent external beam radiation after enucleation. At the time of last follow-up, 52 (81%) patients were alive, five (8%) patients were dead, and seven (11%) patients had lost to follow-up or unknown vital status. The mean follow-up period was 121.5 months (range, 12–360 months). In five cases with immunohistochemistry analysis in the eye specimen, neuron-specific enolase-, Ki-67 protein-, and B-cell lymphoma 2-positive cells were found in all five (100%) cases and Rb protein was detected in three (60%) cases. Conclusion Retinoblastoma in Mongolia is frequently diagnosed at late stages and has a poor outcome. These data show the importance of early pediatric eye examinations and better treatment of retinoblastoma in children younger than 3 years in Mongolia.

Background: Retinoblastoma (RB) is the most common intraocular malignancy of childhood. It accounts for 10-15% of cancers that occur in infants. Objective of the study was to determine the pattern, presentation and management of Retinoblastoma (RB) patients at a tertiary cancer care center in South India, during a period of 5 years (1 st November 2009 to 1 st December 2014). Methods: This study was a retrospective analysis of the medical records of patients diagnosed as having retinoblastoma obtained from hospital information system and our cancer registry. Data sought was demographic characteristics, clinical presentation, investigations done, the methods of management and the treatment outcome of retinoblastoma patients. Results: Total number of cases studied was 31 and total number of eyes studied was 47. Out of 31 cases, 58% were female. 52% had bilateral involvement. The median age at presentation was 24 months. The commonest mode of presentation was leukocoria (55%) followed by proptosis (22%). Out of 47 eyes studied, 87% were advanced tumours belonging to Group D or Group E. Metastasis to the central nervous system was noted in 22.6% patients belonging to either Group D or Group E. Out of the 31 patients, 64.6% patients underwent enucleation. 6 out of 31 cases succumbed to death. Conclusions: Retinoblastoma continues to be a challenge in developing countries. Lack of awareness and inaccessibility to proper healthcare facilities are major stumbling blocks in achieving high cure rates. Educating the public and healthcare professionals, importance of early diagnosis and prompt referral are vital in reducing morbidity and mortality associated with the disease.

Purpose To study the presenting signs of Retinoblastoma in Egypt at Egypt’s main pediatric oncology referral center. Methods This is a prospective descriptive study (hospital-based registry) conducted at Children’s Cancer Hospital Egypt between July 2007 and December 2012. Results Out of 262 patients diagnosed with retinoblastoma, 244 were suffering from intra-ocular disease at presentation. One hundred thirty-nine (57%) patients presented with unilateral disease, while 105 (43%) suffered bilateral disease. The mean age at presentation was 20.6 ± 17 months, averaging 18.87 ± 11.76 months for bilateral and 25.72 ± 18.78 months for unilateral disease. The most common clinical presentation was leukocoria in 180 (73.8%) patients, strabismus in 32 (13.1%) patients and decreased visual acuity in 12 (4.9%) patients. Group D and E disease represented 62% of all affected eyes. Patients with advanced disease (Group C–E) had longer duration of symptoms. Conclusion In Egypt, retinoblastoma patients present more frequently with advanced disease. There is an ever-increasing need to develop a national team dedicated to studying disease significance and formulating a national awareness program.

Ein bilateraler persistierender hyperplastischer primarer Glaskorper (PHPV) stellt eine seltene Entitat einer kongenitalen Fehlbildung dar. Die vorliegende Kasuistik prasentiert erstmalig in der deutschsprachigen Literatur ein 4 Monate altes Madchen mit beidseitigem posteriorem PHPV.

Purpose Intraarterial chemotherapy for retinoblastoma is usually reserved for infants aged 3 months or older because of the intricacy of the newborn vascular anatomy making the procedure technically challenging. The authors report a successful case of intraarterial chemotherapy performed in a 2-month-old infant using a minimal exposure approach. Methods Case report. Results A 2-month-old infant presented with leukocoria and was subsequently diagnosed with an exophytic Group D retinoblastoma in the right eye. The infant received melphalan 3 mg delivered into the ostium of the ophthalmic artery of the right eye under fluoroscopic guidance. Examination under anesthesia a month later showed complete tumor regression to a calcified Type I scar. After a second cycle of intraarterial chemotherapy, no further treatment was necessary. There were no complications. Conclusion Intraarterial chemotherapy is generally used for retinoblastoma in infants aged 3 months or older. The patient was successfully catheterized and treated at 2 months of age, with complete tumor regression after a single chemotherapy dose. Thus, in expert hands, intraarterial chemotherapy can be considered in such young infants.

The purpose of our study was to evaluate the ocular survival and event-free survival after multimodal therapy for group D and E of retinoblastoma (RB). Enucleation of group D and E is controversial as the risks of chemotherapy must be weighed against the potential for vision. A 10-year retrospective study from one center of 86 patients with advanced intraocular disease defined as International Classification Retinoblastoma (ICRB) group “D” or “E.” Cases with visible extraocular extension at diagnosis were excluded. Ocular survival and patient survival were assessed. Indirect ophthalmoscopy at examination under anesthesia to visualize the tumor was used to evaluate clinical response. The median onset age in 86 patients with group D or E eye was 16 months (1–167 months). There were 29 (34%) bilateral cases. Leukocoria was the most common presentation sign (61%). Chemoreduction was primarily used in the treatment of intraocular RB. Selective ophthalmic arterial injection (SOAI) was applied as a component of multimodal therapy in 34 of the 86 cases. The globe preservation rate in patients with group D or E eyes was 19%. Using chemoreduction for advanced eyes, more eyes are being preserved which enables 70% 5-year ocular survival in patients with group D eyes. In triaging appropriate patients, multidisciplinary strategy can reduce tumor size with chemoreduction and consolidate the regressed tumor with local ophthalmic therapy to ensure globe salvage.

Purpose To provide clinical and pathological features of posterior persistent fetal vasculature (PFV) presenting with vitreous hemorrhage. Observations Case 1 was a one-year old male with PFV reaching up to the posterior lens capsule. Case 2 and 3 both had history of blunt trauma. B-scans in cases 2 and 3 revealed vitreous hemorrhage and an intravitreal tissue attached to the optic disc. Pre-operative visual acuity in cases 1, 2 and 3 was undetermined due to age, hand motion and light perception, respectively. During vitrectomy, a fibrotic stalk attached to the optic nerve was removed, which consisted of fibrovascular tissue enveloping pockets of hemorrhage histopathologically. The fibrovascular tissue contained smooth muscle actin (SMA) positive spindle-shaped myofibroblasts in one case and hemosiderin-laden macrophages in another case. Glial fibrillary acidic protein (GFAP) stain was focally positive in two specimens. The proliferation index was low using Ki-67 stain in all cases. Post-operative visual acuity in case 3 remained unchanged, while improved in case 2 from hand motion to 20/70. There was no recurrence of the vitreous hemorrhage. Conclusion and Importance Vitreous hemorrhage may occur in cases of PFV with or without history of blunt trauma. Hemorrhage within the persistent fetal vasculature may become organized with reactive process in the hyaloid stalk. The fibrovascular stalk contained astrocytes and myelofibroblasts which contribute to the formation and contractile function of PFV, respectively. The outcomes following vitrectomy seemed to be satisfactory.

espanolEl retinoblastoma es la neoplasia maligna intraocular mas frecuente en la edad pediatrica. Es diagnosticado entre el primer y tercer ano de vida. Puede afectar uno o ambos ojos, siendo el signo clinico mas caracteristico la leucocoria, seguida del estrabismo, nistagmo y ojo rojo. En cuanto a su incidencia, el retinoblastoma es mucho mas frecuente en los paises menos industrializados, de clase baja y en areas rurales. Es considerado una enfermedad autosomica dominante, donde ambos alelos del gen RB1 se encuentran mutados, ocasionando alteraciones en el ciclo celular. Existen dos presentaciones del retinoblastoma, la forma hereditaria la cual esta asociada a mutaciones de la linea germinal en el gen del retinoblastoma, conocido como RB1, esta presentacion generalmente se presenta en ninos de edad temprana, la mayoria de los casos son bilaterales o multifocales. La forma no hereditaria se caracteriza por mutaciones somaticas de RB1 en el tumor, generalmente tienen una enfermedad unilateral y unifocal, presentan antecedentes familiares negativos y a diferencia de la forma hereditaria, se presenta a una edad posterior. Las tecnicas de imagen utilizadas para confirmar el diagnostico y evaluar el estadiaje del tumor comprenden la ecografia ocular, tomografia computarizada y la imagen por resonancia magnetica. La importancia de la deteccion temprana y el tratamiento oportuno va a permitir una mayor sobrevida en el paciente. EnglishRetinoblastoma is the most common intraocular malignant neoplasm in pediatric age. It is diagnosed between the first and third year of life. It can affect one or both eyes, with the most characteristic clinical sign being leukocoria, followed by strabismus, nystagmus and red eye. Regarding its incidence, retinoblastoma is much more frequent in less industrialized, lower-class countries and in rural areas. It is considered an autosomal dominant disease, where both alleles of the RB1 gene are mutated, causing alterations in the cell cycle. There are two presentations of retinoblastoma, the inherited form which is associated with germline mutations in the retinoblastoma gene, known as RB1, this presentation usually occurs in young children, most cases are bilateral or multifocal. The non-inherited form is characterized by somatic mutations of RB1 in the tumor, they generally have a unilateral and unifocal disease, have a negative family history and unlike the inherited form, it occurs at a later age. The imaging techniques used to confirm the diagnosis and evaluate the tumor staging include ocular ultrasound, computed tomography and magnetic resonance imaging. The importance of early detection and timely treatment will allow greater survival in the patient.

Diagnostic delay leads to advanced forms of retinoblastoma, compromising survival. The aim of this study was to evaluate the knowledge of health professionals regarding this disease. This survey was carried out among health professionals in the Democratic Republic of the Congo (cities of Lubumbashi, Bukavu and Kinshasa) and Cote d'Ivoire (Abidjan). Health professionals were divided into 3 professional categories: 1) paediatricians and ophthalmologists, 2) other medical doctors, and 3) paramedics. A questionnaire adapted to each category was used. The response to each question was graded as correct or incorrect. Total knowledge of retinoblastoma for the entire questionnaire was evaluated according to a level of overall score for each health professional: score

Uveitis is one of the commonest causes of vision loss worldwide and its exact etiology is still not clarified in most patients. The current study is a trial to assess the efficacy of serum anti-Toxocara immunoglobulin G (IgG) by enzyme-linked immunosorbent assay (ELISA) as a diagnostic tool for ocular toxocariasis (OT) and to detect OT prevalence and the associated ocular manifestations in sera of patients with uveitis. One hundred and twelve patients (62 females and 50 males) with uveitis were diagnosed by ophthalmologists, radiologists and rheumatologists according to ocular manifestations, laboratory and radiological investigations. Serum anti-Toxocara IgG titers were determined by ELISA in sera of all patients. Our results revealed that OT is highly associated with intermediate and posterior uveitis. Children and young adult females, especially those residing in rural areas, complained mainly of diminution of vision in the left eye, with strabismus and leukocoria. At a cut-off value of 0.258, the sensitivity and specificity of IgG ELISA were 93.3% and 100%, respectively. In conclusion, at a novel cut-off value of 0.258 the serum anti-Toxocara IgG ELISA is predicted to be a diagnostic tool for OT regarding sensitivity and specificity. Also, it has potential importance in the interpretation and differential diagnosis of OT. Thus, serum anti-Toxocara IgG ELISA should be a routine test for screening of suspected cases.

Leukocoria in children should always raise the concern for retinoblastoma. However, a variety of non-neoplastic conditions can also present with leukocoria, including persistent fetal vasculature (PFV), a nonhereditary, congenital anomaly caused by a failure of the fetal intraocular vasculature to regress during development. Classically PFV presents with features that make it easily distinguishable from retinoblastoma, including microphthalmia, retrolental fibrovascular membrane, central dragging of ciliary processes, and cataract. We present an atypical case of PFV in a 9-month-old boy who presented with the unusual features of axial myopia and platyphakia.

Retinoblastoma can present in 1 or both eyes and is the most common intraocular malignancy in childhood. It is typically initiated by biallelic mutation of the RB1 tumor suppressor gene, leading to malignant transformation of primitive retinal cells. The most common presentation is leukocoria, followed by strabismus. Heritable retinoblastoma accounts for 45% of all cases, with 80% being bilateral. Treatment and prognosis of retinoblastoma is dictated by the disease stage at initial presentation. The 8th Edition American Joint Committee on Cancer (AJCC) TNMH (tumor, node, metastasis, heritable trait) staging system defines evidence-based clinical and pathological staging for overall prognosis for eye(s) and child. Multiple treatment options are available in 2018 for retinoblastoma management with a multidisciplinary team, including pediatric ocular oncology, medical oncology, radiation oncology, genetics, nursing, and social work. Survival exceeds 95% when disease is diagnosed early and treated in centers specializing in retinoblastoma. However, survival rates are less than 50% with extraocular tumor dissemination. We summarize the epidemiology, genetics, prenatal screening, diagnosis, classification, investigations, and current therapeutic options in the management of retinoblastoma.

Coats' Disease is an idiopathic condition of the eye affecting young children although it can be seen in adults. Most patients present early in life with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma. In this study we report a case of coat's disease in an young girl, and evaluate histopathological and clinical findings.

Purpose Retinoblastoma (Rb) is a potentially fatal intraocular malignancy in children, and hand-held optical coherence tomography (HH-OCT) can assist in submillimeter detection and monitoring after treatment of Rb. Retinoblastoma located in the macula, or those with chemoresistance, can be among the most difficult to manage. We describe HH-OCT features in a case of chemoresistant macular Rb that eventually responded to plaque radiotherapy after failing intravenous chemotherapy and intraarterial chemotherapy. Methods Observational case report. Results A 15-month-old girl with leukocoria was found to have Group D Rb in the right eye of 6-mm thickness and macular Group B Rb in the left eye of 4-mm thickness. She was treated with 6-monthly cycles of systemic intravenous chemotherapy and focal consolidation therapies to both eyes, with tumor regression in both eyes. However, macular tumor in the left eye demonstrated subsequent recurrence, from regressed thickness of 792 μm on HH-OCT to a dome-shaped hyperreflective retinal mass of >2000 μm thickness. Three cycles of intraarterial chemotherapy were sufficient for tumor regression down to 977 μm thickness on HH-OCT. Six months later, macular tumor in the left eye recurred again to >2000 μm thickness and necessitated plaque radiotherapy using apex dose of 35 Gy over 95.25 hours. Hand-held OCT confirmed rapid tumor regression to 722 μm after plaque treatment and regression remained stable at 6-month follow-up. Conclusion Hand-held OCT was critical in assessment of Rb after failed intravenous chemotherapy and intraarterial chemotherapy and later documenting regression after plaque radiotherapy. Hand-held OCT is vital in providing cross-sectional imaging and measurements of small macular and paramacular Rbs.

Purpose: Leukocoria is the most common presenting sign for pediatric eye disease including retinoblastoma and cataract, with worse outcomes if diagnosis is delayed. We investigated whether individuals could identify leukocoria in photographs (photoleukocoria) and examined their subsequent Internet search behavior. Methods: Using a web-based questionnaire, in this cross-sectional study we invited adults aged over 18 years to view two photographs of a child with photoleukocoria, and then search the Internet to determine a possible diagnosis and action plan. The most commonly used search terms and websites accessed were recorded. Results: The questionnaire was completed by 1639 individuals. Facebook advertisement was the most effective recruitment strategy. The mean age of all respondents was 38.95 ± 14.59 years (range, 18-83), 94% were female, and 59.3% had children. An abnormality in the images presented was identified by 1613 (98.4%) participants. The most commonly used search terms were: "white," "pupil," "photo," and "eye" reaching a variety of appropriate websites or links to print or social media articles. Conclusions: Different words or phrases were used to describe the same observation of photoleukocoria leading to a range of websites. Variations in the description of observed signs and search words influenced the sites reached, information obtained, and subsequent help-seeking intentions. Translational Relevance: Identifying the most commonly used search terms for photoleukocoria is an important step for search engine optimization. Being directed to the most appropriate websites informing of the significance of photoleukocoria and the appropriate actions to take could improve delays in diagnosis of important pediatric eye disease such as retinoblastoma or cataract.

Orbital tumors in children are characterised by a wide histological diversity due to the architectural complexity of the orbit. Several classifications may be proposed according to their location, histologic type and malignant or benign character. The most common clinical manifestation is the leukocoria. Diagnostic delay of these tumors, even if they are benign, may affect prognosis and lead to a loss of vision and/or a morphological deformation. Imaging plays an important role in positive diagnosis, in the differentiation between benign processes and potentially malignant processes, in the assessment of local and loco-regional staging and in follow-up monitoring under treatment. This study aimed to highlight the radiological features of primary intra-orbital tumors in children which are, in general, different from those of adults, by conducting a retrospective study of 40 medical records whose data were collected in the Division of Pediatric Radiology at the Mohammed VI University Hospital in Marrakech, Morocco over a period of 4 years (2014-2017).

The persistence of fetal vasculature (PFV) is an abnormal resorption in hyaloid vascular system. We report the case of a 2-year old child with evolutionary posterior form of bilateral vasculature presenting with leukocoria in the right eye. Clinical examination showed bilateral leukocoria, exotropia in the right eye and an exotropia in the left eye. Fundus examination of the right eye showed fibrovascular retinal band stretched to the ora serrata associated with total retinal detachment and peripheral pigment changes. Fundus examination of the left eye showed fibrovascular retinal band stretched from the papilla to the ora serrata, associated with partial inferior, anterior temporal detachment and peripheral pigment changes.

Objectives The number of enucleations and visual sequels due to retinoblastoma is high. The aim of this study was to evaluate the different diagnostic aspects and propose strategies that might improve the clinical management of this condition. Method A retrospective study was conducted on 38 patients with retinoblastoma studied genetically (29 unilateral, 9 bilateral). The evaluation included: age of onset, clinical signs, and time since onset, number of enucleations, time to diagnosis, and survival at 5 years. Results Leukocoria was the main clinical sign (present in 90% of cases). The mean diagnostic delay was 3.2 months. Among the unilateral cases, the eyes were enucleated in 76%, and 55% in the bilateral forms. Only one death was found among the 25 patients followed-up for at least 5 years. Conclusions Retinoblastoma diagnostic and treatment strategies need to be updated. Good coordination between pediatricians and ophthalmologists is essential for this. Its management in reference centers, which have the necessary technology and experience, should contribute to increase the rate of organ preservation.

We report a case of a 7-week-old boy with bilateral leukocoria and asymmetric microphthalmia who was found to have Norrie disease. Symmetrically hyperdense globes with no evidence of calcification were seen on CT scan. The MRI showed bilateral retinal hemorrhages resulting in conical vitreous chambers—narrow at the optic disc and widened toward the lens—characteristic of persistent fetal vasculature. Genetic evaluation revealed a previously undescribed mutation in the Norrie disease protein gene.

PURPOSE To present the clinical aspects and treatment outcomes of Coats disease in Saudi Arabia. METHODS A retrospective chart review was performed of 92 patients (97 eyes) diagnosed with Coats disease at King Khalid Eye Specialist Hospital from 1983 to 2010. RESULTS The most common presenting complaint was decreased visual acuity followed by strabismus and then leukocoria. Snellen visual acuity was 20/20 to 20/50 in 9 eyes (9%), 20/50 to 20/160 in 11 eyes (11%), 20/200-counting fingers in 29 eyes (30%), and hand motion to no light perception in 24 eyes (25%). Telangiectasia was located in the preequatorial area in 71 eyes (73%) and most commonly involved the temporal retina in 67 eyes (69%). In eyes with clear view to the fundus, quadrant involvement by telangiectasia had the following distribution: 1) quadrant (n = 36, 37%); 2) quadrants (n = 26, 27%); 3) quadrants (n = 8, 8%); and 4 quadrants (n = 15, 15%). Total retinal detachment was present at presentation in 28 eyes (29%) and neovascular glaucoma in 8 (8%). Based on the Shields classification, the eyes were Stage 1 (n = 1, 1%), Stage 2A (n = 7, 7%), Stage 2B (n = 23, 24%), Stage 3A1 (n = 26, 27%), Stage 3A2 (n = 12, 12%), Stage 3B (n = 16, 17%), Stage 4 (n = 11, 11%), and Stage 5 (n = 5, 1%). Stage 3A was the most commonly presented stage (39%). Primary management included cryotherapy (19%), laser photocoagulation (64%), intravitreal agents (9%), and surgical drainage (4%). Combination treatment was performed in 29% of eyes. Thirteen eyes (13%) were enucleated because of clinical suspicion of retinoblastoma or the presence of glaucoma. Factors that were associated with a poor visual outcome of 20/200 or worse included age less than 10 years (relative risk: 1.27), Stages 3 and 4 disease (relative risk: 1.40), presence of subretinal fluid in all 4 quadrants including the fovea (relative risk: 14.25), and initial visual acuity of 20/200 (relative risk: 6.72) or worse (P < 0.005 for all factors). CONCLUSION Although rare, Coats disease is usually advanced at presentation and has a poor visual prognosis in the Saudi population, like in other populations. The findings of this study give validity and applicability to the Shields staging scheme, which we recommend using in clinical practice.

Purpose: To report a case of Coats' disease in which spontaneous reattachment occurred after total retinal detachment. Patient and Methods: A young boy (patient age: 4 years and 11 months) presented with leukocoria in the left eye. Slit-lamp examination revealed total retinal detachment with abnormal retinal blood vessels and subretinal exudation just behind the lens. Computed tomography imaging showed no solid mass lesion in the intraocular space. Secondary total retinal detachment as a complication of Coats' disease was diagnosed. No light perception was detected, so we determined that surgical treatment was not indicated. Results: Four months after the initial diagnosis, the retina showed complete reattachment with a large amount of subretinal hard exudate. Visual acuity remained unchanged, with no light perception. Conclusions: We speculate that the spontaneous retinal reattachment in the present case was caused by the decreased permeability of the abnormal retinal vessels and the good functional effect of the retinal pigment epithelium.

Objective To report the results of the multidisciplinary management of patients with retinoblastoma, including survival, enucleation rate, and systemic chemoreduction success. Methods A retrospective study was conducted on 27 patients (37 eyes) diagnosed with retinoblastoma, and treated by a multidisciplinary team in San Juan de Dios Hospital. Demographic information, clinical characterization, survival, local and systemic treatments were included in the analysis. Patients treated with intra-arterial chemotherapy (IAC) were also reviewed. Results The study included 14 male patients (52%). The median of age at presentation was 8 months (0.16–90). The median follow-up time was 33 ± 21 months. The diagnosis was made in 10 (37%) cases after 15 months old, with a median of 35 months (24–90). 17 (63%) patients had unilateral retinoblastoma, and 10 (37%) bilateral retinoblastoma. Leukocoria, isolated or associated with other signs, was the most frequent reason for referral (63%). Global enucleation rate was 57% (n = 21), being the primary treatment in 15 (55%) patients. Enucleation rate in unilateral retinoblastoma was 76.5%, and for bilateral retinoblastoma, it was 60% for one eye and 10% for both. Systemic chemotherapy was prescribed in 17 (63%) patients, with a mean number of cycles of 5.3 ± 2.1. The overall success of chemoreduction and focal therapy in order to avoid external radiotherapy and/or enucleation was 68%. Three patients were treated with IAC as a salvage therapy, controlling the tumor in 2 patients at 6 months of follow-up. These are the first cases reported in Chile. Survival rate was 100%. Conclusion Multidisciplinary management of retinoblastoma led to a survival rate and morbidity comparable with international reports.

Background: Congenital Cataract is the most important cause of treatable childhood blindness. Rubella is one of the major causes of preventable disease in many countries. There are scanty reports on congenital cataract in Nepal. Objective: To find out the demographic and etiological factors of congenital cataract in children. Method: In a hospital based cross sectional study, 46 children with congenital cataract were evaluated to find out morphology of cataract, laterality, associated ocular and systemic abnormality, visual status and etiology of cataract. Assessment included antenatal, birth and neonatal history, a detailed eye examination in slit lamp or the operating microscope under general anaesthesia, serum serology for TORCH infections, random blood sugar, urine reducing substance and thyroid profile. Result: Among 46 children with congenital cataract, 76.1% children presented before 5 years of age and 78.2% had bilateral onset. Male to female ratio was 1.3:1. Most of the children were legally blind (79.3%) in cataractous eye. Family history of congenital cataract was present in 15.2% cases. The most common mode of presentation was leukocoria in 91.3%. Microcornea (28.3%), resolved uveitis (13.0%), and iris atrophy (8.7%) were the most common ocular associations. Delayed developmental milestone (21.7%) and cardiac anomalies (10.9%) were the most common systemic anomalies. Lamellar cataract (51.3%) was the most common morphology of cataracts observed. The maternal infection was the major cause of congenital cataract in 17.4% cases with predominantly rubella infection in 13% cases. Conclusion: Most of the children with cataract are legally blind. Maternal infection in the antenatal period is the major cause of congenital cataract. DOI: http://dx.doi.org/10.3126/hren.v12i1.11975 Health Renaissance 2014;12(1):3-10

A 3-month wild rabbit was presented for examination of ocular opacities in the left eye. A complete bilateral ocular examination including slit-lamp examination, indirect ophthalmoscopy, tonometry, and ultrasound biomicroscopy was performed. Biomicroscopy of the lens of the left eye showed a retrolental fibrovascular membrane causing leukocoria. The opacity prevented biomicroscopy of the vitreous and funduscopy OS. No other disorder was present in either eye. Ultrasound examination did not show any difference between the right and left eye. Histopathological examination showed a 50-μm thick, preretinal, retrolental, nonpigmented, fibrovascular tissue. Posterior synechiae were present, but no other lesion of the posterior segment was found in this eye. These ocular abnormalities are consistent with a persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous (PHTVL/PHPV), similar to those described in other species.

Retinoblastoma is the most common malignant tumour of the eye in children and accounts for 3% of all childhood cancers. It can occur either unilaterally or bilaterally and generally develops before the first five years of life. A red reflex test must be performed if a child presents with any of the following: a white reflex (leukocoria) or an abnormal reflex in flash photographs; a recently onset squint (strabismus); a change in colour to the iris; deterioration in vision; a red, sore or swollen eye without infection. The presentation of any of these signs or symptoms in isolation can indicate retinoblastoma. However, it is vital to note that a child with retinoblastoma may appear well, apart from one or more of these signs. This article gives detailed instruction on how to carry out a red reflex test and appropriate referral information for suspected cases. A swift referral pathway is vital to reduce loss of vision and the risk of mortality.

We aim to report an uncommon presentation of retinoblastoma and its management. A 4-yearold boy presented with left eye pain for 1 week with eye redness after being hit by a ball. On examination, the visual acuity in his right eye was 6/6 and left eye was no perception to light. The conjunctiva in his left eye was injected and cornea was edematous. There was pseudohypopyon inferiorly. Lens was opaque and there was no fundus view. Intraocular pressure was 50 mmHg. Patient was initially treated as phacolytic glaucoma with severe inflammation. B scan showed intralesional calcification with retinal detachment. CT orbit showed left intraocular mass with calcification and optic nerve involvement. Thus, our final diagnosis was left eye retinoblastoma. Patient was performed a enucleation with chemotherapy in left eye. One year after the treatment, there was no local and distant invasion of the tumor. Anterior inflammation is a rare form of masquerade syndrome associated with retinoblastoma.

Purpose The study reports an unusual presentation of a young female patient presenting with a granulomatous posterior pole mass and profound vision loss secondary to infection with Bartonella henselae. Methods A single case report in a child. Results An 8-year-old female presented with a recent history of flu-like illness associated with profound vision loss, panuveitis and leukocoria in the left eye. She was found to have a posterior granulomatous mass associated with an exudative retinal detachment presumed as a toxocara granuloma. Magnetic Resonance Imaging ruled out retinoblastoma. Lab work done was negative for toxocariasis and positive for Bartonella henselae titers. She was treated for Cat Scratch Disease (CSD) with steroids and azithromycin. With treatment, the inflammation and exudative retinal detachment resolved, however, the patient had no improvement in visual acuity. Conclusions Young patients presenting with leukocoria need a full work up, which includes ruling out retinoblastoma. CSD can present as a granulomatous mass similar to toxocariasis, which can rarely lead to debilitating and irreversible vision loss.

Background Incontinentia pigmenti is a rare X-linked dominant syndrome caused by mutation in the NEMO/IKKgamma gene, and characterized by a spectrum of cutaneous, ocular, neurologic and dental abnormalities. In the eye, findings include retinal vascular non-perfusion, occasionally with traction retinal detachment, retinal fibrosis, and retinal pigment epithelium defects. These findings can resemble retinoblastoma, especially when vitreoretinal fibrosis produces leukocoria. Case report A 2-month-old girl born full-term presented with leukocoria, suspicious for retinoblastoma. She was found to have an ischemic retrolental fibrovascular retinal detachment. In addition, there was linear cutaneous hyperpigmentation, diagnostic of incontinentia pigmenti. Conclusions Retinoblastoma can be a challenge to diagnose. There are numerous simulating lesions that can present with leukocoria and retinal detachment, including incontinentia pigmenti. Recognition of the cutaneous features of incontinentia pigmenti contributes to early detection of related ophthalmologic, neurologic and dental abnormalities.

Purpose: Retinoblastoma (RB) is a common paediatric intraocular tumour. In the developed world, RB accounts for

A 13-month-old female child was brought to our tertiary eye hospital with a history of a rapidly enlarging mass in the right eye for 4 months. Her medical history was suggestive of leukocoria in the right eye, noted 6 months ago, for which medical attention was sought elsewhere. At that time, a diagnosis of group E retinoblastoma in the right eye was made. The left eye was normal. The caretakers were explained about …

A 9-month-old male infant with a history of Down syndrome underwent bilateral medial rectus recession. Two weeks postoperatively, he developed leukocoria of the left eye with a white opacity posterior to the lens, numerous undulations, necrosis, and hemorrhages in the retrolental space. His contralateral eye had white retinal lesions nasally. Ultrasound examination of the left eye showed a funnel retinal detachment, loculated debris, and a small, central, hyperechoic area concerning for calcification. He underwent enucleation because retinoblastoma could not be definitively ruled out. Given this patient's low visual potential, enucleation was a definitive and safe treatment option. Ocular pathology showed significant inflammation and necrosis. There was an area of scleral perforation by a suture adherent to the retina and vitreous, surrounded by inflammatory cells. This case uniquely demonstrates pediatric endophthalmitis following strabismus surgery, secondary to scleral perforation confirmed by histopathological analysis. Care must be taken during scleral passage of sutures to prevent inadvertent scleral perforation and the potential complication of endophthalmitis. [ J Pediatr Ophthalmol Strabismus . 2017;54:e37–e41.]

Persistent fetal vasculature (PFV) is a rare congenital developmental malformation of the eye. It is caused by the incomplete regression of the fetal hyaloid vasculature. It is a rare and sporadic disease with an overall prevalence of 0.064%. Persistent fetal vasculature, previously referred to as persistent hyperplastic primary vitreous (PHPV), is usually characterized by microphthalmia, a shallow anterior chamber, elongated ciliary processes, a posterior subcapsular cataract and a fibrovascular stalk that extends from the optic disc to the lens.This case presented with a history of poor vision on the right eye. On ophthalmologic examination, no light perception was noted in the affected eye. Slit-lamp findings for both eyes were unremarkable. There was no note of microphthalmia, shallow anterior chamber nor was there a subcapsular cataract. On dilated funduscopy of the affected eye, a fibrovascular stalk was seen emanating from the optic nerve. This was further documented with ocular ultrasound. Based on the clinical findings, a diagnosis of persistent fetal vasculature, posterior type, was made. Since the affected eye had no visual potential, a conservative mode of management was instituted. Persistent fetal vasculature is a rare and sporadic disease, which usually presents with microphthalmos, microcornea and leukocoria. Isolated posterior forms of PFV only accounts for 11% of persistent fetal vasculature cases in the literature.This documented case of a solely posterior type of persistent fetal vasculature, could somehow contribute to the small amount of information on this diseases entity. This, in turn, would eventually enable us to identify and formulate a more successful management strategy

Background: Ophthalmic pathology is a subspecialty of cellular pathology which deals with the diagnosis and characterization of eye diseases. Ophthalmic pathologists study tissues excised by ophthalmologists through evisceration and enucleation and other small biopsies from various ophthalmic surgeries. Ophthalmic lesions include a wide spectrum of lesions ranging from benign, premalignant to malignant lesions. The diagnosis of these lesions is based on the clinical as well as histopathological features. Methods: The present study was an observational prospective study conducted on a 62 ophthalmic biopsies received in Histopathology laboratory, Department of Pathology, Pt. J.N.M. Medical College and Dr. B.R.A.M. Hospital, Raipur, Chhattisgarh, India, between March 2016 and October 2016. Institutional ethics committee of Pt. J.N.M. Medical College, Raipur, India has approved this study. Informed written consent was collected from the patients or their relatives. Results: Out of 62 cases of ophthalmic biopsies, 38 (61.29%) were non-neoplastic and 24 (38.71%) were neoplastic lesions. Among non-neoplastic lesions, inflammatory lesions of lacrimal sac were more common 16 (42%) followed by conjuctival lesions 10 (26.3%). Among the neoplastic lesions, retinoblastoma was the most common malignancy 11 (45.83%). Conclusions: The most common ocular malignancy was retinoblastoma; among non-neoplastic disorder, chronic dacryoadenitis followed by benign lesions of conjunctiva. Some ocular masses composed of rhino sporangium involving conjunctiva, lid or lacrimal sac.

Stage 5 represents the most advanced stage of retinopathy of prematurity (ROP). Children present with a total retinal detachment, limited or no vision, and oftentimes with leukocoria due to the visibility of fibrous proliferation immediately posterior to the lens. As a result, the disease was first described with the term “retrolental fibroplasia” [1]. Since that time, we have learned a great deal about this blinding condition. Here we will review the pathogenesis, management, and prevention of stage 5 ROP.

Background: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia. Materials and Methods: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications. Results: The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012. There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majority of patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%). Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It was found that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis (12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%). More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients. Conclusions: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

Retinoblastoma is the most common intraocular malignancy in children, with a reported incidence ranging from 1 in 15,000 to 1 in 18,000 live births. It is second only to uveal melanoma in the frequency of occurrence of malignant intraocular tumors. Pawius described retinoblastoma as early as in 1597 referred to the tumor as fungus hematodes and suggested enucleation as the primary mode of management. The discovery of ophthalmoloscope in 1851 facilitated recognition of specific clinical features of retinoblastoma. Initially thought to be derived from the glial cells, it was called a glioma of the retina by Virchow (1864). Flexner (1891) and Wintersteiner (1897) believed it to be a neuroepithelioma because of the presence of rosettes. Later, there was a consensus that the tumor originated from the retinoblasts and the American Ophthalmological Society officially accepted the term retinoblastoma in 1926. Retinoblastoma was associated with near certain death just over a century ago. There has been a dramatic change in the overall management of retinoblastoma in the last decade. Specific genetic protocols have been able to make pre natal diagnosis of retinoblastoma. Early diagnosis and advancements in focal therapy have resulted in improved eye and vision salvage. This article explains the complexity of retinoblastoma, genetic association, clinical features, management and prognosis.

History This patient was a 20-month-old full-term girl who had not received any routine pediatric care. During a physical examination, left-sided leukocoria was detected. Subsequently, a left-sided cataract was diagnosed. The patient was sent for magnetic resonance (MR) imaging of the brain and orbits.

Tujuan penelitian ini ialah menilai penyebab leukokoria pada pasien anak di RSUP Dr. M. Djamil Padang dengan rancang studi cross sectional. Data dikumpulkan dari medical record pasien dengan riwayat leukokoria atau pupil putih yang berusia kurang dari 13 tahun di bagian mata RSUP Dr M Djamil Padang periode Oktober 2009 Februari 2011. Diagnosis banding disingkirkan melalui pemeriksaan USG dan patologi. Dari 36 pasien dengan leukokoria, 21 orang (58,3%) laki-laki dan umur rata-rata 31,3 bulan. Penyebab leukokoria adalah retinoblastoma 12 pasien (33,4%), tiga diantaranya bilateral, katarak kongenital 11 pasien (30,5%), delapan diantaranya bilateral, katarak traumatika 5 pasien (13,8%), uveitis intra uterin 2 pasien (5,5%), keduanya bilateral, dan ROP 2 pasien (5,5%), satu diantaranya bilateral. Kami juga menemukan penyebab lain, masing-masing satu pasien dengan leukokoria unilateral, antara lain: PHPV (2,7%), endoftalmitis eksogen (2,7%), uveitis anterior (2,7%), dan glioma nervus optikus (2,7%). Sangat krusial mengetahui perbedaan gambaran klinik retinoblastoma dan katarak kongenital, karena pengobatannya berbeda, dan retinoblastoma mengancam kehidupan, sedangkan katarak kongenital mengancam penglihatan.AbstractThe objective of this study was to determine the etiology of leukocoria in children using cross sectional study conducted in eye clinic of Dr. M. Djamil Hospital Padang. Samples were taken from medical records of patients with children leukocoria during the period of October 2009 to February 2011. Differential diagnosis eliminated by USG and pathology examination. There were 36 children with leukocoria in total, 21 of them were boys (58,3%). The mean age was 31.3 months old. The causes of leukocoria were retinoblastoma in 12 patients (33,4%) three of them were bilateral, followed respectively by congenital cataract in 11 patients (30.5%) which 8 of them were bilateral, traumatic cataract in 5 patients (13.8%), intrauterine uveitis in 2 patients (5,5%) both were bilateral, and ROP in 2 patients (5,5%) which one of them was bilateral. We also found one patient each with unilateral leukocoria for following causes: PHPV (2.7%), exogen endophthalmitis (2.7%), anterior uveitis (2,7%), and optic nerve glioma (2.7%). Thus, awareness and clinical knowledge to differentiate retinoblastoma and congenital cataract are crucial for appropriate treatment, since retinoblastoma can cost patient’s life while congenital cataract can threat sight condition.

Retinoblastoma is the most common intraocular malignancy of infancy with an incidence of 1/15,000 to 1/20,000 births. Sixty percent of retinoblastomas are unilateral, with a median age at diagnosis of two years, and in most cases are not hereditary. Retinoblastoma is bilateral in 40% of cases, with an earlier median age at diagnosis of one year. All bilateral and multifocal unilateral forms are hereditary and are part of a genetic cancer predisposition syndrome. All children with a bilateral or familial form, and 10 to 15% of children with an unilateral form, constitutionally carry an RB1 gene mutation. The two most frequent symptoms revealing retinoblastoma are leukocoria and strabismus. Diagnosis is made by fundoscopy, with ultrasound and magnetic resonance imaging (MRI) contributing both to diagnosis and assessment of the extension of the disease. Treatment of patients with retinoblastoma must take into account the various aspects of the disease (unilateral/bilateral, size, localization…), the risk to vision and the possible hereditary nature of the disease. The main prognostic aspects are still premature detection and adapted coverage by a multi-disciplinary specialized team. Enucleation is still often necessary in unilateral disease; the decision for adjuvant treatment is taken according to the histological risk factors. The most important recent therapeutic advances concern the conservative treatment which is proposed for at least one of the two eyes in most bilateral cases: laser alone or in combination with chemotherapy, cryotherapy or brachytherapy. Recently, the development of new conservative techniques of treatment, such as intra-arterial selective chemotherapy perfusion, aims at preserving visual function in these children and decreasing the number of enucleations and the need for external beam radiotherapy. The vital prognosis related to retinoblatoma is now excellent in industrialized countries, but long-term survival is still related to the development of secondary tumors, mainly secondary sarcoma. Retinoblastoma requires multi-disciplinary care as well as a long term specialized follow-up. Early counseling of patients and their family concerning the risk of transmission of the disease and the risk of development of secondary tumors is necessary.

Leukocoria or white pupillary reflex in infancy or childhood is mostly associated with retinoblastoma (an intraocular malignancy). The possibility is increased if buphthalmos is also present, because non-malignant conditions like persistent hyperplastic primary vitreous (PHPV) usually have normal or small eyes. They rarely present with enlarged buphthalmic eye. Such was the condition in our neonate who presented with buphthalmos and leukocoria and was referred to us for management having been diagnosed with retinoblastoma. However, on investigating by imaging techniques (ultrasonography, Doppler and CT Scan) PHPV was confirmed, which is non-malignant. The patient was managed accordingly without enucleation. Even though rare, neonates or infants presenting with buphthalmos and leukocoria, should be investigated for non-malignant conditions like PHPV by imaging techniques to rule out a malignant condition – retinoblastoma.

We report a unique case of bilateral intraocular calcification due to necrotizing cytomegalovirus (CMV) retinitis associated with congenital CMV infection. A 7-month-old boy with a history of congenital CMV infection showed bilateral intraocular calcific plaques on computed tomography (CT) and ultrasonography. We reviewed the patient's medical files for the purpose of this report. The patient had a prior medical history of hospitalization for fever and swelling in the neck at 3 months of age. Systemic findings (anemia, neutropenia, hepatosplenomegaly, and reactive lymphadenomegaly) in association with a low CD4 count, high blood CMV viral load, and positivity for urine CMV DNA by polymerase chain reaction led to the diagnosis of bone marrow suppression and congenital CMV infection. At 7 months, he developed horizontal nystagmus and bilateral leukocoria over 20 days. Cranial CT and ultrasonography revealed bilateral intraocular calcific plaques and the patient was referred to rule out retinoblastoma. Fundoscopy was consistent with bilateral hemorrhagic, necrotizing CMV retinitis. Significant resolution of the retinal infiltrations occurred 2 weeks after initiation of systemic treatment with ganciclovir. Intraocular calcification may be a sign of active CMV retinitis. To our knowledge this is the first report of bilateral intraocular calcification serving as the presenting clinical manifestation of necrotizing CMV retinitis.

The parents of a 2 year old boy notice a difference in appearance of his two eyes in a recent flash photograph, and they alert their paediatrician. A white red reflex is ‘leukocoria’—‘leukos’ Greek for white and ‘core’ for pupil. Evaluation of the red reflex is a useful screening test that should not be missed from the routine paediatric examination. It is quick, non-invasive and only needs a bright direct ophthalmoscope. An asymmetrical red reflex—with abnormal colour, size, shape or positioning—can indicate need for urgent ophthalmology referral. In children, leukocoria can represent a wide range of underlying pathologies, intraocular and systemic. Having the confidence to conduct a thorough history and examination is crucial to reach a differential diagnosis and exclude rare sight and life-threatening conditions. Swiss ophthalmologist Bruckner in 19621 described the red reflex as a screening tool in infants and children. The beauty of using the red reflex screening tool is that it is non-invasive and can be done rapidly, without even touching an apprehensive child. Furthermore, it can be easily learnt and is not a skill reserved solely for specialists. A study in 1995 by Gole and Douglas2 demonstrated that the transillumination of the red reflex by non-ophthalmologists has a high sensitivity for detecting abnormalities. Anyone who has been behind a flash camera has probably generated a red reflex. The light travels through the cornea, aqueous humour, lens and vitreous body and reflects off the chorio-retinal sheen and vasculature. To elicit the reflex, darken the room to allow the subject's pupils …

Purpose: Seasonal Hyperacute Panuveitis (SHAPU) is a mysterious but rapidly devastating intraocular inflammatory disease reported only from Nepal during odd years after monsoon. Though it predominantly affects children, it has been reported between 9 months and 50 years age. But herein, we report a case of SHAPU in a 38-days-baby during 2017 SHAPU outbreak. Observations: Parents of a 38-days-healthy male baby from Pokhara noticed redness in child's right eye since 5 days (October 2017). They noticed the fall of a white moth from the tubelight over the child's face 1-day prior to symptom onset. On examination, both eyes followed and fixated at light. The right eye was congested with dense anterior chamber reaction and white pupillary reflex obscuring the glow of the retina. Ultrasonography showed hyperechoic vitreous shadows with choroidal thickening. Left eye was normal. The child underwent vitreous tap with core vitrectomy + lensectomy with intravitreal antibiotics and steroid ↓GA. The blood investigations were normal and the vitreous sample yielded no organism growth. Viral PCR analysis was negative. The intraocular inflammation decreased and fundal glow was visible. Conclusions: AND IMPORTANCE: SHAPU can occur in a child as young as 38 days. Link with moth remains the strongest suspect. Keywords: Intravitreal injection, Leukocoria, Moth, SHAPU, Vitrectomy

PURPOSE: The purpose was to study the clinical features, treatment, and outcome of retinoblastoma (RB) in neonates. METHODS: This was a retrospective study of five patients with RB detected in the 1st month of life. RESULTS: The mean age at diagnosis of RB was 22 days (median, 24 days; range, 14–28 days). There were one female and four males with neonatal RB. Two patients had a known family history of RB, and the tumor was detected by routine fundus screening in these children. Two patients were brought in with complaints of leukocoria and one patient with complaints of red-eye. All patients had an intraocular tumor at presentation. Four patients had bilateral involvement, whereas only one had unilateral involvement. Based on the International Classification of Intraocular Retinoblastoma, the tumors were classified as Group A (n = 2), Group B (n = 3), Group C (n = 1), Group D (n = 1), and Group E (n = 2). Macular involvement was noted in 6 (67%) eyes. The primary treatment included systemic chemotherapy with/without focal treatment in all patients. One patient subsequently underwent secondary enucleation as the globe became phthisical. One child died while on treatment due to pneumonia secondary to chemotherapy-induced neutropenia. Of the four patients who completed treatment, globe salvage was achieved in 6 (86%) eyes over a mean follow-up period of 89 months (median, 92 months; range, 29–144 months). CONCLUSION: Neonatal RB though rare, if detected early, has a favorable outcome of ocular and life salvage. Sporadic RB can occur in neonates, and a family history may not always be elicited.

Objectives: This study aims to determine the incidence, pattern, and problems of management of retinoblastoma at the Guinness Eye Center, Onitsha, Nigeria. Materials and Methods: Case files of all patients with histology-proven retinoblastoma between October 2012 and September 2017 were reviewed. Information obtained included age, sex, disease duration, presenting features, and treatment. Results: There were 31 patients, 14 males (45.2%) and 17 females (54.8%), who had the disease in 38 eyes; age range was 5 months to 6 years; and median was 2.4 years. Disease duration was 3 weeks to 3 years and median was 8.8 months. Seven patients (22.6%) had bilateral disease, 11 (35.5%) had the disease only in the right eye, whereas 13 (41.9%) had the disease in the left eye; 32 (84.2%) eyes had no visual potential at presentation. The most common clinical features were leukocoria in 23 (60.5%) eyes and proptosis in 20 (52.6%). While all patients with advanced disease had enucleation or modified exenteration, only 15 (48.4%) accepted chemotherapy with two completing the full course and five not returning after the first course. Five (16.1%) had distant metastasis, namely, brain 2 (6.5%) and orbit, jaw, and skull 1 (3.2%) each. Conclusions: Retinoblastoma is seen at the Guinness Eye Center at the rate of about one case in 2 months. Most patients present late with proptosis and, in some cases, fungating necrotic tumor mass with distant metastases. There is a high default rate with treatment as only two patients completed the full course of chemotherapy.