Elizabeth Y Wei, Marko M Popovic, Panos G Christakis
{"title":"Systemic Lymphoma Masquerading as Birdshot Chorioretinopathy.","authors":"Elizabeth Y Wei, Marko M Popovic, Panos G Christakis","doi":"10.1097/ICB.0000000000001743","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001743","url":null,"abstract":"<p><strong>Purpose: </strong>To report a rare case of systemic lymphoma causing a birdshot-like chorioretinopathy (BSCR) in a histocompatibility leukocyte antigen (HLA)-A29 negative patient.</p><p><strong>Methods: </strong>A 58-year-old man presented with a one year history of blurry vision and intermittent floaters. He had evidence of bilateral choroidal birdshot-like lesions which appeared atrophic without any vitritis. He had a history of low-grade B-cell lymphoma and chronic myeloid leukemia, both of which were diagnosed >10 years prior to presentation and treated with chemotherapy.</p><p><strong>Results: </strong>The differential diagnosis of these birdshot-like lesions included autoimmune, neoplastic, and infectious etiologies. Work-up revealed a negative HLA-A29, and negative results for syphilis and tuberculosis. Magnetic resonance imaging (MRI) revealed enhancement of the trigeminal and oculomotor nerve cisternal segments but no overt evidence of malignancy. Full field electroretinogram (ffERG) and multifocal electroretinogram (mfERG) tests were within normal limits. Serial follow-up clinical examinations, retinal imaging, ffERG and mfERG demonstrated no disease progression. Two years after his initial consultation, he developed a paraspinal mass which was biopsied and found to be diffuse large B-cell lymphoma (DLBCL) and was treated with chemotherapy.</p><p><strong>Conclusion: </strong>Clinicians should consider BSCR masquerade syndromes in patients that present with birdshot-like choroidal lesions with negative HLA-A29, history of lymphoma, or atypical characteristics. In this case, we suspect the patient's past low-grade B-cell lymphoma and chemotherapy resulted in the chronic choroidal findings. It is unclear whether choroidal involvement predisposes a patient to high-grade transformation to DLBCL. His ocular findings remained inactive throughout the treatment of his DLBCL.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143528005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bilateral giant retinal tear detachments in an infant with Donnai-Barrow syndrome: A case report and review of literature.","authors":"Alan Y Huang, Sonny Caplash, Drew Scoles","doi":"10.1097/ICB.0000000000001744","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001744","url":null,"abstract":"<p><strong>Purpose: </strong>To report a rare case of bilateral giant retinal tear (GRT)-related retinal detachments in a 24-month-old male with Donnai-Barrow syndrome (DBS) and to review the current literature on this disorder.</p><p><strong>Methods: </strong>Clinical information was obtained from the medical records of a patient with Donnai-Barrow syndrome seen at the Children's Hospital of Philadelphia. A literature review was conducted to identify all published cases of genetically or clinically diagnosed Donnai-Barrow syndrome.</p><p><strong>Results: </strong>We report a case of a 24-month-old male with a history of DBS presenting with sudden loss of vision in both eyes. Ophthalmic examination revealed bilateral retinal detachments with GRT, complicated by grade C proliferative vitreoretinopathy (PVR) in the right eye. The patient underwent sequential scleral buckling, pars plana vitrectomy, membrane peeling, perfluoron, endolaser, fluid air exchange, and silicone oil placement in both eyes. Both eyes developed re-detachment, requiring repeat pars plana vitrectomy. Literature review revealed 10 reported cases of retinal detachments out of 38 DBS patients who survived past 1 year (26.3%).</p><p><strong>Conclusion: </strong>This case highlights the early presentation and complexity of GRT-related retinal detachment in DBS patients. Early, aggressive management using prophylactic laser retinopexy or scleral buckling may be essential in improving visual outcomes in DBS patients.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143527979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Julia L Xia, Jennifer M Lai, Alan G Palestine, Amit K Reddy
{"title":"Epstein-Barr Virus Associated Frosted Branch Angiitis.","authors":"Julia L Xia, Jennifer M Lai, Alan G Palestine, Amit K Reddy","doi":"10.1097/ICB.0000000000001742","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001742","url":null,"abstract":"<p><strong>Purpose: </strong>To describe two cases of frosted branch angiitis (FBA) in the setting of Epstein-Barr virus (EBV) infection.</p><p><strong>Methods: </strong>Retrospective case series of two patients at the University of Colorado Sue Anschutz-Rodgers Eye Center.</p><p><strong>Results: </strong>Case 1 is a 17-year-old female who presented with bilateral floaters and blurry vision two weeks after a diagnosis of mononucleosis. Visual acuity was 20/20 in both eyes and exam was significant for vitreous cell and FBA appearance to the retinal vasculature in both eyes. Treatment with oral prednisone and valacyclovir was initiated with improvement in vitritis and vascular sheathing. One month after all treatment was discontinued, there was a recurrence of vitritis that was treated with bilateral sub-Tenon's triamcinolone acetonide injections. Final visual acuity was 20/20 in both eyes with resolved vitritis. Case 2 is a 20-year-old male who presented with headache and acute bilateral vision loss. He was found to have a meningoencephalitis with cerebrospinal fluid positive for EBV that was treated with intravenous methylprednisolone and acyclovir. Visual acuity was count fingers in both eyes. Exam was significant for diffuse bilateral frosted branch angiitis which the subsequent development of bilateral bacillary detachments and neuroretinitis that required treatment with oral prednisone, oral acyclovir, and bilateral intravitreal dexamethasone implants, which resolved all ocular inflammation. Final visual acuity was 20/30 right eye and 20/25 left eye.</p><p><strong>Conclusion: </strong>Bilateral FBA may present following EBV infection and responds well to corticosteroids and antiviral therapy.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sergio Mendoza, Lindsay Klofas Kozek, Da Meng, Sandra Hoyek, Efren Gonzalez, Nimesh A Patel
{"title":"Exudative retinal detachment in a pediatric patient with Rubinstein-Taybi syndrome.","authors":"Sergio Mendoza, Lindsay Klofas Kozek, Da Meng, Sandra Hoyek, Efren Gonzalez, Nimesh A Patel","doi":"10.1097/ICB.0000000000001740","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001740","url":null,"abstract":"<p><strong>Purpose: </strong>To highlight findings of retinal exudation with exudative retinal detachment in a patient with Rubinstein-Taybi syndrome (RSTS) due to CREBBP mutation, successfully treated with laser, with the goal of adding to the known phenotypic spectrum of ophthalmic manifestations of RSTS.</p><p><strong>Methods: </strong>A case report of a pediatric patient evaluated with multimodal imaging including fundus photography and fluorescein angiography.</p><p><strong>Results: </strong>A 15-year-old female with RSTS caused by a frameshift mutation in CREBBP was referred for retinal examination under anesthesia. In additional to typical features of RSTS, including developmental delay, microcephaly, and broad thumbs and toes, she had an ocular history of bilateral nasolacrimal duct obstructions and intermittent exotropia. Dilated fundus examination revealed left iris coloboma, bilateral optic nerve colobomas, and exudation of the left temporal retina with associated exudative retinal detachment and inferotemporal hemorrhage. Fluorescein angiography demonstrated leakage and avascularity of both eyes, with diffuse leakage and nonperfusion noted in the left eye. The avascular areas of the right eye were treated prophylactically with laser as well as sub-Tenon's triamcinolone. The left retinal exudation initially improved with several rounds of laser treatment, however vitreoretinal membranes eventually developed, resulting in a focal tractional detachment requiring vitrectomy and lensectomy.</p><p><strong>Conclusion: </strong>Patients with RSTS have a wide spectrum of ophthalmic manifestations. Our patient demonstrated marked peripheral retinal avascularity and vascular leakage, ultimately resulting in a novel finding of sub-retinal exudation. Laser to the avascular retina led to initial regression of the exudation, however the patient ultimately developed a focal tractional detachment requiring surgery.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amit Bidasaria, Sampurna Bhattacharya, Aarzoo Juneja, Pragati Paul, Ahana Sen, Bristi Majumdar, Ruchira Chowdhury, Kumar Saurabh, Sourav Sinha, Rupak Roy
{"title":"Multimodal imaging of a case of intraretinal silicone oil in an eye with retinochoroidal coloboma: Insights into possible routes of migration.","authors":"Amit Bidasaria, Sampurna Bhattacharya, Aarzoo Juneja, Pragati Paul, Ahana Sen, Bristi Majumdar, Ruchira Chowdhury, Kumar Saurabh, Sourav Sinha, Rupak Roy","doi":"10.1097/ICB.0000000000001741","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001741","url":null,"abstract":"<p><strong>Purpose: </strong>We report multimodal imaging of intraretinal silicone oil in a case operated for coloboma-associated retinal detachment (RD) and provide imaging based anatomical insights of the route on entry of intraretinal emulsified silicone oil droplets.</p><p><strong>Method: </strong>A 19-year-old male presented with gradual onset diminution of vision in right eye for several years with worsening for 1 month. The patient was evaluated with examination, multimodal imaging, including fundus photography, optical coherence tomography, multicolor imaging, and green reflectance imaging.</p><p><strong>Result: </strong>The right eye showed large inferonasal chorioretinal coloboma sparing the fovea, with dull foveal reflex on color fundus photography, loss of foveal contour with multiple hyperreflective dots predominantly collected in the outer nuclear layer in the foveal region on optical coherence tomography, greenish hue in the foveal region with feather-like projections on multicolor imaging, and crystalline deposits on green reflectance imaging. This was diagnosed as collection of intraretinal silicone oil post-RD surgery in a case of chorioretinal coloboma.</p><p><strong>Conclusion: </strong>We highlight a possible pathway for the intraretinal entry of emulsified silicone oil particles in a case of chorioretinal coloboma.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143517299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Livia Faes, Jesse J Jung, John Sorenson, K Bailey Freund
{"title":"Atypical Autofluorescence Findings in Geographic Atrophy: The Influence of Age-Related Choroidal Atrophy.","authors":"Livia Faes, Jesse J Jung, John Sorenson, K Bailey Freund","doi":"10.1097/ICB.0000000000001739","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001739","url":null,"abstract":"<p><strong>Purpose: </strong>To describe atypical fundus autofluorescence (FAF) patterns in geographic atrophy (GA) secondary to age-related macular degeneration (AMD) with associated age-related choroidal atrophy (ARCA).</p><p><strong>Methods: </strong>Multimodal imaging of two cases using (pseudo-)color fundus photography, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, and FAF employed with blue- and green excitation wavelengths on several devices (Spectralis, Heidelberg and (ultra-)widefield [UWF] FAF [California, Optos and EIDON, iCare]).</p><p><strong>Results: </strong>Two female patients, with foveal-involving GA secondary to AMD, were assessed. All eyes demonstrated concurrent features indicative of ARCA on multimodal imaging including a paucity of choroidal vasculature, reduced choroidal pigmentation, macular pigmentary changes, peripapillary atrophy, and subretinal drusenoid deposits. Clinically, progression of GA with coalescence of lobular lesions was observed. Notably, UWF FAF with green-(California) and blue excitation wavelengths (California and EIDON) revealed atypical patterns characterized by isofluorescent FAF signals (indistinguishable from surrounding tissue) or hyperautofluorescent GA lesions. In these cases, blue excitation wavelengths were more effective than green light for delineating GA, owing to increased contrast from hypoautofluorescence related to macular pigment surrounding the lesion.</p><p><strong>Conclusion: </strong>In patients with GA and concomitant ARCA, atypical FAF patterns on UWF imaging complicate the accurate delineation and monitoring of GA. Atypical FAF patterns appear due to the properties of the confocal apertures and postprocessing features of UWF imaging that allow for the detection of scleral autofluorescence in patients with reduced choroidal vasculature, pigment and thickness. In patients with concomitant ARCA, multimodal imaging plays a crucial role in precisely identifying and tracking GA progression.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143505947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Saleh Sahal H, Stepanian Rima, Juliano Joseph D, Folberg Robert, Laurie Lau-Sickon, Faia Lisa J, Weiner Adam J
{"title":"A Case of Uveal Melanoma Masquerading as Choroidal Lymphoma.","authors":"Saleh Sahal H, Stepanian Rima, Juliano Joseph D, Folberg Robert, Laurie Lau-Sickon, Faia Lisa J, Weiner Adam J","doi":"10.1097/ICB.0000000000001738","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001738","url":null,"abstract":"<p><strong>Purpose: </strong>To present a case of uveal melanoma masquerading as choroidal lymphoma.</p><p><strong>Methods: </strong>A retrospective case report in which medical and imaging records, including fundus photography, optical coherence tomography, fundus autofluorescence, fluorescein angiography, and ultrasonography were reviewed.</p><p><strong>Results: </strong>A 77-year-old woman presented with decreased vision was found to have a diffuse, shallow, yellow choroidal mass associated with RPE pigmentary changes and exudative detachment in her right eye. Multi-modal imaging demonstrated thickened choroid with a \"lumpy-bumpy\" appearance on OCT, \"leopard-spotting\" on fundus autofluorescence, and a shallow \"table-top\" configuration on B scan. Systemic workup was unremarkable. The patient elected to undergo treatment with low-dose external beam radiation for presumed choroidal lymphoma. There was no response to radiation prompting retinochoroidal biopsy, which revealed spindle-cell uveal melanoma and resulted in enucleation.</p><p><strong>Conclusion: </strong>We present a case of uveal melanoma masquerading as choroidal lymphoma. This case highlights that uveal melanoma can rarely masquerade as other entities, including uveal lymphoma.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143473179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nithya Boopathiraj, Isabella V Wagner, Ross Powers, Arman Mashayekhi
{"title":"Chorioretinitis following Bacillus Calmette-Guerin Treatment for Bladder Cancer.","authors":"Nithya Boopathiraj, Isabella V Wagner, Ross Powers, Arman Mashayekhi","doi":"10.1097/ICB.0000000000001736","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001736","url":null,"abstract":"<p><strong>Purpose: </strong>To report a rare case of multifocal chorioretinitis subsequent to intravesical Bacillus Calmette-Guerin (BCG) therapy for bladder cancer.</p><p><strong>Methods: </strong>Case report.</p><p><strong>Results: </strong>A 74-year-old man presented with ill-defined visual changes in his right eye (RE), along with constitutional symptoms such as low-grade fever, chills with rigors, night sweats, headache, dry cough, memory problems, and unintentional weight loss. These symptoms occurred 11 months after receiving intravesical BCG treatment for bladder cancer. His visual acuity in the RE was 20/30. Examination of his RE revealed multiple yellow white chorioretinal lesions, and optical coherence tomography imaging showed focal involvement of the choroid and overlying neurosensory retina. Blood-based testing utilizing next generation sequencing for detection of microbial cell free DNA (Karius test ®) was positive for Mycobacterium tuberculosis complex. The patient was treated with 12 months of triple therapy consisting of isoniazid, rifampin, and ethambutol. The chorioretinal lesions showed complete regression to flat scars, with no evidence of recurrence 15 months after discontinuation of systemic treatment.</p><p><strong>Conclusion: </strong>Despite being rare, the possibility of mycobacterial chorioretinitis should be considered in patients with new-onset visual symptoms following intravesical BCG for treatment of bladder cancer.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143473180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Williamson Jeremy P, Sarafian Richard Y, Pham David, Park Joseph J
{"title":"A curious case of endogenous Ramularia endophthalmitis presenting as acute angle closure.","authors":"Williamson Jeremy P, Sarafian Richard Y, Pham David, Park Joseph J","doi":"10.1097/ICB.0000000000001734","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001734","url":null,"abstract":"<p><strong>Purpose: </strong>To report on an interesting case of fungal endogenous endophthalmitis in a patient that was referred in for acute angle closure. A unique presentation with a novel pathogen, Ramularia, identified on vitreous sampling.</p><p><strong>Methods: </strong>Case report.</p><p><strong>Results: </strong>This report describes a patient referred for presumed angle closure with a painful red left eye, an intraocular pressure of 39mmHg and perception to light vision. Ramularia species DNA - a novel fungus, was identified on PCR from sterile vitreous sampling during subsequent vitrectomy, performed due to worsening vision. Following vitrectomy, the patient improved with topical voriconazole and oral posaconazole which was second line therapy after oral voriconazole caused hepatoxicity. The patient had a good visual outcome with a final visual acuity of 20/50, 18 months post presentation.</p><p><strong>Conclusion: </strong>This case highlights the unique presentation, treatment, and good outcome of a patient with endogenous endophthalmitis from a novel fungus, Ramularia.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maja Magazin, Skyler G Jones, Alexis Fazio, Dilip A Thomas, Diego Espinosa-Heidmann
{"title":"Chorioretinal Findings in Intraocular IgG4-Related Disease: A Case Report and Review of the Literature.","authors":"Maja Magazin, Skyler G Jones, Alexis Fazio, Dilip A Thomas, Diego Espinosa-Heidmann","doi":"10.1097/ICB.0000000000001735","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001735","url":null,"abstract":"<p><strong>Purpose: </strong>This manuscript reports intraocular IgG4-related disease (IgG4-RD) presenting as panuveitis with a large subretinal mass and contralateral orbital mass. It includes a literature review highlighting IgG4-RD's diagnostic challenges, emphasizing clinical, histopathologic, and multimodal-imaging findings.</p><p><strong>Methods: </strong>PubMed was searched for \"all fields\" with keywords \"IgG4\" AND \"retina\" AND \"choroid\" from 1974 to March 2024, revealing 16 cases of 23 eyes detailing IgG4-related chorioretinal manifestations.</p><p><strong>Results: </strong>The most common presenting symptom was painful vision loss (31%). Chorioretinal findings included: large subretinal or choroidal masses (35%), multifocal subretinal lesions (26%), and retinal detachment (22%). Associated ophthalmic findings included: scleritis or sclerouveitis (52%), vitritis (48%), orbital disease (13%), and papillitis (9%). IgG4+/IgG ratios in ocular tissue varied 20-54.5%. IgG4+ plasma cells per high power field ranged 50-133. Ophthalmic modalities used to aid in diagnosis included B-scan ultrasonography (US-B, 56%), optical coherence tomography (OCT, 31%), fluorescein angiography (FA, 25%), fundus-autofluorescence (FAF, 13%), and indocyanine-green angiography (ICGa, 13%). Masses on B-scan were heterogenous or homogenous with medium-to-high reflectivity. Magnetic resonance imaging (MRI) evaluation was hypointense on T2-weighted imaging (75%) and hyperintense on T1-weighted imaging (38%) with progressive contrast enhancement (50%). Management required a multidisciplinary approach with steroid and steroid-sparing agents.</p><p><strong>Conclusion: </strong>IgG4-RD has diverse and variable ocular presentations and diagnostic manifestations. Definitive diagnosis is made via biopsy. Further studies are necessary for non-invasive diagnosis.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469991","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}