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Purpose: To report a case with peripheral eosinophilia and elevated immunoglobulin (Ig) E levels, subsequently diagnosed as IgG4-related ophthalmic disease involving the extraocular muscles.Case summary: A 56-year-old male visited the allergy department presenting with systemic urticaria and bilateral eyelid swelling that began 5 months prior. Laboratory examinations showed elevated levels of serum eosinophil and IgE, 1,309 IU/uL and 1,793 IU/mL, respectively. Orbital computed tomography revealed that all extraocular muscles and the bilateral exophthalmos were enlarged, and the patient was referred to the ophthalmology department. Eye alignment was orthophoric for all gaze directions, and limited abduction (-1) was noted in both eyes. An incisional biopsy of the extraocular muscles was conducted. Histopathological findings showed lymphoid aggregates, diffuse fibrosis, and an increased IgG4+/IgG+ plasma cell ratio of 40%, which led to the diagnosis of IgG4-related ophthalmic disease. An elevated IgG4 serum level (1,710 mg/dL) was also noted. The patient received high-dose intravenous steroids and eyelid swelling improved after two months. Levels of serum eosinophil, IgE, and IgG4 all decreased after three months.Conclusions: IgG4-related ophthalmic disease may be accompanied by eosinophilia and elevated IgE. These findings may facilitate future diagnoses of this disease.

Ophthalmic disease in people living with HIV (PLWH) and at-risk controls in Sub-Saharan Africa was evaluated. PLWH were more likely to have ophthalmic disease at enrollment, but there was no difference in incidence once enrolled.

Background Behcet's disease (BD) is a relapsing-remitting vasculitis, which can manifest in different organ systems including the eyes. There is currently limited published data describing the incidence of ophthalmic disease within the United Kingdom. The primary aim of this study was to survey the incidence and manifestations of ophthalmic BD prospectively, with a secondary aim of reviewing treatment modalities initiated in first-line therapy. Methods Using the British Ophthalmic Surveillance Unit reporting system between October 2016 and November 2018, we prospectively surveyed the number of cases of BD presenting to UK ophthalmologists. A total of 89 cases of ophthalmic manifestations of BD were reported and complete information was collected on 58 patients. Results 93 eyes of 58 patients were affected. The median age of reported cases was 31 years (range 13-55 years) who were born in 15 different countries. Most cases (n = 35, 60%) had bilateral involvement. Vitritis was the most common ocular manifestation (68%; n = 63) followed by anterior uveitis (46%; n = 43). The greatest causes of visual morbidity were cystoid macular oedema, vitritis and retinal ischaemia. Most patients were prescribed either topical or oral corticosteroids (59%; n = 34), with some given intravitreal or intravenous corticosteroids. Five patients (8.6%) were initiated on disease-modifying anti-rheumatic drugs and one given an anti-TNF monoclonal antibody. Conclusions This is the first prospective study to analyse the incidence of ophthalmic involvement in BD over a 2-year period, finding an annual incidence of 0.04 per 100,000 individuals in the UK.

Teleophthalmology, a subfield of telemedicine, has recently been widely applied in ophthalmic disease management, accelerated by ubiquitous connectivity via mobile computing and communication applications. Teleophthalmology has strengths in overcoming geographic barriers and broadening access to medical resources, as a supplement to face-to-face clinical settings. Eyes, especially the anterior segment, are one of the most researched superficial parts of the human body. Therefore, ophthalmic images, easily captured by portable devices, have been widely applied in teleophthalmology, boosted by advancements in software and hardware in recent years. This review aims to revise current teleophthalmology applications in the anterior segment and other diseases from a temporal and spatial perspective, and summarize common scenarios in teleophthalmology, including screening, diagnosis, treatment, monitoring, postoperative follow-up, and tele-education of patients and clinical practitioners. Further, challenges in the current application of teleophthalmology and the future development of teleophthalmology are discussed.

Annemarie Klingenstein,1 Aylin Garip-Kuebler,1 Siegfried Priglinger,1 Christoph Hintschich,1 Ullrich G Mueller-Lisse2 1Department of Ophthalmology, Ludwig-Maximilians-University, Munich, Germany; 2Department of Radiology, Ludwig-Maximilians-University, Munich, GermanyCorrespondence: Annemarie KlingensteinLudwig-Maximilians-University, Department of Ophthalmology, Klinikum der Universität München, Campus Innenstadt, Mathildenstraße 8, Munich, D-80336, GermanyTel +49 89 44005 3811Fax +49 89 44005 5160Email Annemarie.Klingenstein@med.uni-muenchen.deAim: To detect radiological features that, in addition to clinical findings, may aid in correct differentiation between IgG4-related ophthalmic disease (IgG4-ROD) and ocular adnexal lymphoma (OAL).Methods: In this retrospective, single-center, comparative analysis, we compared cross-sectional imaging findings of 13 consecutive patients with histologically proven IgG4-ROD and a control group of 29 consecutive OAL-patients diagnosed between 10/2014 and 09/2019. Statistical significance was accepted at a p< 0.05 significance level.Results: IgG4-ROD-patients had longer time-to-diagnosis, higher orbital recurrence rates, but smaller lesions compared to OAL-patients (p=0.002; p=0.006 and p=0.006; Mann–Whitney U-test). Frequent cross-sectional imaging findings in both IgG4-ROD-patients and OAL-patients included extraocular muscle enlargement (92% and 93%, respectively; most often in the lateral rectus muscles and the levator-complex), and lacrimal-gland enlargement (85% and 83%, respectively). Other imaging findings comprised infraorbital nerve-involvement (IgG4-ROD, 23%, OAL, 17%) and orbital fat inflammation (IgG4-ROD, 23%, OAL, 28%). Bony infiltration and remodeling, heterogenous contrast-media distribution, and infiltration of the lacrimal system were seen slightly more often in IgG4-ROD (23%, 38%, 15% and 15% versus 17%, 14%, 3% and 7%). However, cross-sectional imaging features did not differ significantly between patient subgroups. Clinical symptoms predominantly occurred unilaterally (IgG4-ROD, 9/13, 69%, OAL, 24/29, 83%), while imaging findings were most often bilateral (IgG4-ROD, 11/13, 85%, OAL, 23/29, 79%, p< 0.001, McNemar test).Conclusion: No morphological cross-sectional imaging sign could reliably distinguish between IgG4-ROD and OAL, leaving histopathology indispensable for definite diagnosis. Yet, importantly, for both IgG4-ROD and OAL, cross-sectional imaging frequently detected bilateral orbital disease when only one eye was clinically affected.Keywords: IgG4, idiopathic orbital inflammation, CT, ocular adnexal lymphoma

The diagnostic criteria for IgG4-related disease were previously published and serum IgG4 measurement has been reimbursed by national health insurance in Japan since 2012. Eight patients diagnosed with IgG4-related disease based on lacrimal gland masses were retrospectively reviewed. The 8 patients were 3 men and 5 women ranging in age from 52 to 77 (median, 63) years at the initial visit and their follow-up period ranged from 0.25 to 11 (median, 7) years. Bilateral and unilateral involvement were noted in 4 patients each; 2 on the right side and 2 on the left side in those with unilateral involvement. Serum IgG4 was high in 5 of 8 patients at the initial visit. Five patients with no systemic signs were followed without treatment, whereas oral steroids were administered and tapered in the other 3 patients who exhibited systemic signs. One patient with a history of radiation for MALT lymphoma in bilateral lacrimal glands developed IgG4-related disease in the left lacrimal gland 10 years later and was followed without treatment. Nine years later, her serum IgG4 level increased to 1500 mg/dL and paracardiac lesions, found on positron emission tomography, were confirmed to be MALT lymphoma by needle biopsy, leading to systemic chemotherapy. The other 7 patients had neither local recurrence nor additional systemic signs. Serum IgG4 monitoring may be useful to detect systemic complications in IgG4-related ophthalmic disease and markedly high serum IgG4 levels may indicate new lymphoma at other sites.

Radiologic orbital imaging provides important information in the diagnosis and management of orbital inflammation. However, the diagnostic value of orbital imaging is not well elucidated. This study aimed to investigate the diagnostic accuracy of orbital imaging to diagnose orbital inflammatory diseases and its ability to detect active inflammation. We collected 75 scans of 52 patients (49 computed tomography (CT) scans; 26 magnetic resonance (MR) imaging scans). Clinical diagnoses included thyroid eye disease (TED) (41 scans, 31 patients), non-specific orbital inflammation (NSOI) (22 scans, 14 patients), sarcoidosis (4 scans, 3 patients), IgG4-related ophthalmic disease (IgG4-ROD) (5 scans, 3 patients), and granulomatosis with polyangiitis (GPA) (3 scans, 1 patient). Two experienced neuroradiologists interpreted the scans, offered a most likely diagnosis, and assessed the activity of inflammation, blinded to clinical findings. The accuracy rate of radiological diagnosis compared to each clinical diagnosis was evaluated. Sensitivity and specificity in detecting active inflammation were analyzed for TED and NSOI. The accuracy rate of radiologic diagnosis was 80.0% for IgG4-ROD, 77.3% for NSOI, and 73.2% for TED. Orbital imaging could not diagnose sarcoidosis. Orbital CT had a sensitivity of 50.0% and a specificity of 75.0% to predict active TED using clinical assessment as the gold standard. The sensitivity/specificity of orbital MR was 83.3/16.7% for the detection of active NSOI. In conclusion, orbital imaging is accurate for the diagnosis of IgG4, NSOI, and TED. Further studies with a large number of cases are needed to confirm this finding, especially with regard to uncommon diseases. Orbital CT showed moderate sensitivity and good specificity for identifying active TED.

Background Administrative health claims data have been used for research in neuro-ophthalmology, but the validity of International Classification of Diseases (ICD) codes for identifying neuro-ophthalmic conditions is unclear. Evidence acquisition We performed a systematic literature review to assess the validity of administrative claims data for identifying patients with neuro-ophthalmic disorders. Two reviewers independently reviewed all eligible full-length articles and used a standardized abstraction form to identify ICD code-based definitions for 9 neuro-ophthalmic conditions and their sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). A quality assessment of eligible studies was also performed. Results Eleven articles that met criteria for inclusion are as follows: 3 studies of idiopathic intracranial hypertension (PPV 54%-91% and NPV 74%-85%), 2 studies of giant cell arteritis (sensitivity 30%-96% and PPV 94%), 3 studies of optic neuritis (sensitivity 76%-99%, specificity 83%-100%, PPV 25%-100%, and NPV 98%-100%), 1 study of neuromyelitis optica (sensitivity 60%, specificity 100%, PPV 43%-100%, and NPV 98%-100%), 1 study of ocular motor cranial neuropathies (PPV 98%-99%), and 2 studies of myasthenia gravis (sensitivity 53%-97%, specificity 99%-100%, PPV 5%-90%, and NPV 100%). No studies met eligibility criteria for nonarteritic ischemic optic neuropathy, thyroid eye disease, and blepharospasm. Approximately 45.5% provided only one measure of diagnostic accuracy. Complete information about the validation cohorts, inclusion/exclusion criteria, data collection methods, and expertise of those reviewing charts for diagnostic accuracy was missing in 90.9%, 72.7%, 81.8%, and 36.4% of studies, respectively. Conclusions Few studies have reported the validity of ICD codes for neuro-ophthalmic conditions. The range of diagnostic accuracy for some disorders and study quality varied widely. This should be taken into consideration when interpreting studies of neuro-ophthalmic conditions using administrative claims data.

Purpose: To evaluate the incidence of ocular diseases in Dakhla Oasis Egypt Setting: Examination in 1 central hospital and 17 primary health care units and 10 small villages and cities in the western desert in Egypt.Methods: There were 15 ophthalmic volunteers were divided into 3 screening groups and one operation team. The 3 groups screen the village and give free medical treatments for the patients, the major operations referred to the central hospitals to be done by the operation team. Screenings include full eye exam, IOP and VA measurement, fundus examination and minor operations. Major operations, laser treatments and some investigations were done by the surgical team. Results: The campaign examined 3780 patients and treatment was given, 81 Cataract operations, 14 Other Major operations and 29 minor operations were done. The diseases were 6.59% lid, 1.11% lacrimal system, 49.60% conjunctive, 6.30% corneal, 0.40% uvea, 5.58% glaucoma, 12.59% cataract, 4.92% fundus diseases and 1.11%EOM problems. Conclusions: Prevention of blindness program is important for curing different ocular diseases and preventing their progression.

Corticosteroids are the first-line treatment for immunoglobulin G4-related ophthalmic disease (IgG4-ROD). However, the therapeutic effect of corticosteroids is unpredictable, and recurrences occur frequently. We present a case of a 60-year-old woman with IgG4-ROD of the lacrimal fossa. She was first treated with oral prednisolone, which was tapered within three months. However, proptosis recurred two months after prednisolone withdrawal. En bloc excision of the lesion was performed via the eyelid crease approach. The patient is lesion-free without corticosteroids at 18 months after surgery. Complete resection can be a powerful strategy for IgG4-ROD if a well-defined lesion is located in accessible areas.

Optical-resolution photoacoustic microscopy (OR-PAM) is used for in vivo imaging of a variety of albino and pigmented eyes taking advantages of requiring no exogenous dye, performing high-resolution imaging, and achieving morphologic and functional imaging at the same time. However, to accurately diagnose the ophthalmic disease in the OR-PAM images, vascular enhancement algorithms are necessary for extracting vessels and quantifying them correctly. Vascular enhancement algorithms developed for other imaging technologies, are not suitable to be used for OR-PAM, because of the underlying differences in the physics of the formation of images. In this study, a new vascular enhancement algorithm called photoacoustic imaging vasculature enhancement filter (PAIVEF) is proposed, which not only enhances vasculature including micro-vessels signals, suppresses noise signals effectively, but also achieves highly sensitive and accurate enhancement of the vasculature within a large depth range in and out of the system's depth of focus (DOF). Using the PAIVEF, the morphologic and functional 3D images of the whole rat's ocular anterior vasculature segment was displayed simultaneously for a depth range of ~0.6 mm, which was ~7 times of the system's DOF. This study paves the way for the application of OR-PAM technology in ophthalmic disease research.

In vivo functional imaging of human photoreceptors is an emerging field, with compelling potential applications in basic science, translational research, and clinical management of ophthalmic disease. Measurements of light-evoked changes in the photoreceptors has been successfully demonstrated using adaptive optics (AO) coherent flood illumination (CFI), AO scanning light ophthalmoscopy (SLO), AO optical coherence tomography (OCT), and full-field OCT with digital AO (dAO). While the optical principles and data processing of these systems differ greatly, and while these differences manifest in the resulting measurements, we believe that the underlying physiological processes involved in each of those techniques are likely the same. AO-CFI and AO-SLO systems are more widely used than OCT systems. However, those systems produce only two-dimensional images and so, less can be said about the anatomical and physiological origins of the observed signal. OCT signal, on the other hand, provides 3D imaging but at a cost of high volume of data, making it impractical to clinical purposes. In light of this, we employed a combined AO-OCT-SLO system–with point-for-point correspondence between the OCT and SLO images–to measure functional responses simultaneously with both and investigate SLO retinal functional biomarkers based on OCT response. The resulting SLO images reveal reflectance changes in the cones which are consistent with those previously reported using AO-CFI and AO-SLO. The resulting OCT volumes show phase changes in the cone outer segment (OS) consistent with those previously reported by us and others. We recapitulate a model of the cone OS previously proposed to explain AO-CFI reflectance changes, and show how this model can be used to predict the signal in AO-SLO. The limitations of the model is also discussed in this manuscript.

AimsTo determine the long-term outcomes of ocular adnexal lesions in immunoglobulin G4-related ophthalmic disease (IgG4-ROD).MethodsThis retrospective, non-randomised exploratory study included 82 patients with ocular adnexal lesions. We evaluated the long-term outcomes in 71 patients during the median follow-up period of 30 months, who underwent either watchful waiting (n=20; range 12–90 months) or systemic corticosteroid treatment, delivered according to consensus guidelines (n=51; range 9– 115 months). We also analysed factors that might contribute to recalcitrance to treatment.ResultsOf 82 patients, 40 (49%) were male, and the median patient age was 60 years old. Twenty-one (26%) patients with extraocular muscle (EOM) and/or trigeminal nerve branch (CN V) enlargements had a significantly high frequency of multiple ocular adnexal lesions (pConclusionsThis exploratory study showed that different types of ocular adnexal lesions in IgG4-ROD displayed distinct proliferative activities. Our results suggested that EOM and/or CN V enlargements might be secondary lesions that confer refractoriness to systemic corticosteroid treatment recommended by consensus guidelines.

Background The following study investigates the involvement of optic neuropathy in IgG4-related ophthalmic diseases (IgG4-ROD) based on the magnetic resonance imaging (MRI) data, and different imaging features of IgG4-ROD related optic neuropathy related to other orbital diseases. Methods This retrospective study included 225 patients with IgG4-RD admitted at two ophthalmology centers between January 2014 and December 2017. Twenty-six patients had both pre-therapeutic orbital MRI and optic never injury. The causes of optic neuropathy were analyzed, and the special sign in MRI to diagnose IgG4-ROD was also evaluated. Results Twelve cases had inflammation of the optic nerve sheath, while 14 cases had compression due to extraocular muscles and pseudo tumor masses. Two cases had hypertrophic cranial pachymeningitis, while one case had hypophysis involving optic chiasma. Conclusion The most common causes of optic nerve injury in IgG-4 ROD are inflammation of optic nerve sheath, compression of extraocular muscles, pseudo tumor mass and hypertrophic cranial pachymeningitis, and hypophysis involving optic chiasma.

Diabetic Retinopathy (DR) is an ophthalmic disease that damages retinal blood vessels. DR causes impaired vision and may even lead to blindness if it is not diagnosed in early stages. DR has five stages or classes, namely normal, mild, moderate, severe and PDR (Proliferative Diabetic Retinopathy). Normally, highly trained experts examine the colored fundus images to diagnose this fatal disease. This manual diagnosis of this condition (by clinicians) is tedious and error-prone. Therefore, various computer vision-based techniques have been proposed to automatically detect DR and its different stages from retina images. However, these methods are unable to encode the underlying complicated features and can only classify DR's different stages with very low accuracy particularly, for the early stages. In this research, we used the publicly available Kaggle dataset of retina images to train an ensemble of five deep Convolution Neural Network (CNN) models (Resnet50, Inceptionv3, Xception, Dense121, Dense169) to encode the rich features and improve the classification for different stages of DR. The experimental results show that the proposed model detects all the stages of DR unlike the current methods and performs better compared to state-of-the-art methods on the same Kaggle dataset.

BackgroundTo evaluate the impact of age-related macular degeneration (AMD) on the quality of life (QoL) in a Brazilian population using The National Eye Institute-Visual Function Questionnaire-25 (NEI-VFQ-25).MethodsThis observational study included 462 participants from the Departments of Ophthalmology of the University of Campinas and Conderg-Divinolândia. The NEI-VFQ-25 questionnaire and Rasch analysis were used to assess the vision-related quality of life (VRQoL). Patients with macular neovascularization were interviewed at enrollment and after three loading doses of intravitreal anti-vascular endothelial growth factor (anti-VEGF) treatment.ResultsOne hundred thirty-three patients were excluded because they had another ophthalmic disease, for a total of 349 patients included in the study (177 in the AMD group, 172 in the control group; 56.4% were women; mean ± standard deviation age, 70.6 ± 9.5 years). Most NEI-VFQ-25 subscale scores were significantly lower in the AMD group compared with the control group. The Rasch-calibrated NEI-VFQ-25 median score in the visual-functioning component was 56.41 for the AMD group and 61.53 for the control group, a difference of ± 4.00 (P = 0.0001). Separate analyses of the sociodemographic and ocular characteristics showed that the NEI-VFQ-25 scores were affected mostly by family income, educational level, descent, diet (vegetables/fruits), physical activity, and visual acuity (VA). The longitudinal component assessed a different group of 48 patients with exudative disease treated with anti-VEGF drugs. The mean logarithm of the minimum angle of resolution change in VA in treated eyes was a 0.16 decrease (P = 0.01). The mean change in the optical coherence tomography macular thickness was a 36.74-μm decrease (P = 0.012) from baseline to 4 months. The mean NEI-VFQ-25 scores improved significantly from baseline to follow-up at 4 months in almost all subscales.ConclusionsIn a Brazilian community, patients with AMD had a worse VRQoL than controls. The AMD severity and bilaterality were associated with decreased NEI-VFQ-25 scores. Higher family income, educational level, descent, and lifestyle significantly improved several subscales of the NEI-VFQ-25 questionnaire. Treated patients with exudative AMD had improvements in the VA, macular thickness, and most NEI-VFQ-25 subscale scores.

BACKGROUNDImmunoglobulin G4–related ophthalmic disease (IgG4-ROD) accounts for 22% of Japanese lymphoproliferative orbital disease and occurs in 4%–34% of patients with IgG4-related disease, according to the largest case series published to date. Because the optic nerve mass often appears as a tumor-like lesion, it is important, although difficult, to differentiate IgG4-ROD from other orbital tumors and diseases, and biopsy is essential for diagnosis. Here, the authors describe the surgical management of an IgG4-ROD case.OBSERVATIONSA 63-year-old man presented to the authors’ hospital with proptosis and visual impairment. Ophthalmic examination revealed intraocular hypertension. IgG4-related disease with an ophthalmic lesion was suspected on the basis of a blood test and imaging studies. Transcranial biopsy with extraorbital decompression was performed. The patient’s symptoms, including visual impairment, improved 3 days after operation, and his IgG4-related disease resolved after corticosteroid treatment.LESSONSThe standard treatment for IgG4-related disease is systemic corticosteroid therapy. However, this treatment should not be administered to patients with IgG4-ROD who a high risk of blindness. In this case, the authors completed a diagnostic and symptom-relieving transcranial biopsy without affecting the patient’s aesthetic characteristics. This is the first study, to our knowledge, to report extraorbital decompression via a transcranial approach as a surgical option for IgG4-ROD.

Background To evaluate the change of accommodation and ocular discomfort according to the display size, using quantitative measurements of accommodation and ocular discomfort through subjective and objective metrics. Methods Forty six subjects without any ophthalmic disease history were asked to watch the documentary movie, using two different sizes of smart devices; smartphones and tablets. Before and after using devices, the near point accommodation (NPA) and the near point convergence (NPC) were measured, and objective accommodation was measured using an auto refractometer/keratometer. The subjective ocular discomfort was assessed through a survey. Results Both devices showed a decrease in post-use NPA and NPC, and the change after use of the smartphone was significantly severe, 1.8 and 2.5 folds respectively, compared to tablet (p = 0.044, p = 0.033, respectively). Neither smartphone nor tablet showed significant changes in the accommodative response induced by dynamic accommodative stimulus of auto refractometer/keratometer (p = 0.240 and p = 0.199, respectively). Subjects showed a more severe increase in ocular discomfort after using smartphones (p = 0.035) and reported feeling tired even with shorter use times (p = 0.012). Conclusions Both devices showed significant decreases in NPA and NPC, and the larger changes were seen when using the small display smartphone. Even within 20minutes of using, subjects start to feel ocular discomfort, and it was more severe and faster after smartphones than tablets. Therefore, the smaller the display size, the greater the adverse impact on eyes, and thus, appropriate display size will need to be selected depending on the time and purpose of use.

Summary Genome-wide association studies (GWASs) require accurate cohort phenotyping, but expert labeling can be costly, time intensive, and variable. Here, we develop a machine learning (ML) model to predict glaucomatous optic nerve head features from color fundus photographs. We used the model to predict vertical cup-to-disc ratio (VCDR), a diagnostic parameter and cardinal endophenotype for glaucoma, in 65,680 Europeans in the UK Biobank (UKB). A GWAS of ML-based VCDR identified 299 independent genome-wide significant (GWS; p ≤ 5 × 10−8) hits in 156 loci. The ML-based GWAS replicated 62 of 65 GWS loci from a recent VCDR GWAS in the UKB for which two ophthalmologists manually labeled images for 67,040 Europeans. The ML-based GWAS also identified 93 novel loci, significantly expanding our understanding of the genetic etiologies of glaucoma and VCDR. Pathway analyses support the biological significance of the novel hits to VCDR: select loci near genes involved in neuronal and synaptic biology or harboring variants are known to cause severe Mendelian ophthalmic disease. Finally, the ML-based GWAS results significantly improve polygenic prediction of VCDR and primary open-angle glaucoma in the independent EPIC-Norfolk cohort.

Background. IgG4-related ophthalmic disease is a rare, newly recognized entity with high failure rates on first-line therapy of systemic corticosteroids and no other proven management options. Case Presentation. Here, we present the clinical course of a patient with IgG4 ophthalmic disease who achieved a favorable response from radiotherapy. Our patient initially presented with a history of recurrent painful flares of orbital inflammation, a pathologic diagnosis follicular lymphoid hyperplasia from a right lacrimal gland biopsy, and MRI imaging noting expansion of the lateral rectus muscle of the right eye. Initial treatment with dacryoadenectomy and multiple courses of corticosteroids failed to keep his symptoms at bay. Further evaluation revealed florid IgG4 staining. In this context, he was evaluated for image-guided intensity-modulated radiotherapy (IG-IMRT) to the orbit to 20 Gy in 10 fractions. His ophthalmic symptoms resolved. Conclusions. This treatment experience suggests radiotherapy may be a favorable option for symptom relief in patients with IgG4-related ophthalmic disease not controlled by corticosteroids.

Recent advances have driven the development of stem cell-derived, self-organizing, three-dimensional miniature organs, termed organoids, which mimic different eye tissues including the retina, cornea, and lens. Organoids and engineered microfluidic organ-on-chips (organ chips) are transformative technologies that show promise in simulating the architectural and functional complexity of native organs. Accordingly, they enable exploration of facets of human disease and development not accurately recapitulated by animal models. Together, these technologies will increase our understanding of the basic physiology of different eye structures, enable us to interrogate unknown aspects of ophthalmic disease pathogenesis, and serve as clinically-relevant surrogates for the evaluation of ocular therapeutics. Both the burden and prevalence of monogenic and multifactorial ophthalmic diseases, which can cause visual impairment or blindness, in the human population warrants a paradigm shift towards organoids and organ chips that can provide sensitive, quantitative, and scalable phenotypic assays. In this article, we review the current situation of organoids and organ chips in ophthalmology and discuss how they can be leveraged for translational applications.