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Background: Chronic myeloid leukemia (CML) can manifest ocular complications stemming from hematologic irregularities or direct infiltration of neoplastic cells. This article details the case of a patient with newly diagnosed CML exhibiting elevated platelet counts (PLT) who developed panuveitis accompanied by retinal vascular occlusion. Case presentation: A 52-year-old woman experienced a notable decline in vision in her left eye over a 2-week period. Classical anterior uveitis, vitreous cavity opacity, optic nerve edema, and retinal vascular obstruction were observed. The right eye exhibited papilledema and retinal vein tortuosity. Despite admission, the condition of both eyes deteriorated, accompanied by a continuous increase in PLT. She was diagnosed with CML based on bone marrow biopsy and chromosomal examination. Following platelet apheresis therapy and chemotherapy, the condition of her right eye significantly improved, but the left eye's condition remained irreversible. Conclusions: This is a rare case of newly diagnosed CML presenting with diverse ocular manifestations in both eyes. The disparate outcomes in eyes with varying lesion stages underscore the importance of prompt diagnosis. [ABSTRACT FROM AUTHOR]

Abstract Background Chronic myeloid leukemia (CML) can manifest ocular complications stemming from hematologic irregularities or direct infiltration of neoplastic cells. This article details the case of a patient with newly diagnosed CML exhibiting elevated platelet counts (PLT) who developed panuveitis accompanied by retinal vascular occlusion. Case presentation A 52-year-old woman experienced a notable decline in vision in her left eye over a 2-week period. Classical anterior uveitis, vitreous cavity opacity, optic nerve edema, and retinal vascular obstruction were observed. The right eye exhibited papilledema and retinal vein tortuosity. Despite admission, the condition of both eyes deteriorated, accompanied by a continuous increase in PLT. She was diagnosed with CML based on bone marrow biopsy and chromosomal examination. Following platelet apheresis therapy and chemotherapy, the condition of her right eye significantly improved, but the left eye’s condition remained irreversible. Conclusions This is a rare case of newly diagnosed CML presenting with diverse ocular manifestations in both eyes. The disparate outcomes in eyes with varying lesion stages underscore the importance of prompt diagnosis.

Abstract Background With the advent of targeted therapies, the survival prognosis for metastatic tumors has extended, and it has become necessary to diagnose and consider treatment that takes into account Quality of Life for metastatic tumors of the eye. The reports of checking tumor marker in the aqueous humor for diagnosis of metastatic intraocular tumors are few. Here, we report a case of masquerade syndrome with secondary glaucoma in which a high carcinoembryonic antigen (CEA) level in the aqueous humor could assist diagnosis, and continuing targeted therapy and trabeculectomy were effective. Case presentation A 73-year-old man was referred to us for iritis and high intraocular pressure (IOP) with severe eye pain in the left eye. He had Stage IVB lung adenocarcinoma treated with a molecularly targeted drug, Osimertinib. His best corrected visual acuity was 0.15, and IOP was 52 mmHg in the left eye. Anterior chamber cells (+), numerous small nodules in the iris, and small masses in the inferior angle were observed. In the aqueous humor, the CEA level was higher than in the blood. Napsin A and Thyroid Transcription Factor-1 (TTF-1) positive cells showed in the resected tissue at iridectomy performed during trabeculectomy. The pathological diagnosis of metastatic iris tumor of the lung adenocarcinoma was made, and we injected bevacizumab intravitreally once and continued Osimertinib. His IOP lowered to 8–10 mmHg, and the iris masses disappeared. He lost vision by metastasis to the left optic nerve after termination of Osimertinib one and a half years later. The metastasis shrank after restarting the drug. He passed away from an exacerbation of his primary lung cancer two years and nine months after the first visit. Although he lost vision in his left eye, the metastatic tumor in his left eye and optic nerve had disappeared, and his quality of life was maintained without any pain in his eye. Conclusions Checking tumor markers in the aqueous humor can aid in diagnosis, and aggressive treatment of metastatic iris tumors must help maintain patients’ Quality of Life.

Background: With the advent of targeted therapies, the survival prognosis for metastatic tumors has extended, and it has become necessary to diagnose and consider treatment that takes into account Quality of Life for metastatic tumors of the eye. The reports of checking tumor marker in the aqueous humor for diagnosis of metastatic intraocular tumors are few. Here, we report a case of masquerade syndrome with secondary glaucoma in which a high carcinoembryonic antigen (CEA) level in the aqueous humor could assist diagnosis, and continuing targeted therapy and trabeculectomy were effective. Case presentation: A 73-year-old man was referred to us for iritis and high intraocular pressure (IOP) with severe eye pain in the left eye. He had Stage IVB lung adenocarcinoma treated with a molecularly targeted drug, Osimertinib. His best corrected visual acuity was 0.15, and IOP was 52 mmHg in the left eye. Anterior chamber cells (+), numerous small nodules in the iris, and small masses in the inferior angle were observed. In the aqueous humor, the CEA level was higher than in the blood. Napsin A and Thyroid Transcription Factor-1 (TTF-1) positive cells showed in the resected tissue at iridectomy performed during trabeculectomy. The pathological diagnosis of metastatic iris tumor of the lung adenocarcinoma was made, and we injected bevacizumab intravitreally once and continued Osimertinib. His IOP lowered to 8–10 mmHg, and the iris masses disappeared. He lost vision by metastasis to the left optic nerve after termination of Osimertinib one and a half years later. The metastasis shrank after restarting the drug. He passed away from an exacerbation of his primary lung cancer two years and nine months after the first visit. Although he lost vision in his left eye, the metastatic tumor in his left eye and optic nerve had disappeared, and his quality of life was maintained without any pain in his eye. Conclusions: Checking tumor markers in the aqueous humor can aid in diagnosis, and aggressive treatment of metastatic iris tumors must help maintain patients' Quality of Life. [ABSTRACT FROM AUTHOR]

To describe the ophthalmic findings and diagnosis of a case of intravascular large B-cell lymphoma. Clinical case observational report. A Spanish 51-year-old man referred to our hospital with a diagnosis of panuveitis. The patient presented with blurred vision, photophobia, fever, and weight loss. Ocular examination revealed anterior uveitis, vitritis, and multiple round and oval creamy spots on the posterior pole. Fluorescein angiography, optical coherence tomography (OCT), and angio-OCT were used for the ocular examination. The diagnosis of lymphoma was formulated on the basis of a random normal skin biopsy, which showed significant CD20 cellularity within the vessels and extensive CD3 expression. Intravascular lymphoma is a rare form of extranodal diffuse large B-cell lymphoma, often with delayed diagnosis because of the nonspecific symptoms. Hence, random skin biopsy could be useful in the diagnosis. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo report the first case of Kytococcus schroeteri ocular infection manifesting as chronic post-operative endophthalmitis (CPOE) following routine phacoemulsification with intraocular lens (IOL) implantation. This gram-positive bacterium is recognised as a cause of hardware infections in immunocompetent hosts, such as prosthetic cardiac valves, ventriculoperitoneal shunts, and orthopaedic hardware, often necessitating surgical removal of the infected prostheses for cure.Case report and literature review.A 64-year-old male with a history of uncomplicated cataract extraction and IOL insertion had multiple presentations over 6 years, with relapsing-remitting intraocular inflammation with multiple negative vitreous cultures treated as non-infectious panuveitis. An eventual positive vitreous culture for K. schroeteri led to a diagnosis of CPOE, which was successfully treated with vitrectomy, removal of IOL, and intravitreal vancomycin injections. However, advanced vision loss occurred due to secondary glaucoma.K. schroeteri is a novel cause of CPOE, which is typically attributed to other low virulence organisms. As with infections of non-ocular implanted hardware by K. schroeteri, surgical removal of the infected IOL-capsule complex was required for cure. The CPOE diagnosis requires a combination of high index of suspicion and culture of vitreous and capsular material. The role of IOL removal for the treatment of CPOE is discussed. [ABSTRACT FROM AUTHOR]

Purpose: To report an aggressive and rapidly progressive case of atypical T-cell lymphoproliferative infiltration both with intraocular and orbital involvement and preretinal infiltrates.Methods: Medical records and imaging of the patient were retrospectively reviewed.Case presentation: A 25-year-old woman presented first with preretinal infiltrates resembling uveitis and developed orbital and intracranial signs eventually during her evaluation. Clinical presentation worsened gradually. The patient developed bilateral proptosis, pupillary dilation and uvula deviation. Diagnostic orbital incision biopsy revealed T-cell lymphoproliferative disease. Conclusion: This case gives evidence that intraocular involvement due to T-cell lymphoproliferative disease may present as a masquerade syndrome and should be kept in mind in patients with extraordinary presentation.

Purpose: To describe ocular sarcoid-like reaction as a unique manifestation of paraneoplastic syndrome in the context of concurrent pulmonary sarcoid-like reaction and lung adenocarcinoma. Methods: Single case report and narrative review. Results: A 59-year-old male patient presented with a year-long history of diminished vision and weight loss. Clinical examination revealed panuveitis and multiple chorioretinal lesions. A CT scan of the chest revealed mediastinal and hilar lymphadenopathy as well as a spiculated right lower lung nodule concerning for malignancy. Subsequent bronchoscopy and biopsy confirmed lung adenocarcinoma and non-caseating granulomas in sentinel lymph nodes. Conclusion: Although pulmonary granulomatous reaction can be seen in the setting of lung malignancy, and ocular sarcoid-like reaction may present as a paraneoplastic manifestation of systemic malignancy, the presence of concomitant pulmonary and ocular sarcoid-like reactions distinguishes this case. The findings underscore the importance of a systemic workup for patients with concerning constitutional symptoms, as paraneoplastic syndromes and metastatic diseases may mimic uveitis. Recognition of paraneoplastic sarcoidosis as a potential clinical manifestation is essential, especially in patients with chronic illness indicators, necessitating a comprehensive evaluation for malignancy.

Uveitis masquerade syndromes are a diverse group of clinical entities which mimic conventional immune‐mediated uveitis due to the presence of inflammatory signs but are resistant to anti‐inflammatory therapy. Misdiagnosis hinders appropriate management in these conditions and may result in poor outcomes. This review discusses commonly encountered neoplastic and non‐neoplastic disease processes that masquerade as intraocular inflammation with a focus on relevant clinical features and adjunctive investigations that are helpful in reaching a correct diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy. Methods: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported. Results: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival. Conclusions: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function. [ABSTRACT FROM AUTHOR]

Purpose:This is a retrospective case report illustrating the diagnostic and therapeutic challenges associated with a chronic rhegmatogenous retinal detachment masquerading as a severe panuveitis with intense anterior chamber inflammation. We have included clinical features, anterior segment and fundus photography, B-scan ultrasonography, fluorescein angiography, and intraoperative findings. Observations:A 26-year-old male presented with features of unilateral panuveitis: hypotony, anterior segment inflammation (posterior synechiae and anterior chamber cell with fibrin clumping), diffuse choroidal thickening, and retinal detachment. Laboratory investigations for infectious or rheumatologic processes were negative, and empiric systemic corticosteroid therapy was unsuccessful. This prompted suspicion for an alternate primary etiology, and pars plana vitrectomy revealed small retinal breaks as the underlying cause of the retinal detachment and inflammation. Conclusions:Rhegmatogenous retinal detachments are a known cause of intraocular inflammation. Nevertheless, it remains a challenge to recognize retinal breaks in this setting, particularly with robust anterior segment inflammation and posterior findings resembling severe exudative uveitis. Being aware of this unique presentation may prevent delays in diagnosis and have important prognostic implications.

X-linked retinoschisis (XLRS) shows features also seen in patients with uveitis and is recognized as an uveitis masquerade syndrome. This retrospective study aimed to describe characteristics of XLRS patients with an initial uveitis diagnosis and to contrast these to patients with an initial XLRS diagnosis. Patients referred to a uveitis clinic, which turned out to have XLRS (n = 4), and patients referred to a clinic for inherited retinal diseases (n = 18) were included. All patients underwent comprehensive ophthalmic examinations, including retinal imaging with fundus photography, ultra-widefield fundus imaging, and optical coherence tomography (OCT). In patients with an initial diagnosis of uveitis, a macular cystoid schisis was always interpreted as an inflammatory macular edema; vitreous hemorrhages were commonly interpreted as intraocular inflammation. Patients with an initial diagnosis of XLRS rarely (2/18; p = 0.02) showed vitreous hemorrhages. No additional demographic, anamnestic, and anatomical differences were found. An increased awareness of XLRS as a uveitis masquerade syndrome may facilitate early diagnosis and may prevent unnecessary therapies. [ABSTRACT FROM AUTHOR]

Ocular tuberculosis is a great mimicker of various uveitis entities. We present a case of a 29-year-old male who came in with blurring of vision and floaters in the left eye. On examination, the left eye had anterior chamber cells and vitritis associated with retinitis. He had no other symptoms. The initial presentation was consistent with ocular toxoplasmosis, and he was started on oral sulfamethoxazole-trimethoprim and showed a good response to the treatment. However, work-up revealed negative toxoplasma antibody titers but a positive inefeon-gamma release assay test and Mantoux test, making the diagnosis of ocular tuberculosis more likely. The patient was shifted to antituberculous therapy, which eventually resulted in the resolution of the inflammation with a recovery of the visual acuity. The diagnosis of ocular tuberculosis requires a detailed medical history as well as microbiologic and immunologic studies. A high index of suspicion by the treating ophthalmologist is necessary to reveal the diagnosis.

Purpose: Tumor metastases to the retina are a relatively rare occurrence. We report a unique case of retinal metastasis of a systemic malignancy with clinical and histopathologic correlations. Observations: A 62-year-old female with a history of stage IV small cell carcinoma of the lung (SCC, status post chemotherapy and maintenance immunotherapy) presented with hand motions vision and vitreous hemorrhage, status post prior vitrectomy and biopsy that was non-diagnostic. She was found to have unilateral retinal metastatic tumor and underwent a repeat vitrector-assisted biopsy which confirmed the diagnosis. The eye became blind and painful due to recurrent non-clearing vitreous hemorrhage and ghost cell glaucoma and was enucleated. Detailed histopathologic analysis of the globe confirmed small cell carcinoma metastatic to the retina and vitreous cavity and sparing the choroid. Conclusions and importance: This case demonstrates the importance of maintaining a high index of suspicion for metastasis in patients with a known history of malignancy who present with new vitreoretinal lesions.

A 66-year-old man presented with chronic bilateral periorbital edema with associated yellowish hue, scattered violaceous smooth macules and contracture of the forehead. He had undergone dental surgery 3 months prior to symptom onset. Laboratory workup for common causes of eyelid edema was unremarkable and MRI of the orbits was unrevealing. The patient did not respond to oral corticosteroids or antibiotics. Punch biopsies were obtained which revealed atypical lymphatic endothelial cells consistent with a diagnosis of cutaneous angiosarcoma. The patient was deemed not to be a surgical candidate and underwent 3 cycles of immunotherapy with limited response. He declined further treatment and transitioned to hospice care. Although cutaneous angiosarcoma uncommonly involves the periorbital region, it should be considered in the differential diagnosis of eyelid edema as early recognition and treatment are critical to prevent rapid intradermal spread and metastases. [ABSTRACT FROM AUTHOR]

Abstract Purpose To report an unusual case of unilateral anterior segment large B-cell intraocular lymphoma (IOL) presenting as a recurrent hypopyon anterior uveitis. Case presentation A 55-year-old female was referred because of recurrent unilateral anterior hypopyon uveitis with partial response to topical corticosteroid. All of the laboratory tests, review of systems and ocular sampling results were unremarkable. Given a high concern for masquerades syndromes, cytological specimens were obtained 3 times and the last sample showed large B cell lymphoma. First, it appeared confined to the eye and initially responded favorably to local chemotherapy (methotrexate and rituximab) but later went on to develop systemic involvement. CNS lymphoma was detected on the third brain MRI 6 months following ocular involvement. At this time, systemic chemotherapy was started. In the last 18 months’ follow-up, visual acuity was 20/30 in the right eye without posterior segment or fellow eye involvement. Conclusion Unusual presentation of intraocular lymphoma as a unilateral isolated anterior hypopyon uveitis should be kept in mind. This report emphasizes the importance of precise work-ups and multiple ocular biopsies to confirm the diagnosis of intraocular lymphoma.

(1) Background: To determine the diagnostic value of vitreous cytology in patients with vitreoretinal lymphoma (VRL) and evaluate its diagnostic accuracy relative to that of other diagnostic tests. (2) Methods: In total, 38 eyes from 38 patients with VRL who underwent diagnostic vitrectomy and were followed up for at least 6 months were analyzed. The clinical manifestations and VRL diagnostic rates for all diagnostic tests were determined. (3) Results: The presence of vitreous cells/opacity was the most common ophthalmic finding (97.4%), followed by sub-retinal pigment epithelial infiltration (65.8%) and retinal hemorrhage (21.1%). The VRL diagnostic rates were 89.3% for interleukin (IL)-10 levels > 50 pg/mL; 82.1% for IL-10/IL-6 ratios > 1; 60.0% and 63.3% for immunoglobulin heavy chain and kappa light chain clonality assays, respectively; and 44.4% for vitreous cytology. The VRL diagnostic rate for vitreous cytology was significantly lower in the steroid pretreatment group than in the non-steroid pretreatment group (p = 0.007). (4) Conclusions: The VRL detection rate for vitreous cytology was lower than that for the other tests, especially in patients who received steroid pretreatment. These findings suggest that even if vitreous cytology findings are negative, other tests and characteristic fundus findings should be evaluated to confirm VRL diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To present a case of a young adult with bilateral chronic posterior uveitis resulting from Vogt-Koyanagi-Harada (VKH) disease treated with mycophenolate mofetil who subsequently developed vitreoretinal lymphoma in the right eye. Methods: A case and its findings were analyzed. Results: A 34-year-old Hispanic woman presented with an 8-year history of chronic recurrent posterior uveitis in both eyes secondary to VKH disease. A vitreous infiltrate that was treatment resistant occurred in the right eye. Subsequently, a diagnostic vitrectomy was performed, and it was determined that the patient had developed unilateral vitreoretinal lymphoma. Conclusions: Masquerade syndromes should remain in the differential in patients with well-established uveitis who are on immunomodulatory treatment. A diagnostic vitrectomy should be considered when the treatment response is uncharacteristic. Furthermore, guidelines are lacking for central nervous system prophylaxis for isolated unilateral ocular intraocular lymphoma in young adults., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Purpose: To report the first case of Kytococcus schroeteri ocular infection manifesting as chronic post-operative endophthalmitis (CPOE) following routine phacoemulsification with intraocular lens (IOL) implantation. This gram-positive bacterium is recognised as a cause of hardware infections in immunocompetent hosts, such as prosthetic cardiac valves, ventriculoperitoneal shunts, and orthopaedic hardware, often necessitating surgical removal of the infected prostheses for cure., Methods: Case report and literature review., Results: A 64-year-old male with a history of uncomplicated cataract extraction and IOL insertion had multiple presentations over 6 years, with relapsing-remitting intraocular inflammation with multiple negative vitreous cultures treated as non-infectious panuveitis. An eventual positive vitreous culture for K. schroeteri led to a diagnosis of CPOE, which was successfully treated with vitrectomy, removal of IOL, and intravitreal vancomycin injections. However, advanced vision loss occurred due to secondary glaucoma., Conclusion: K. schroeteri is a novel cause of CPOE, which is typically attributed to other low virulence organisms. As with infections of non-ocular implanted hardware by K. schroeteri , surgical removal of the infected IOL-capsule complex was required for cure. The CPOE diagnosis requires a combination of high index of suspicion and culture of vitreous and capsular material. The role of IOL removal for the treatment of CPOE is discussed.

Purpose: To report an unusual case of unilateral anterior segment large B-cell intraocular lymphoma (IOL) presenting as a recurrent hypopyon anterior uveitis. Case presentation: A 55-year-old female was referred because of recurrent unilateral anterior hypopyon uveitis with partial response to topical corticosteroid. All of the laboratory tests, review of systems and ocular sampling results were unremarkable. Given a high concern for masquerades syndromes, cytological specimens were obtained 3 times and the last sample showed large B cell lymphoma. First, it appeared confined to the eye and initially responded favorably to local chemotherapy (methotrexate and rituximab) but later went on to develop systemic involvement. CNS lymphoma was detected on the third brain MRI 6 months following ocular involvement. At this time, systemic chemotherapy was started. In the last 18 months' follow-up, visual acuity was 20/30 in the right eye without posterior segment or fellow eye involvement. Conclusion: Unusual presentation of intraocular lymphoma as a unilateral isolated anterior hypopyon uveitis should be kept in mind. This report emphasizes the importance of precise work-ups and multiple ocular biopsies to confirm the diagnosis of intraocular lymphoma. [ABSTRACT FROM AUTHOR]

The aim of this series is to report challenges faced in diagnosis of three cases of recurrent or atypical uveitis not responding to conventional treatment. A high index of suspicion, aided by newer techniques, such as cytology, immunohistochemistry, flow cytometry of ocular fluids, and contrast-enhanced magnetic resonance imaging, may be necessary for a prompt diagnosis of uveitis masquerade syndromes.

To report a case of non-Hodgkin lymphoma (NHL) that was diagnosed 35-month of initial ocular manifestation. Retrospective chart review. A 53-year-old male presented with painless diminution of vision in both eyes. He subsequently underwent extensive laboratory investigations including multiple vitreous biopsies with a suspicion of intraocular lymphoma. Cytology from the vitreous aspirate failed to diagnose any relevant pathology. After 35-month from the onset of his ocular symptom, a brain biopsy revealed a round cell tumor suggestive of NHL. Even with high index of suspicion, consultation with ocular oncologist, imaging, and diagnostic vitrectomy, the diagnosis of lymphoma remains challenging

Purpose: To report a case with unusual initial manifestation of uveal melanoma in a 46-year-old adult. Observations: A 46-year-old man demonstrated a mid-peripheral temporal partially hyperpigmented lesion in his left eye. Initial clinical appearance as well as multimodal imaging approaches were similar to a chorioretinitic disease. Over the course of 9 months, a massive tumor growth and exudative retinal detachment could be observed. The diagnosis of choroidal melanoma was histopathologically confirmed after endoresection. Conclusions and importance: Classical choroidal melanoma manifest as pigmented, prominent tumors with >2 mm tumor thickness, exudative retinal detachment, and orange pigment. In our case, none of these classical clinical characteristic signs of melanoma were present at initial presentation, but the tumor underwent progressive development with significant tumor growth and exudative retinal detachment during the following 9 months.

Abstract Background Cases of panuveitis caused by Epstein–Barr virus (EBV) associated with primary intraocular lymphoproliferative disorder (LPD) are rare in immunocompetent individuals. Case presentation A 67-year-old man noted blurred vision in both eyes and was referred to our hospital. His best-corrected visual acuity (BCVA) was 20/20 in both eyes. He had mild inflammation in the anterior chamber but not in the vitreous of both eyes. The inflammation was resolved with topical corticosteroid but 10 months later both eyes presented recurrence. Treatment with a sub-Tenon’s injection of steroid was effective for OS but not for OD and 2 months after, the inflammation in the anterior chamber and vitreous opacities got worsen in OD and BCVA decreased to 6/20 OD. Thus, pars plana vitrectomy was performed on OD, and EBV was detected in the aqueous humor by multiplex polymerase chain reaction, and an infiltration of CD19κ positive B cells was revealed in the vitreous specimens by flow cytometry. Systemic workup revealed no other sites of lymphoproliferation, no active EBV infection, or underlying immunodeficiency. Conclusion Panuveitis caused by EBV associated with primary intraocular LPD can occur in patients with no history of congenital or acquired immunodeficiencies.

To report a rare ocular presentation of unilateral anterior uveitis with hypopyon in a 5-year-old child, suspected as masquerade. Retrospective report A 5-year-old boy presented with hypopyon-uveitis. Detailed systemic work-up was negative for masquerade and uveitis entities. He was started on oral and topical steroids but had reactivation on taper. Real-time polymerase chain reaction (PCR) test of aqueous fluid was positive for herpes simplex virus (HSV)-1 DNA. The inflammation resolved completely on oral acyclovir and topical corticosteroids. He subsequently underwent pars plana lensectomy, primary posterior capsulotomy and vitrectomy for complicated cataract. Post-operatively, vision improved to 20/400 with aphakic correction. Maintenance oral acyclovir was stopped after 3 months with no reactivation and amblyopia therapy was continued. This is a rare presentation of hypopyon uveitis due to HSV-1 in a child. The role of real-time PCR in establishing the diagnosis is crucial in such cases of diagnostic dilemma. [ABSTRACT FROM AUTHOR]

Purpose: To describe the visual acuity and anatomic outcomes of intravitreal methotrexate (MTX) for the treatment of primary vitreoretinal lymphoma (PVRL). Methods: Single-center retrospective case series of patients with a diagnosis of PVRL treated with intravitreal MTX. Patient records were reviewed for demographic information, ocular exam findings, and treatment regimens including number of MTX injections. Clinical outcomes recorded included visual acuity (VA), time to partial (PR) or complete response (CR), disease-free survival, time to relapse, and any CNS progression. Results: Ten eyes of 7 patients (4 male, 6 female) were reviewed. The mean age ± standard deviation (SD) was 70 ± 12 years. Five patients had prior or concomitant diagnosis of primary CNS lymphoma with a history of systemic chemotherapy including MTX. Three eyes (30%) exhibited isolated vitreous involvement, four (40%) had subretinal lesions, and three (30%) presented with both vitreous and subretinal disease. Mean initial logMAR VA was 0.38 ± 0.52 (Snellen visual equivalent 20/50), while mean final logMAR VA ± SD was 0.34 ± 0.27 (Snellen visual equivalent 20/40) with a mean follow-up time of 26 months (Range, 3–49 months). Patients received an average of 6 intravitreal MTX injections (Range 1–10) over the course of treatment. Two patients received concomitant systemic chemotherapy. Mean time to either PR or CR was 57 days, and 6 eyes (60%) exhibited regression with no relapse after local treatment. For the 4 eyes that eventually relapsed, the mean time ± SD to first relapse was 193 days ± 155 days, and one eye experienced a second relapse. Two of 3 patients with subretinal disease showed complete regression with extended follow-up of 1 and 4 years following treatment with less than 3 doses of intravitreal MTX. One patient with PVRL developed CNS lymphoma during the study period. VA remained stable overall between the initial treatment visit, 3, 6, and 12-months (P > 0.05 for paired comparisons of VA over time). Conclusions: Intravitreal methotrexate was well-tolerated and led to local disease response in the majority of patients at approximately 2 months after initiation of treatment of intraocular lymphoma. Further studies on the efficacy of intravitreal treatment alone versus combined systemic and intravitreal treatment are warranted. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of anterior uveitis in a patient under treatment with Cyclophosphamide / Bortezomid / Dexamethasone (CyBorD) protocol for Multiple Myeloma and concomitant use of Zoledronic acid. Methods: Observational case report of a 50-year-old woman presenting with acute, severe, bilateral uveitis on a medical history of Multiple Myeloma, undergoing the first cycle of CyBorD protocol, including infusion with Zoledronic acid. Results: Continuation of therapy, with parallel treatment with topical and per os steroids led to resolution of the inflammation. Three more chemotherapy cycles were performed with the same protocol including Zoledronic acid at a lower dosage and were all, uneventful. Conclusion: Our case mirrored uveitis cases with either masquerade syndrome due to Multiple Myeloma or following Zoledronic acid infusion, despite concomitant usage of Dexamethasone and Cyclophosphamide. Three consecutive rechallenges were performed without any topical prophylactic treatment and with no relapse of signs or symptoms. [ABSTRACT FROM AUTHOR]

Purpose: Post-transplant lymphoproliferative disorder (PTLD) represents a spectrum of disorders associated with Epstein Barr Virus infection in up to 80% of cases in the setting of pharmacologic immunosuppression following hematopoietic stem cell or solid organ transplantation. Ocular involvement is a rare finding in PTLD. Observation: We report the case of a 38-year-old man who presented with unilateral retinal infiltrates as first manifestation of PTLD relapse. Diagnosis relied on the presence of EBV DNA in anterior chamber fluids and vitrectomy that showed the presence of a B cell clone. Systemic relapse of PTLD was detected 12 weeks after retinal findings. Treatment of ocular disease included systemic injections of rituximab and intravitreal injections of methotrexate, halting the extension of retinal infiltrates. Conclusion: Ocular involvement in PTLD is rare and needs to be acknowledged because it can precede a systemic relapse of the hematological condition.

The aim of this series is to report challenges faced in diagnosis of three cases of recurrent or atypical uveitis not responding to conventional treatment. A high index of suspicion, aided by newer techniques, such as cytology, immunohistochemistry, flow cytometry of ocular fluids, and contrast-enhanced magnetic resonance imaging, may be necessary for a prompt diagnosis of uveitis masquerade syndromes. [ABSTRACT FROM AUTHOR]

To report a case of non-Hodgkin lymphoma (NHL) that was diagnosed 35-month of initial ocular manifestation. Retrospective chart review. A 53-year-old male presented with painless diminution of vision in both eyes. He subsequently underwent extensive laboratory investigations including multiple vitreous biopsies with a suspicion of intraocular lymphoma. Cytology from the vitreous aspirate failed to diagnose any relevant pathology. After 35-month from the onset of his ocular symptom, a brain biopsy revealed a round cell tumor suggestive of NHL. Even with high index of suspicion, consultation with ocular oncologist, imaging, and diagnostic vitrectomy, the diagnosis of lymphoma remains challenging. [ABSTRACT FROM AUTHOR]

Sebaceous carcinoma (SebCa), is the second most frequent malignancy of the eyelid. It often presents with non-specific clinical features and is known for mimicking benign inflammatory lesions such as chalazion. The masquerading ability of this highly invasive neoplasm can delay or mislead the diagnosis, resulting in significant morbidity and mortality. Here, we report a rare case of a 91-year-old man presenting with a lesion clinically apparent to a chalazion with a histopathologically proven diagnosis of SebCa adjacent to a chalazion. This report highlights the presentation of sebaceous carcinoma masquerade syndrome and supports a thorough histopathological examination of all surgically excised chalazions.

Purpose: This is a retrospective case report illustrating the diagnostic and therapeutic challenges associated with a chronic rhegmatogenous retinal detachment masquerading as a severe panuveitis with intense anterior chamber inflammation. We have included clinical features, anterior segment and fundus photography, B-scan ultrasonography, fluorescein angiography, and intraoperative findings. Observations: A 26-year-old male presented with features of unilateral panuveitis: hypotony, anterior segment inflammation (posterior synechiae and anterior chamber cell with fibrin clumping), diffuse choroidal thickening, and retinal detachment. Laboratory investigations for infectious or rheumatologic processes were negative, and empiric systemic corticosteroid therapy was unsuccessful. This prompted suspicion for an alternate primary etiology, and pars plana vitrectomy revealed small retinal breaks as the underlying cause of the retinal detachment and inflammation. Conclusions: Rhegmatogenous retinal detachments are a known cause of intraocular inflammation. Nevertheless, it remains a challenge to recognize retinal breaks in this setting, particularly with robust anterior segment inflammation and posterior findings resembling severe exudative uveitis. Being aware of this unique presentation may prevent delays in diagnosis and have important prognostic implications.

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Background: Cases of panuveitis caused by Epstein–Barr virus (EBV) associated with primary intraocular lymphoproliferative disorder (LPD) are rare in immunocompetent individuals. Case presentation: A 67-year-old man noted blurred vision in both eyes and was referred to our hospital. His best-corrected visual acuity (BCVA) was 20/20 in both eyes. He had mild inflammation in the anterior chamber but not in the vitreous of both eyes. The inflammation was resolved with topical corticosteroid but 10 months later both eyes presented recurrence. Treatment with a sub-Tenon's injection of steroid was effective for OS but not for OD and 2 months after, the inflammation in the anterior chamber and vitreous opacities got worsen in OD and BCVA decreased to 6/20 OD. Thus, pars plana vitrectomy was performed on OD, and EBV was detected in the aqueous humor by multiplex polymerase chain reaction, and an infiltration of CD19κ positive B cells was revealed in the vitreous specimens by flow cytometry. Systemic workup revealed no other sites of lymphoproliferation, no active EBV infection, or underlying immunodeficiency. Conclusion: Panuveitis caused by EBV associated with primary intraocular LPD can occur in patients with no history of congenital or acquired immunodeficiencies. [ABSTRACT FROM AUTHOR]

Many localized and systemic diseases in children may occur with symptoms observed during uveitis, or symptoms that may imitate uveitis. These include neoplastic diseases, such as: leukemia, lymphomas, Langerhans cell histiocytosis, retinoblastoma, medulloepithelioma, uveal melanoma and non-cancerous illnesses: juvenile xanthogranuloma, retinal detachment, persistent hyperplastic primary vitreous, Coats' disease and pigment dispersion syndrome. It is often difficult or even impossible to cooperate with young children during an ophthalmological examination, which makes the detection of subtle changes inside the eyeball even more challenging. The proper diagnosis may decide upon the right treatment at the early stage and thus improve the prognosis not only in terms of visual acuity, but also as far as the child's life is concerned. [ABSTRACT FROM AUTHOR]

Abstract Background The purpose of this single case report was to report the use of anterior segment optical coherence tomography for the diagnosis and management of a retained vegetal intraocular foreign body. Results A 23-year-old otherwise healthy male presented with a progressive vision loss in the right eye (RE). He reported a mild ocular trauma with a tree leaf 1 year ago followed by recurrent episodes of redness and pain in the RE that partially resolved after a self-medication with topical steroids. Visual acuity of the RE was limited to light perception. Slit-lamp examination of the RE showed an iris granuloma with overlying exudate and associated anterior chamber inflammatory reaction. Film X-rays, contact B-scan ultrasonography, and CT scan showed no abnormalities. Anterior segment optical coherence tomography revealed an enclaved iris foreign body. The foreign body was removed after a short course of local antibio-corticosteroid therapy. This was followed 2 months later by cataract surgery with intraocular lens implantation, with subsequent improvement of visual acuity to 20/40. Conclusions A missed intraocular foreign body can lead to sight-threatening complications. Anterior segment optical coherence tomography may be useful for detecting non-clinically evident intraocular foreign body involving the anterior segment masquerading as chronic anterior uveitis.

A masquerade syndrome is an ophthalmological entity where a neoplasm mimics an inflammatory condition. Ocular melanoma (chiefly uveal) may present with symptoms suggestive of intraocular inflammation such as endogenous endophthalmitis. Ocular melanoma is most commonly found in middle-aged and older caucasian patients. One-third of all uveal melanoma cases present asymptomatically. Early diagnosis facilitates treatment before ocular melanoma reaches metastatic stage IV. Current therapy options for stage IV patients are palliative care and clinical trial participation.eed for clinicians to be aware of rare metastases at the time of diagnosis.

Competing Interests: Declaration of Competing Interest No conflicting relationship exists for any authors.

Abstract Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, which consists a subset of primary central system lymphoma (PCNSL) and the most common type of intraocular lymphoma. The involvement of eyes is estimated to be approximately 20% of PCNSL, but the brain involvement may be up to 80% of PVRL. Typically, PVRL is a high grade B-cell malignancy of the retina and needs to be assorted from choroidal low-grade B-cell lymphomas. Very often PVRL masquerades and can be erroneously diagnosed as chronic uveitis, white dot syndromes or other neoplasms. Establishing an accurate diagnosis may involve cytology/pathology, immunohistochemistry, flow cytometry, molecular pathology and cytokine profile analysis. There is inadequate information about PVRL's true incidence, ethnic/geographical variation and pathogenetic mechanisms. The therapeutic approach of PVRL involves aggressive chemotherapy and radiation therapy. Although PVRL tends to have a good response to the initial treatment, the prognosis is poor and the survival restricted due to the high relapse rates and CNS involvement. [ABSTRACT FROM AUTHOR]

Purpose: To describe clinical manifestations, diagnostic approaches, therapy, and outcomes of biopsy-proven intraocular lymphoma.Methods: Review of tertiary referral center records between 2005 and 2015.Results: A total of 51 eyes of 26 patients were included; mean age of onset was 60.42 years. Common ocular complaints included floaters (42%) and blurred vision (35%); 62% of patients had ocular and central nervous system involvement; 11% had systemic lymphoma; and 27% had only ocular involvement. Vitreous analysis was positive for malignant cells in 77% of patients on initial biopsy, and in 100% of patients on repeat biopsy. In total, 20/26 patients received systemic and topical treatment before IOL diagnosis was made; 25 patients received intravitreal methotrexate and/or rituximab; one patient received intracameral rituximab. All patients achieved remission by their final visit.Conclusions: Intraocular lymphoma often masquerades as intraocular inflammation, resulting in delayed or misdiagnosis with subsequent inappropriate management. Optimal therapy is a challenge for oncologists and ophthalmologists. [ABSTRACT FROM AUTHOR]