{"title":"A NOVEL FINDING IN THE DIAGNOSIS OF PRIMARY VITREORETINAL LYMPHOMA: A CASE REPORT.","authors":"Arash Maleki","doi":"10.1097/ICB.0000000000001489","DOIUrl":"10.1097/ICB.0000000000001489","url":null,"abstract":"<p><strong>Purpose: </strong>Introduce a unique case of primary vitreoretinal lymphoma with a new optical coherence tomography finding.</p><p><strong>Methods: </strong>A case report. A 67-year-old healthy man with complaints of blurry vision in his right eye.</p><p><strong>Results: </strong>The patient's visual acuity was 20/60 and 20/20 in the right and left eyes, respectively. Anterior segment examination of the right eye demonstrated mild inflammation. Dilated fundoscopy revealed 2+ vitreous haze and 4+ disk edema. Optical coherence tomography of the macula in the right eye revealed optic nerve head swelling and thickening of the retina. Fluorescein angiography demonstrated mild leakage and staining of vessels along the inferotemporal arcade in the right eye. Labs were within normal limits except positive Herpes simplex virus 1 IgG. Initially, the patient was treated for herpetic panuveitis for 3 weeks with a favorable response. However, the clinical condition deteriorated as a new abnormality was identified in the macular region of the right eye through optical coherence tomography. Considering intraocular lymphoma as a potential diagnosis, the patient underwent a diagnostic vitrectomy. The vitreous sample analysis confirmed primary vitreoretinal lymphoma through immunohistochemistry and flow cytometry. The patient exhibited a rapid response after the initiation of intravenous and intravitreal methotrexate treatment.</p><p><strong>Conclusion: </strong>The presence of subretinal fluid accompanied by suspended hyperreflective lesions originating from the roof of the subretinal fluid pocket on the optical coherence tomography of macula \"stalactite sign\" may serve as a characteristic sign indicative of primary vitreoretinal lymphoma; however, further investigation using robust studies is necessary to examine this hypothesis.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":"28-33"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41219920","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":"FAMILIAL EXUDATIVE VITREORETINOPATHY WITH NASAL RETINAL INVOLVEMENT: A RARE PRESENTATION.","authors":"Amelia Cooper, Henry Stevens, Ivan Batlle","doi":"10.1097/ICB.0000000000001511","DOIUrl":"10.1097/ICB.0000000000001511","url":null,"abstract":"<p><strong>Purpose: </strong>To present a case of familial exudative vitreoretinopathy with nasal retinal involvement and aim to describe the clinical presentation, diagnostic findings, management strategies, and genetic testing.</p><p><strong>Methods: </strong>An evaluation of a 31-year-old female patient with familial exudative vitreoretinopathy presenting with predominate nasal retinal involvement. Diagnostic tests-including fundus examination, optical coherence tomography, fluorescein angiography, and genetic testing-were performed. The patient's management involved intravitreal injection of 1.25 mg/0.05 mL of bevacizumab in the left eye and sectoral panretinal photocoagulation in both eyes.</p><p><strong>Results: </strong>The patient exhibited discrete neovascularization and tortuosity predominantly localized to the nasal retinal blood vessels. Optical coherence tomography imaging showed macular schisis and vitreomacular traction without retinal detachment. Genetic testing identified a likely pathogenic variant associated with autosomal dominant and recessive exudative vitreoretinopathy. Treatment with bevacizumab and panretinal photocoagulation resulted in regression of neovascularization and improvement in macular schisis. Conclusion: This case report highlights an atypical presentation of familial exudative vitreoretinopathy with nasal retinal involvement. Early recognition and genetic testing aids in diagnosis and management.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":"107-111"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11649170/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71488881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Caroline C Cotton, Anirudha S Chandrabhatla, Patrick H Andrews, Benjamin W Purrow, Yevgeniy Eugene Shildkrot
{"title":"BELZUTIFAN FOR TREATMENT OF GIANT RETINAL HEMANGIOBLASTOMA WITH EXTRASCLERAL EXTENSION ASSOCIATED WITH VON HIPPEL-LINDAU SYNDROME.","authors":"Caroline C Cotton, Anirudha S Chandrabhatla, Patrick H Andrews, Benjamin W Purrow, Yevgeniy Eugene Shildkrot","doi":"10.1097/ICB.0000000000001516","DOIUrl":"10.1097/ICB.0000000000001516","url":null,"abstract":"<p><strong>Purpose: </strong>To describe the clinical response and safety profile of the novel HIF-2ɑ inhibitor belzutifan in treating a giant retinal hemangioblastoma with extrascleral extension associated with von Hippel-Lindau syndrome.</p><p><strong>Methods: </strong>A 71-year-old woman with Von Hippel-Lindau syndrome presented with a giant retinal hemangioblastoma with extrascleral extension in her only remaining eye. She had no light perception in the right eye and intraocular pressure was 48. She requested enucleation because of chronic pain, but because of concern for significant bleeding given the size of the neoplasm, a trial of belzutifan was initiated.</p><p><strong>Results: </strong>Within 3 months of treatment initiation, the patient reported an 80% reduction in pain. Magnetic resonance imaging showed 30% reduction in longest tumor diameter. Dose adjustments were guided by serum hemoglobin levels, allowing the patient to remain on the medication for over a year with continued tumor regression on MRI and avoid enucleation.</p><p><strong>Conclusion: </strong>Retinal hemangioblastoma with extrascleral extension is exceedingly rare and its treatment is complex, often requiring enucleation or external beam radiotherapy. This report demonstrates the use of belzutifan to safely and successfully reduce ocular tumor burden of complicated retinal hemangioblastoma with extrascleral extension, ultimately decreasing the need for enucleation.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":"66-69"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11649176/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136400291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Anterior uveitis, pigmentary retinopathy, and pars plana exudates in Celiac disease.","authors":"Aniruddha Agarwal, Surinder S Rana, Kirti Gupta","doi":"10.1097/ICB.0000000000001708","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001708","url":null,"abstract":"<p><strong>Purpose: </strong>To report a case of bilateral anterior uveitis, pigmentary retinopathy, and pars plana exudates in a patient with Celiac disease with complete resolution of inflammation following gluten-free diet.</p><p><strong>Methods: </strong>Retrospective case report.</p><p><strong>Results: </strong>A 19-year-old Asian Indian girl presented with bilateral non-granulomatous anterior uveitis for the past 2 months. Examination revealed bilateral pigmentary retinopathy and exudates near the inferior pars plana in the left eye. Laboratory evaluation revealed positive anti-tissue transglutaminase antibody, low thyroid stimulating hormone, low serum ferritin, anemia, and vitamin D deficiency. Her duodenal biopsy was confirmatory for Celiac disease. After initiation of gluten-free diet, there was complete resolution of anterior segment inflammation and vitreous exudates.</p><p><strong>Conclusions: </strong>Celiac disease is a rare cause of corticosteroid-resistant uveitis with a handful of cases reported in the literature. This condition has protean manifestations and resolves with gluten-free diet.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928541","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}
Spencer S Burt, Yakub Bayhaqi, Shuibin Ni, Yifan Jian, J Peter Campbell, Benjamin K Young
{"title":"Nerve Fiber Layer Schisis in Coats Disease.","authors":"Spencer S Burt, Yakub Bayhaqi, Shuibin Ni, Yifan Jian, J Peter Campbell, Benjamin K Young","doi":"10.1097/ICB.0000000000001709","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001709","url":null,"abstract":"<p><strong>Purpose: </strong>To describe two cases of pediatric patients with Coats disease who developed nerve fiber layer (NFL) schisis.</p><p><strong>Methods: </strong>Observational case series.</p><p><strong>Results: </strong>Two male pediatric patients, ages 2 and 14, who were being treated for Coats disease were found to have NFL schisis on optical coherence tomography. Both were treated with panretinal photocoagulation (PRP) and indirect laser. The 14-year-old patient has had recurrent disease with a most recent visual acuity of 20/80+1 in the affected eye. The 2-year-old patient continues to be followed after receiving two rounds of PRP, followed by two rounds of superotemporal laser.</p><p><strong>Conclusion: </strong>Nerve fiber layer schisis is a rare manifestation of Coats disease.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928592","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}
Amrita Pradhan, Ahana Sen, Ruchira Chowdhury, Survee Sharma, Bristi Majumdar, Surabhi Chattree, Debmalya Das, Eesh Nigam, Kumar Saurabh, Rupak Roy
{"title":"OCT Angiography characteristics of Bilateral Mactel type 1: revealing the Aneurysmo occlusive variety.","authors":"Amrita Pradhan, Ahana Sen, Ruchira Chowdhury, Survee Sharma, Bristi Majumdar, Surabhi Chattree, Debmalya Das, Eesh Nigam, Kumar Saurabh, Rupak Roy","doi":"10.1097/ICB.0000000000001707","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001707","url":null,"abstract":"<p><strong>Purpose: </strong>To report OCTA (Optical coherence tomography angiography) characteristics of bilateral MacTel type 1 with capillary obliteration.</p><p><strong>Method: </strong>Colour fundus photo (CFP), OCT angiography (OCTA), Fundus fluorescein angiography (FFA), Fundus autofluorescence (FAF).</p><p><strong>Result: </strong>We describe three cases of bilateral MacTel Type 1 with a special emphasis on OCTA characteristics.</p><p><strong>Conclusion: </strong>OCTA is a non-invasive method to detect capillary telangiectasia and abnormal vascular tortuosity in the superficial capillary plexus and deep capillary plexus. OCT angiography demonstrates that bilateral mactel type 1 may have both aneurysmal and occlusive components.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928606","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}
Kyle M Green, Taariq K Mohammed, Jonathan F Russell, Timothy M Boyce
{"title":"Drusen Regression after Macula-involving Rhegmatogenous Retinal Detachment Repair.","authors":"Kyle M Green, Taariq K Mohammed, Jonathan F Russell, Timothy M Boyce","doi":"10.1097/ICB.0000000000001711","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001711","url":null,"abstract":"<p><strong>Purpose: </strong>To investigate the effect of macula-involving rhegmatogenous retinal detachment (RRD) repair on drusen regression.</p><p><strong>Methods: </strong>A retrospective review was performed of patients with drusen who underwent macula-involving RRD repair. Longitudinal optical coherence tomography scans were reviewed by three graders, and each case was grouped into one of three categories: drusen regression, drusen persistence, or mixed.</p><p><strong>Results: </strong>14 eyes with drusen that underwent macula-involving RRD repair were identified. Surgical repair consisted of pars plana vitrectomy (PPV) in 7 eyes, combined PPV and scleral buckle in 6 eyes, and primary buckle with cryotherapy in 1 eye. Regression of drusen occurred in 6 eyes (43%), persistence of drusen in 5 eyes (36%), and 3 eyes were categorized as mixed (21%). One patient with drusen regression with long-term follow up resulted in geographic atrophy.</p><p><strong>Conclusion: </strong>Although the course of drusen following repair of macula-involving RRDs was highly variable, roughly half of eyes demonstrated marked drusen regression.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928546","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}
Luis Zeman, Mauricio Martínez Cartier, Cristóbal Couto, Ariel Schlaen
{"title":"Optical Coherence Tomography Angiography findings in Syphilitic Outer Retinitis.","authors":"Luis Zeman, Mauricio Martínez Cartier, Cristóbal Couto, Ariel Schlaen","doi":"10.1097/ICB.0000000000001706","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001706","url":null,"abstract":"<p><strong>Purpose: </strong>to report optical coherence tomography angiography findings in syphilitic outer retinopathy, a singular clinical manifestation of ocular syphilis.</p><p><strong>Methods: </strong>case report.</p><p><strong>Results: </strong>Multimodal imaging including optical coherence tomography angiography was performed in a patient presenting syphilitic outer retinopathy. Optical coherence tomography angiography showed multifocal areas of flow deficit at the choriocapillaris level at presentation, whereas no vascular abnormalities were observed at deep and superficial capillary plexuses. After a one-year follow-up period, choriocapillaris flow showed an almost complete recovery.</p><p><strong>Conclusion: </strong>Syphilitic outer retinopathy represents a singular clinical manifestation of ocular syphilis. The almost complete recovery of initial ischemic changes at one year of follow-up suggests that this entity may have a good prognosis with timely and proper antibiotic treatment.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928613","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}
Angela A Cao, Christian D Curran, Pedro Monsalve Diaz, Emily Hause, Dara Koozekanani, Max R Johnson, Karen R Armbrust
{"title":"Epstein-Barr Virus-Associated Frosted Branch Angiitis: A Case Report and Brief Review.","authors":"Angela A Cao, Christian D Curran, Pedro Monsalve Diaz, Emily Hause, Dara Koozekanani, Max R Johnson, Karen R Armbrust","doi":"10.1097/ICB.0000000000001710","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001710","url":null,"abstract":"<p><strong>Purpose: </strong>To report the clinical presentation, treatment course, and outcome of a case of bilateral frosted branch angiitis (FBA) and neuroretinitis associated with acute Epstein-Barr virus (EBV) infection in a pediatric patient with Turner Syndrome.</p><p><strong>Methods: </strong>Case report with multimodal ocular imaging and extensive systemic workup.</p><p><strong>Results: </strong>A 16-year-old female with Turner syndrome presented with acute bilateral vision loss, hearing loss, and ataxia. Ocular examination showed bilateral extensive perivascular sheathing, optic disc edema, and macular exudates. Fluorescein angiography showed focal retinal ischemia and late optic disc leakage in both eyes. Macular optical coherence tomography was remarkable for intraretinal and subretinal fluid with scattered hyperreflective foci in both eyes. MRI brain and orbits showed a supratentorial T2 signal without enhancement, consistent with inactive vasculopathy. Results of a broad systemic workup were notable only for elevated EBV viral capsid antigen IgM with undetectable EBV viral capsid antigen IgG. After treatment with intravenous methylprednisolone followed by oral prednisone, the patient's best corrected visual acuity in both eyes improved from 20/200 to 20/20.</p><p><strong>Conclusion: </strong>Ocular symptoms and imaging biomarkers in this case of EBV-associated FBA resolved after high-dose systemic corticosteroid monotherapy. Although treatment with antiviral medications is reported in other FBA cases associated with acute EBV infection, this case underscores the treatable nature of this disease without antivirals, the use of which are not supported by the broader literature for EBV infection or post-viral inflammation.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142928548","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}
Elodie Bousquet, Ahmad Santina, Swathi Somisetty, Veronica Romero Morales, Gary N Holland, David Sarraf
{"title":"Hydroxychloroquine retinopathy in a 23-year-old male.","authors":"Elodie Bousquet, Ahmad Santina, Swathi Somisetty, Veronica Romero Morales, Gary N Holland, David Sarraf","doi":"10.1097/ICB.0000000000001704","DOIUrl":"https://doi.org/10.1097/ICB.0000000000001704","url":null,"abstract":"<p><strong>Purpose: </strong>To report a case of hydroxychloroquine (HCQ) retinopathy after long-term exposure in a 23-year-old male.</p><p><strong>Methods: </strong>Multimodal imaging including fundus photography, fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT), and en face OCT were performed, in addition to functional testing with full-field electroretinography (ERG) and Humphrey visual field (HVF).</p><p><strong>Results: </strong>A 23-year-old man with a history of juvenile systemic lupus erythematosus and HCQ treatment for 13 years at a dosage of 200 mg/d (cumulative dose: 949 grams) presented to the retinal clinic (DS). Although fundus photography and FAF were unremarkable, SD-OCT and en face OCT showed paracentral ellipsoid zone loss with thinning of the outer nuclear layer, OS greater than OD consistent with HCQ retinopathy. HVF 10-2 showed possible nasal loss OU. Genetic testing revealed heterozygous mutations in the CNGA1 and CRX genes but full-field ERG was unremarkable without evidence of a cone dystrophy. Family history was negative for genetic disease.</p><p><strong>Conclusion: </strong>This report highlights a case of HCQ retinopathy in a young patient who started treatment at the age of 10 years. There are currently no specific guidelines for the screening of pediatric patients, and studies evaluating the effect of HCQ on children are lacking. Whether the genetic carrier status rendered the patient more susceptible to the toxic effect of HCQ remains to be determined.</p>","PeriodicalId":53580,"journal":{"name":"Retinal Cases and Brief Reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142848048","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}