Importance of Baseline Fluorescein Angiography for Patients Presenting to Tertiary Uveitis Clinic.

PURPOSE: To ascertain whether the use of ultra-wide-field fluorescein angiography (UWFFA) at baseline visit alters the assessment of disease activity and localization, as well as the management of patients presenting to a tertiary uveitis clinic. DESIGN: Retrospective comparison of diagnostic approaches. METHODS: Baseline visits of 158 patients who presented to the Uveitis Clinic at the Byers Eye Institute at Stanford between 2017 and 2022 were evaluated by three uveitis-trained ophthalmologists (I.K., A.B., and H.G.). Each eye had undergone clinical examination along with ultra-wide-field fundus photography (UWFFP) (Optos Plc, Dunfermline, Scotland, UK), spectral-domain optical coherence tomography (SD-OCT, Spectralis Heidelberg, Heidelberg Engineering, Heidelberg, Germany) and UWFFA (Optos Plc, Dunfermline, Scotland, UK) at the baseline visit. Investigators were asked to successively determine disease activity, localization of disease (anterior, posterior or both), and management decisions based on clinical examination and UWFFP and SD-OCT (Set 1) and Set 1 plus UWFFA (Set 2). The primary outcome was the percentage of eyes whose management changed based on the availability of UWFFA, compared with Set 1. RESULTS: The mean age of the patients was 46.9±22.4 (range, 7-96) and 91 (57.6%) were female. With Set 1 alone, 138 (55.2%) eyes were found to have active disease; localization was anterior in 58 (42.0%) eyes, posterior in 53 (38.4%) eyes and anterior + posterior in 27 (19.6%) eyes. With Set 2, 169 eyes of 107 patients had active anterior, posterior or pan-uveitis. In comparison with Set 1, assessment with Set 2 identified additional 31 (18.3%) eyes with active disease (p=0.006), and additional 31 (18.3%) eyes having disease in both anterior + posterior segments (p<0.001). Regarding the primary outcome, management was changed in 68 (27.4%) eyes in Set 2, compared to Set 1. CONCLUSION: Baseline UWFFA may alter assessment of disease activity, localization, and management decisions compared to clinical examination with only UWFFP and SD-OCT for eyes with uveitis. Thus, UWFFA may be considered as an essential tool in the evaluation of uveitis patients at the baseline visit.

A practical model for effective eye care delivery in Southeast Asian rural communities: A proposal built based on experts' recommendations.

PURPOSE: To evaluate rural community-based eye care models from the perspective of community ophthalmology experts and suggest sustainable technological solutions for enhancing rural eye care delivery. METHODS: A semi-structured descriptive survey, using close-ended and open-ended questions, was administered to the experts in community ophthalmology sourced through purposive sampling. The survey was self-administered and was facilitated through online platforms or in-person meetings. Uniform questions were presented to all participants, irrespective of their roles. RESULTS: Surveyed participants (n = 22 with 15 from India and 7 from Nepal) in high-volume tertiary eye hospitals faced challenges with resources and rural outreach. Participants had mixed satisfaction with pre-operative screening and theatre resources. Delayed presentations and inexperienced surgeons contributed to the surgery complications. Barriers to rural eye care included resource scarcity, funding disparities, and limited infrastructure. In rural/peri-urban areas 87% of participants agreed with providing primary eye care services, with more than 60% of the experts not in agreement with the makeshift center model of eye care delivery. Key components for an effective eye care model are sustainability, accessibility, affordability, and quality. These can be bolstered through a healthcare management platform and a human-chain supply distribution system. CONCLUSION: Tailored interventions are crucial for rural eye care, emphasizing the need for stronger human resources, optimized funding, and community awareness. Addressing challenges pertinent to delayed presentation and surgical training is vital to minimizing complications, especially with advanced cataracts. Enhancements in rural eye care demand a comprehensive approach prioritizing accessibility, affordability, and consistent quality.

Challenges for further successful development of tumor necrosis factor targeting therapies for uveitis.

INTRODUCTION: Uveitis is a heterogeneous group of ocular conditions characterized by inflammation of the uveal tract and is one of the leading causes of vision impairment. In developed countries, noninfectious uveitis (NIU) represents most cases and is challenging to treat due to its severity, chronicity, and high recurrence rates. The advent of anti-tumor necrosis factor-α (anti-TNF-α) agents have dramatically improved outcomes and changed treatment paradigms in NIU. AREAS COVERED: The index article summarizes the present experience of anti-TNF-α agents in NIU pharmacotherapy and highlights the barriers to further research and development of anti-TNF-α agents for uveitis. Common challenges faced in NIU clinical drugs trials, specific difficulties in anti-TNF-α drug development, and promising competitor drug candidates are discussed and evaluated. EXPERT OPINION: Anti-TNF-α agents have revolutionized NIU pharmacotherapy and greatly improved outcomes with good safety profiles. The great success of systemic infliximab and adalimumab in NIU treatment has resulted in little impetus for further development of this class of medication. Attempts have been made to deliver anti-TNF-α agents intravitreally but that has not been successful thus far. With expiring patents, competition from biosimilars and newer, novel molecules, it may not be viable to continue pursuing anti-TNF-α drug development.

The Role of Corticosteroids and Immunomodulatory Therapy in the Management of Infectious Uveitis.

PURPOSE: The index review aims to provide an update on the role of corticosteroids and steroid-sparing immunomodulatory therapy (IMT) in managing patients with infectious uveitis. METHOD: Narrative literature review. RESULTS: Corticosteroids and immunomodulatory therapy (IMT) focus on the host defense system instead of the pathogen, adjusting exaggerated inflammatory reactions to reduce potential harm to ocular tissues. Systemic or local corticosteroids are primarily selected as adjunctive medication for infectious uveitis. Concomitant corticosteroids have also been used in cases of paradoxical worsening in ocular tuberculosis and immune recovery uveitis in cytomegalovirus (CMV) retinitis. While there is no well-established evidence to support the use of IMT in infectious uveitis, it is occasionally used in clinical settings to treat persistent inflammation following resolution of infection such as cases of ocular tuberculosis and ocular syphilis where an insufficient response is observed with corticosteroids. CONCLUSION: There is no consensus on the position of immunomodulatory therapy in the management of infectious uveitis with different etiologies. The index review provides an overview of available adjunctive corticosteroids and IMT options to assist clinicians in managing such disease entities more efficiently.

Recent advances in uveitis therapy: focus on selected phase 2 and 3 clinical trials.

INTRODUCTION: Uveitis is a heterogeneous group of ocular conditions characterized by inflammation of the uveal tract. It is a leading cause of blindness in developed countries and exerts significant psychological, social, and economic impact on both patients and the larger society. While there are numerous pharmacotherapy options, posterior segment noninfectious uveitis remains a significant challenge to treat due to its severity, chronicity, and high recurrence rates. AREAS COVERED: The index review highlights the unmet needs of uveitis pharmacotherapy and its research and the shortcomings of existing ocular and systemic therapeutic options for noninfectious uveitis. The more promising novel ocular drug delivery methods and therapeutic targets/drugs are discussed, and evidence from the clinical trials is evaluated. EXPERT OPINION: There has been incredible growth in the number of treatment options available to uveitis patients today, especially with the new generation of biologic drugs. Available evidence suggests that these newer options may be superior to conventional immunosuppressive therapies in terms of efficacy and side effect profiles. Further high-quality research and additional clinical trials will be needed to clarify their roles in the stepladder treatment approach of noninfectious uveitis.

Structural and Functional Changes in Non-Paraneoplastic Autoimmune Retinopathy.

BACKGROUND: To describe longitudinal changes in patients with non-paraneoplastic autoimmune retinopathy (npAIR) by utilizing different diagnostic modalities/tests. METHODS: The index study is a retrospective longitudinal review of sixteen eyes of eight patients from a tertiary care eye hospital diagnosed with npAIR. Multiple diagnostic modalities such as wide-angle fundus photography (WAFP), WA fundus autofluorescence (WAFAF), spectral-domain optical coherence tomography (SD-OCT), Goldmann visual field (GVF) perimetry, microperimetry (MP), electrophysiologic testing, and adaptive optics scanning laser ophthalmoscopy (AOSLO) were reviewed and analyzed. RESULTS: At the baseline visits, anomalies were detected by multimodal diagnostic tests on all patients. Subjects were followed up for a median duration of 11.5 [3.0-18.7] months. Structural changes at the baseline were detected in 14 of 16 (87.5%) eyes on WAFP and WAFAF and 13 of 16 (81.2%) eyes on SD-OCT. Eight of the ten (80%) eyes that underwent AOSLO imaging depicted structural changes. Functional changes were detected in 14 of 16 (87.5%) eyes on GVF, 15 of 16 (93.7%) eyes on MP, and 11 of 16 (68.7%) eyes on full-field electroretinogram (ff-ERG). Multifocal electroretinogram (mf-ERG) and visual evoked potential (VEP) tests were performed in 14 eyes, of which 12 (85.7%) and 14 (100%) of the eyes demonstrated functional abnormalities, respectively, at baseline. Compared to all the other structural diagnostic tools, AOSLO had a better ability to demonstrate deterioration in retinal microstructures occurring at follow-ups. Functional deterioration at follow-up was detected on GVF in 8 of 10 (80%) eyes, mf-ERG in 4 of 8 (50%) eyes, and MP in 7 of 16 (43.7%) eyes. The ff-ERG and VEP were stable in the majority of cases at follow-up. CONCLUSIONS: The utilization of multimodal imaging/tests in the diagnosing and monitoring of npAIR patients can aid in identifying anomalous changes over time. Analysis of both the anatomical and functional aspects by these devices can be supportive of detecting the changes early in such patients. AOSLO shows promise as it enables the capture of high-resolution images demonstrating quantifiable changes to retinal microstructure.

Prognostic factors of open-globe injuries: A review.

Open-globe injuries (OGI) can lead to significant visual impairment. The Ocular Trauma Score (OTS) is the most widely recognized tool for predicting visual outcomes. This review aimed to identify prognostic factors and assess the effectiveness of the OTS in predicting visual outcomes. Twenty-one articles published on PubMed and Google Scholar were analyzed. Initial visual acuity and the zone of injury were found to be the most significant prognostic factors for OGI. Other significant prognostic factors include retinal detachment/involvement, relative afferent pupillary defect, vitreous hemorrhage, vitreous prolapse, type of injury, hyphema, lens involvement, and duration from incidence of OGI to vitrectomy. Of the 21 studies evaluated, 11 investigated the effectiveness of OTS. Four studies concluded that OTS was effective overall, while six studies suggested that it was only useful in certain OGI categories. Thus, there is a need for further research to develop an optimized ocular trauma prognosticating system.

Minimally invasive procedure for optic disc pit maculopathy: vitrectomy with scleral plug and analysis on pattern of resolution.

Optic disc pit maculopathy (ODP-M) is a rare complication of optic disc pit which can cause irreversible visual impairment. The aim of this study is to evaluate the anatomical and functional outcomes and pattern of resolution of ODP-M following vitrectomy with posterior vitreous detachment (PVD) induction and scleral tissue plug for treatment of ODP-M without ILM peeling, laser or use of long term gas/tamponade or head positioning. This retrospective study included 7 patients with ODP-M, meeting the inclusion criteria. Patients were followed up for 6 months. Complete anatomical success was defined as "Total resolution of all the fluid in retinal compartments". All of the patients had complete resolution of the optic pit maculopathy following surgery. The mean duration for complete resolution was 18.3 weeks. Pattern of resolution of ODP-M was found to be resolution of the subretinal fluid (SRF) followed by disappearance of the retinoschitic lesions (RL) and finally disappearance of macular edema (ME). The proposed minimally invasive procedure (MIP) can produce comparably good and equally reliable results for the treatment of ODP-M.

Seasonal Hyperacute Panuveitis (SHAPU) Outbreak Amidst COVID-19 Pandemic.

PURPOSE: To document the demographic profile of the SHAPU outbreak amidst the COVID-19 pandemic. METHODS: A multicentric cross-sectional study of the 2021 SHAPU outbreak during the second phase of the COVID-19 outbreak. RESULTS: A total of 135 patients were diagnosed with SHAPU from August to December 2021, 77 (57%) were children <16 years, males 54.8% and 34.8% had direct physical contact with white moths and 41.5% had severe type of SHAPU. Dramatic increment in the moth abundance was noted in these outbreak sites. Few cases presented with atypical ocular findings, unlike past outbreaks. Due to the ongoing COVID-19 pandemic with restrictions on travel and transportation, timely management was difficult and good visual outcome was achieved only in mild-moderate cases with an early presentation. CONCLUSION: The surge in the number of SHAPU patients, its occurrence in areas previously unreported, and some atypical presentation added raised suspicion of a possible link between COVID-19 and SHAPU.

Increase in SHAPU patients, incidence in unreported areas of Nepal, atypical ocular presentations and shift in disease affection from children towards adults population have raised doubt between connections between SHAPU, white moths and COVID pandemic.

The role of a multicentre data repository in ocular inflammation: The Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS).

In the current literature, clinical registry cohorts related to ocular inflammation are few and far between, and there are none involving multi-continental international data. Many existing registries comprise administrative databases, data related to specific uveitic diseases, or are designed to address a particular clinical problem. The existing data, although useful and serving their intended purposes, are segmented and may not be sufficiently robust to design prognostication tools or draw epidemiological conclusions in the field of uveitis and ocular inflammation. To solve this, we have developed the Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS) Clinical Registry. OASIS collects prospective and retrospective data on patients with all types of ocular inflammatory conditions from centers all around the world. It is a primarily web-based platform with alternative offline modes of access. A comprehensive set of clinical data ranging from demographics, past medical history, clinical presentation, working diagnosis to visual outcomes are collected over a range of time points. Additionally, clinical images such as optical coherence tomography, fundus fluorescein angiography and indocyanine green angiography studies may be uploaded. Through the capturing of diverse, well-structured, and clinically meaningful data in a simplified and consistent fashion, OASIS will deliver a comprehensive and well organized data set ripe for data analysis. The applications of the registry are numerous, and include performing epidemiological analysis, monitoring drug side effects, and studying treatment safety efficacy. Furthermore, the data compiled in OASIS will be used to develop new classification and diagnostic systems, as well as treatment and prognostication guidelines for uveitis.

RE-INVENTING CYANOACRYLATE RETINOPEXY IN THE 21ST CENTURY: LITERATURE REVIEW AND CASE SERIES.

PURPOSE: We report use of cyanoacrylate (N-butyl-Cyanoacrylate) in previously failed retinal reattachment surgeries for chorio-retinal colobomas. We report the surgical technique, its challenges, and long-term outcomes in three patients who underwent the surgery. METHODS: A chart review of patients with chorio-retinal colobomas and retinal detachment repair with cyanoacrylate at a tertiary eye care center in Nepal. Cyanoacrylate was used to seal colobomatous retinal breaks in eyes which had undergone multiple retinal surgeries with failed outcome. RESULTS: Three eyes that were operated using cyanoacrylate were included. All three patients had attached retina and none of the patients required a long-term tamponading agent. None of the patients underwent head positioning following the surgery. All of the patients had a visual acuity gain of 3/60 or more at the end of 8 months. No adverse or inflammatory reactions were noted. CONCLUSION: We demonstrate that cyanoacrylate is safe and less resource-demanding without a requirement of second surgery to remove a tamponading agent. It could be helpful in eyes with persistent retinal detachment in colobomatous eyes. Because we were able to achieve favorable outcomes without head positioning, we believe it may also be helpful in patients who are not suitable for positioning because of bodily or bony deformities and in retinal detachment with other coexisting trauma.

Causes and Managements of Early-Onset Ocular Hypertension Following Pars Plana Vitrectomy with Silicone Oil for Retinal Detachment and Exploration of Trabeculectomy as a Viable Alternative Management: A Pilot Study.

INTRODUCTION: This study aims to study a relatively unexplored topic about the causes and managements of early-onset ocular hypertension (OHTN) following the pars plana vitrectomy with silicone oil (PPV with SO) procedure for retinal detachment. Additionally, to explore the outcome of trabeculectomy in managing such patients. MATERIALS AND METHODS: This is a retrospective exploratory pilot study. We studied 23 patients who underwent the procedure then subsequently developed ocular hypertension within a month of the procedure. The probable causes for their early-onset ocular hypertension were identified and addressed with medicine, peripheral iridotomy (PI), complete or partial silicone removal. Trabeculoplasty was done in irretractable causes. This study aimed to evaluate the causes of early onset ocular hypertension after pars plana vitrectomy with silicone oil and explore the outcome of different managements including trabeculectomy. RESULTS: Inflammation (n=11, 47.8%) was the most common cause of early-onset ocular hypertension. Other causes were overfilling/spilling of silicone oil in anterior chamber (n=5, 21.7%), pupillary block (n=4, 17.4%) and angle-recession glaucoma (n=2, 8.69%). Majority of the cases responded to intraocular pressure (IOP) lowering medications (n=11). Three eyes with persistently high intraocular pressure underwent trabeculectomy after which the intraocular pressure was controlled. CONCLUSION: Even though prior studies have reported that trabeculectomy does not address late-onset ocular hypertension, our study shows that the procedure might be helpful in early-onset ocular hypertension. This is probably because at the time of presentation for early-onset ocular hypertension, silicone has not emulsified, which will not be the case in late-onset ocular hypertension. If a large study also shows that trabeculectomy can correct early-onset ocular hypertension, this information can guide the practices of ophthalmologists whose patients cannot afford expensive glaucoma drainage devices.

Panophthalmitis secondary to retained intraocular foreign body amidst a national lockdown during the COVID-19 pandemic: A case series and review of literature.

Introduction: Intraocular foreign bodies (IOFBs) can be serious as they may result in vision-threatening ocular inflammations and even loss of the eye. Delay in presentation or treatment by more than 24 hours from the time of injury results in a poor prognosis. In penetrating wounds, microorganisms enter the eye through penetrating objects. Both bacterial and fungal organisms are responsible for causing panophthalmitis. At the ocular level, these microorganisms produce irreversible damage which includes keratitis, uveitis, hypopyon, vitreous abscesses, retinal necrosis, detachment, and, finally, panophthalmitis. Case scenarios: In this case series, we report three cases of IOFB presenting with panophthalmitis secondary to delay in seeking medical attention. In our cases, there was a delay in the presentation by more than 24 hours of trauma. All cases had panophthalmitis at the time of presentation. In two cases, the causative organism was coagulase-negative staphylococci and in one case it was staphylococcus. Initially, we planned to manage them with intravitreous, intravenous and topical antibiotics till the inflammation subsides, then IOFB removal surgeries were planned. However, in two cases, the clinical presentation worsens with scleral necrosis. Therefore, they had to undergo evisceration. In one case, the antibiotics therapy was enough without IOFB removal surgery to manage her symptoms. All cases recovered uneventfully after the interventions. Discussion/Conclusion: In developing nations, like Nepal, transportation barriers can affect a person's access to health care services. This can be clearly explained from this case series as limited transportation options in rural regions are a major factor for all patients' delayed presentation to the hospital during the time of national lockdown in the second wave of the COVID-19 pandemic. The concerned authority must pay attention to solving such social determinants of health.

Classification of Seasonal Hyperacute Panuveitis (SHAPU).

INTRODUCTION: Classification are essential part of scientific methodology and has important role in medical reporting system. Even after having 46 years long history, Seasonal Hyperacute Panuveitis (SHAPU), the blinding diseases reported mainly from Nepal lacks a standard classification system. Thus, we aim to contribute in the ophthalmic nosology by purposing a classification system for SHAPU. METHODOLOGY: The classification is suggested on the background of prolonged experience of this entity by the group of investigators who have dedicated years of research on this topic. CONCLUSION: We are optimistic that the proposed classification system will help in effective planning and evaluation of this ocular emergency condition and deliver the appropriate and reliable information for timely management and prevention of complications.

Ophthalmic Trauma Correlation Matrix (OTCM): a potential novel tool for evaluation of concomitant ocular tissue damage in open globe injuries.

PURPOSE: To introduce a novel tool to investigate the correlation between concomitant injuries and primary open globe injury (OGI) in the setting of ophthalmic trauma, the "Ophthalmic Trauma Correlation Matrix" (OTCM). METHODS: Retrospective cohort review, performed at a tertiary referral eye care center in Eastern Nepal, involving all eyes with OGI meeting the inclusion criteria from 2015-2018. Clinical data including details of primary injury, concurrent injuries, and clinical course were noted from hospital medical records. A correlation matrix chart was devised using matrix correlation and Pearson's correlation coefficient. This chart was then used to evaluate the association of the various injuries in the setting of OGI. RESULTS: A total of 109 eyes with OGI were included. Majority of the eyes (78, 71.6%) had zone I injuries, while most of the eyes (66, 60.6%) had penetrating injury. The most frequent concomitant injuries in all zones of OGI were traumatic lens injury (77, 70.64%), followed by hyphema (48, 44.03%), and vitreous hemorrhage (35, 32.11%). The most common concomitant injury associated with zone I was hyphema (0.873), while traumatic subluxation/cataract (0.894) and vitreous hemorrhage (0.972) were commonly associated with zone II and III, respectively. CONCLUSIONS: OTCM could be a useful tool to manage injuries related to the primary ocular injury. This additional information will aid in the prognostication, planning, and management of OGI and potentially prevent repeat surgeries and inadequate treatments.

Redefining response in wet AMD to anti VEGF therapy based on non-OCTA versus OCTA evaluation.

PURPOSE: Anti vascular endothelial growth factor (anti VEGF) has been the mainstay of treatment in wet age-related macular degeneration (AMD). Subsequent decision to continue anti VEGF therapy depends on the treatment response quantified by functional (visual acuity) and morphological (optical coherence tomography) parameters then categorized from good to poor. METHODS: This study evaluates the agreement between OCT angiography (OCTA) and non-OCTA (logMAR VA plus OCT) to decide anti-VEGF treatment's continuity. After an anti VEGF treatment, on a follow up visit, a patient underwent non-OCTA evaluation (decision A) then OCTA evaluation (decision B) to judge the necessity of future anti VEGF application. RESULTS: Out of 129 eyes, on 72 eyes (49%), there were agreements on both decision arms, but on 55 eyes (42%) there was disagreement. Particularly, disagreement on 47/55 eyes was important, where OCTA advised "continue anti VEGF" and non-OCTA advised "Stop anti VEGF" therapy. Cohen's Kappa for probability of agreement to continue anti VEGF was fair (0.33) and to stop anti VEGF therapy was none (0.1). CONCLUSIONS: Based on resulting disagreements between the two modalities on deciding the continuity of anti VEGF, we conclude that OCTA must be considered in the conventional decision making algorithm in patients with wet AMD under anti VEGF therapy.

Taenia solium in the Anterior Chamber: Surgical Management of a Rare Case of Live Free-Floating Parasite.

Ocular cysticercosis is a preventable cause of blindness. It is a parasitic infestation caused by Cysticercus cellulosae-which is the larval form of Taenia solium. In 1829, Soemmering reported the first case of a live anterior chamber cysticercosis. In the following, we report a rare case of a 13-year-old male who presented with a live adult Taenia solium in the anterior chamber without any systemic features and its successful management.

Challenges of Pediatric Cataract Surgery in a Case of Seasonal Hyperacute Panuveitis (SHAPU).

A four-year-old female child diagnosed as a case of severe Seasonal Hyperacute Panuveitis (SHAPU) underwent lens-sparing core vitrectomy in her left eye with intravitreal antibiotic and steroid. Patient responded well to treatment and intraocular inflammation subsided. However, three months later, she developed vision impairing dense cataract which also made posterior segment assessment difficult. Lens aspiration with primary posterior capsulotomy and anterior vitrectomy with intraocular lens (IOL) implantation was performed. However, four weeks later, the patient developed occlusio pupillae with iris bombe. She did not respond to medical management so synechiolysis with surgical iridectomy was performed after which a normal depth anterior chamber was attained. Synechia and iris bombe were also relieved, and vision was regained.

Sporadic summer outbreak of SHAPU in even years: Does the pattern match with the usual autumn outbreak?

PURPOSE: Seasonal Hyperacute Panuveitis (SHAPU), is a mysterious blinding disease seen only in Nepal with a higher prevalence among children usually seen in autumn every alternate odd year since 1975. This report highlights the sporadic summer outbreak in the even years with atypical presentation. OBSERVATIONS: Three patients were diagnosed as SHAPU in the summer (May) of 2020. All of them noted the presence of white moths (Gazalina species) in their environment with or without direct physical contact. The clinical patterns were severe in nature including corneal melting. Two out of three patients (66.6%) developed phthisis bulbi and lost their vision. CONCLUSIONS AND IMPORTANCE: White moth has been associated as a risk factor for SHAPU. Despite the known natural history of appearance after monsoon of every odd year, the few unhatched eggs of the moths may hatch under the favorable circumstances in the summer of the even years and may lead to the sporadic outbreak of SHAPU. Though less in numbers, the clinical presentation of such sporadic SHAPU cases may be atypical with less favorable outcome.

Pathologic Risk Factor in Retinoblastoma: An Institutional Experience Based on Analysis of Enucleated Eyes.

BACKGROUND: Mortality resulting from the metastasis of retinoblastoma is uncommon in the developed world, however it still constitutes a major problem in developing countries like Nepal. The cases of retinoblastoma with increased risk of metastasis even after enucleation can be predicted from the histopathological examination of the enucleated specimen. We conducted this study aiming to assess the frequency and spectrum of high-risk histological features in enucleated specimens of retinoblastoma. MATERIALS AND METHODS: Forty-two specimens of primary enucleation done for treatment of retinoblastoma received in the Department of Pathology at UCMS from 1st January 2016 to 31st December 2018 were included in the study. All slides were reviewed for high-risk histological features along with tumor differentiation, tumor extension, necrosis, and staging. Correlation of high-risk histological features with age and tumor size was calculated using unpaired t-test and correlation with tumor differentiation, necrosis and staging was done using Pearson Chi square test. RESULTS: The median age at enucleation was 24 months. All patients had endophytic lesion with a mean tumor size of 1.8cm. One or more high-risk histological features were identified in 30.9% (13/42). The most common high-risk histological feature was retrolaminar optic nerve invasion (10/12, 71.4%). Statistically significant correlation of high risk histological features was noted with tumor size (p=0.011) and AJCC stage of tumor (p=0.0001). CONCLUSION: Complete histopathological evaluation of retinoblastoma requires searching for high-risk histological features, the presence of which will guide the clinician in timely planning for subsequent neoadjuvant therapy.