Multifocal Unilateral Orange Fundus Tumors in a Young Man.

A 38-year-old man had an asymptomatic, orange, macular choroidal mass with macular choroidal folds and a retinal pigment epithelial detachment in the right eye and a second orange mass nasal to the optic disc also in the right eye. What would you do next?

Spontaneous onset and resolution of optic disk pit maculopathy in a young patient.

We report the case of an 8-year-old boy who presented with an optic disk pit and subsequently developed optic disk pit maculopathy, consisting of cystoid retinal edema in the peripapillary space and in the papillomacular bundle, which slowly and spontaneously resolved without intervention.

Global retinoblastoma studies: A review.

In the current era of global health awareness for retinoblastoma (RB), the challenge that lies ahead of us is providing optimal care for children affected with RB in underdeveloped nations. The understanding of similarities and disparities between various nations across the world aids in achieving comparable outcomes. With dissolving geographic barriers and evolving collaboration, global collaborative studies on RB are becoming increasingly common. They provide real-world, robust evidence on several aspects of RB. This review discusses insights gained from global RB studies regarding the demographics, certain aspects of etiopathogenesis and epidemiology, international travel burden, disparities in clinical presentations based on national income levels, management protocols, pathology, treatment outcomes, and the effect of COVID-19 on RB care across the world. These insights are likely to impact individual practice as well as inform policy reforms.

Retinoblastoma in Asia: Clinical Presentation and Treatment Outcomes in 2112 Patients from 33 Countries.

PURPOSE: To describe the clinical presentation and treatment outcomes of children who received a diagnosis of retinoblastoma in 2017 throughout Asia. DESIGN: Multinational, prospective study including treatment-naïve patients in Asia who received a diagnosis of retinoblastoma in 2017 and were followed up thereafter. PARTICIPANTS: A total of 2112 patients (2797 eyes) from 96 retinoblastoma treatment centers in 33 Asian countries. INTERVENTIONS: Chemotherapy, radiotherapy, enucleation, and orbital exenteration. MAIN OUTCOME MEASURES: Enucleation and death. RESULTS: Within the cohort, 1021 patients (48%) were from South Asia (SA), 503 patients (24%) were from East Asia (EA), 310 patients (15%) were from Southeast Asia (SEA), 218 patients (10%) were from West Asia (WA), and 60 patients (3%) were from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, < 1-261 months). The cohort included 1195 male patients (57%) and 917 female patients (43%). The most common presenting symptoms were leukocoria (72%) and strabismus (13%). Using the American Joint Committee on Cancer Staging Manual, Eighth Edition, classification, tumors were staged as cT1 (n = 441 [16%]), cT2 (n = 951 [34%]), cT3 (n = 1136 [41%]), cT4 (n = 267 [10%]), N1 (n = 48 [2%]), and M1 (n = 129 [6%]) at presentation. Retinoblastoma was treated with intravenous chemotherapy in 1450 eyes (52%) and 857 eyes (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, and 20% and 11% for WA (P < 0.0001 and P < 0.0001), respectively. CONCLUSIONS: At the conclusion of this study, significant heterogeneity was found in treatment outcomes of retinoblastoma among the regions of Asia. East Asia displayed better outcomes with higher rates of globe and life salvage, whereas Southeast Asia showed poorer outcomes compared with the rest of Asia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Amelanotic Fundus Lesion in an Older White Woman.

An 81-year-old woman noted decreased vision in her left eye for 6 months. She had a history of macular degeneration in both eyes and retinal detachment in the left eye that was treated 35 years previously. What would you do next?

Anterior segment optical coherence tomography features of ocular surface squamous epithelial hyperplasia: A report of four cases.

Herein, we report the clinico-tomographic and histopathological features of four patients with biopsy-proven ocular surface squamous epithelial hyperplasia (OSSEH), a close mimicker of ocular surface squamous neoplasia (OSSN). The mean age at diagnosis was 58 years (median, 60 years; range, 35-77 years). All lesions were unilateral. Isolated corneal plaque was seen in 50% (n = 2) and nodular lesion at the nasal limbus in 50% (n = 2). Keratinization was seen in 75% (n = 3) of lesions and intrinsic vessels in 75% (n = 3). A clinical diagnosis of OSSN was made in all cases. Anterior segment optical coherence tomography (ASOCT) revealed mild epithelial hyperreflectivity in 100% (n = 4). The epithelium was normal in thickness in 75% (n = 3) and showed mild thickening in 25% (n = 1). Only 25% (n = 1) showed abrupt transition in epithelial thickness from the contiguous corneal epithelium. Histopathological examination revealed hyperplastic squamous epithelium and no cellular atypia in all cases. Stable ocular surface and no recurrences were noted at a mean follow-up of 17 months (median, 11 months; range, 2-43 months). Although OSSEH can mimic OSSN clinically, the presence of mild epithelial hyperreflectivity, lack of epithelial thickening, absence of abrupt transition from normal epithelium, and presence of subepithelial hyperreflectivity on ASOCT favor the diagnosis of OSSEH.

Bilateral Conjunctival Actinic Granuloma With Necrobiotic Vasculitic Pattern: A Diagnosis of Exclusion.

A 53-year-old Caucasian male presented with an inflamed-appearing limbal nodule in his OD, clinically compatible with nodular episcleritis, that was unresponsive to topical corticosteroid therapy. Excisional biopsy of the lesion was performed and histopathological examination revealed foci of necrotizing vasculitis and granulomatous inflammation in a background of intense actinic elastosis. Infectious stains for organisms were negative. A comprehensive systemic evaluation for vasculitides was negative. Three years later, the patient returned with a clinically and histopathologically identical lesion in his OS. Systemic evaluation was noncontributory again, and a diagnosis of bilateral conjunctival actinic granuloma with necrobiotic vasculitic pattern was made.

Truxima (rituximab-abbs) for Periocular Xanthogranuloma with Adult-Onset Asthma and Systemic IgG4-Related Disease.

A 58-year-old female with a 3-year history of adult-onset asthma, bilateral blepharoptosis, dry eye, and yellow-orange xanthelasma-like plaques extensively involving both upper eyelids presented with a diagnosis of adult-onset asthma with periocular xanthogranuloma (AAPOX) and systemic IgG4-related disease. Over the next 8 years, she received 10 intralesional triamcinolone injections (40-80 mg) in the right upper eyelid, 7 intralesional triamcinolone injections (30-60 mg) in the left upper eyelid, underwent right anterior orbitotomy twice followed by 4 doses of rituximab (1000 mg intravenous infusion) without regression of the AAPOX. The patient was then treated with 2 monthly doses of Truxima (1000 mg intravenous infusion), a biosimilar to rituximab. At the most recent follow-up, 13 months later, the xanthelasma-like plaques and orbital infiltration had markedly improved. To the best of the authors' knowledge, this is the first report of Truxima being used to treat AAPOX with systemic IgG4-related disease and to generate a sustained clinical response.

Exogenous Ochronosis With Ocular Involvement From Chronic Use of Teavigo.

Exogenous ochronosis refers to accumulation of homogentisic acid metabolites in tissues, manifesting as pigmentation of affected tissues. Phenolic compounds are most commonly implicated, including hydroquinone, quinine, phenol, resorcinol, mercury, and picric acid. The affected connective tissues exhibit brownish discoloration when heavily pigmented and the histopathological appearance is characteristic with "banana-shaped" ochre-colored pigment deposits. Herein, the authors describe a rare case of exogenous ochronosis involving the conjunctiva, sclera and skin, as a result of chronic use of Teavigo (94% epigallocatechin gallate), a polyphenol compound with postulated antioxidant and antiapoptotic activity.

Artificial intelligence and machine learning in ocular oncology: Retinoblastoma.

Purpose: This study was done to explore the utility of artificial intelligence (AI) and machine learning in the diagnosis and grouping of intraocular retinoblastoma (iRB). Methods: It was a retrospective observational study using AI and Machine learning, Computer Vision (OpenCV). Results: Of 771 fundus images of 109 eyes, 181 images had no tumor and 590 images displayed iRB based on review by two independent ocular oncologists (with an interobserver variability of <1%). The sensitivity, specificity, positive predictive value, and negative predictive value of the trained AI model were 85%, 99%, 99.6%, and 67%, respectively. Of 109 eyes, the sensitivity, specificity, positive predictive value, and negative predictive value for detection of RB by AI model were 96%, 94%, 97%, and 91%, respectively. Of these, the eyes were normal (n = 31) or belonged to groupA (n=1), B (n=22), C (n=8), D (n=23),and E (n=24) RB based on review by two independent ocular oncologists (with an interobserver variability of 0%). The sensitivity, specificity, positive predictive value, and negative predictive value of the trained AI model were 100%, 100%, 100%, and 100% for group A; 82%, 20 21 98%, 90%, and 96% for group B; 63%, 99%, 83%, and 97% for group C; 78%, 98%, 90%, and 94% for group D, and 92%, 91%, 73%, and 98% for group E, respectively. Conclusion: Based on our study, we conclude that the AI model for iRB is highly sensitive in the detection of RB with high specificity for the classification of iRB.