Bilateral neuroretinitis and membranous lupus nephritis: 2 infrequent manifestations in juvenile lupus.

Fifteen-year-old female patient, previously healthy, referred to our center for presenting abdominal pain, vomiting, diarrhea, malar erythema, palpebral and lower limb edema, arthralgia, morning stiffness and bilateral blurred vision. Laboratory and imaging studies together with the clinic allowed the diagnosis of nephrotic syndrome secondary to systemic lupus erythematosus. Ophthalmology examination revealed a visual acuity of 8/10 in both eyes and bilateral disc edema with partial macular star, findings compatible with bilateral neuroretinitis. Renal biopsy established the diagnosis of membranous lupus nephritis. Immunosuppressive treatment was started, obtaining gradual clinical improvement. Although systemic lupus erythematosus with membranous lupus nephritis and neuroretinitis is a very infrequent association, when faced with a patient with bilateral neuroretinitis, we must consider systemic lupus erythematosus within the differential diagnoses.

Outcomes of cataract surgery in patients with Human Immunodeficiency Virus infection in a developing country.

PURPOSE: To report the outcomes of cataract surgery in patients with Human Immunodeficiency Virus (HIV) infection. Setting Tertiary care ophthalmic hospital DESIGN: Retrospective study METHODS: This study included all eyes of patients with known HIV infection undergoing cataract surgery with a minimum follow-up of 6 months between January 2017 and December 2020. Patients who underwent combined surgeries and pediatric patients were excluded from analysis. Data were retrieved from electronic medical records and we documented demographics, history, detailed anterior and posterior segment examination, pre-operative grade and type of cataract, type of surgery done, its complication and post-operative course. All these parameters were recorded at the baseline visit and at 1 month and 6 months postoperatively. RESULTS: One hundred and twenty nine eyes of 107 HIV infected patients that underwent cataract surgery were evaluated. Mature cataract was seen in 31% of the eyes. Features of HIV related uveitis/retinitis were seen in 21 (16.2%) eyes. Phacoemulsification was performed in 44 (34.1%) eyes while manual small incision cataract surgery (MSICS) was done in 85 (65.9%) eyes. Intra-operative complications were encountered in 4 (3.1%) eyes. At the final follow-up, there was a significant improvement in median corrected distance visual acuity (CDVA) from LogMAR 1.08 (5/60) at baseline to LogMAR 0 (6/6) at 6 months follow-up. CONCLUSION: Patients with HIV infection usually present early and with advanced cataracts. Visual outcomes after cataract surgery are generally good but affected by presence of prior HIV related uveitis or retinitis.

Bilateral Optic Nerve Head Drusen Complicated by Choroidal Neovascularization Misdiagnosed as Papilledema and Neuroretinitis.

We report a case of bilateral optic nerve head drusen complicated by choroidal neovascularization (CNV) in the left eye at presentation. The presence of optic disc and macular edema in addition to exudation led to the misdiagnosis of neuroretinitis at an outside medical center. Swept-source optical coherence tomography (SS-OCT) and SSOCT angiography were critical in establishing the diagnosis and follow-up in a noninvasive manner. Secondary CNV associated with optic nerve head drusen responded well to intravitreal injections of anti-vascular endothelial growth factor in the left eye. Asymptomatic nonexudative CNV that developed in the right eye during follow-up regressed spontaneously without treatment. [Ophthalmic Surg Lasers Imaging Retina 2022;53:518-521.].

Enfermedad por arañazo de gato atípica: compromiso ocular y meníngeo por Bartonella henselae / Atypical cat scratch disease: Bartonella henselae ocular and meningeal involvement

Bartonella henselae es el agente etiológico de la enfermedad por arañazo de gato. Típicamente, se presenta como una linfadenopatía regional autolimitada y, con menor frecuencia, con compromiso sistémico y manifestaciones extraganglionares: hígado, bazo, hueso y ojo, entre otros. Se presenta un caso de enfermedad por arañazo de gato atípica en un paciente pediátrico inmunocompetente, en la que se evidenció compromiso meníngeo y ocular, este último como neurorretinitis. Se destaca la importancia de la búsqueda activa de complicaciones oculares en pacientes con compromiso sistémico por Bartonella henselae, que implica un cambio en el tratamiento y pronóstico de la enfermedad

Bartonella henselae is the etiologic agent of cat scratch disease. It typically presents as a self-limited regional lymphadenopathy and less frequently with systemic involvement and extranodal manifestations: liver, spleen, bone, eye, among others. A case of atypical cat scratch disease is presented in an immunocompetent pediatric patient, in which meningeal and ocular involvement was evidenced, the latter manifested as neuroretinitis. The importance of the active search for ocular complications in patients with systemic involvement by Bartonella henselae is highlighted, implying a change in the treatment and prognosis of the disease

Endocarditis por bartonella asociada a glomerulonefritis y neurorretinitis / Bartonella endocarditis associated with glomerulonephritis and neuroretinitis

La endocarditis bacteriana con hemocultivo negativo constituye un dilema diagnóstico. Tanto Bartonella como Coxiella pueden causarla, con presentaciones clínicas similares que pueden simular una vasculitis sistémica no infecciosa. Sin embargo, difieren en el tipo y la duración del tratamiento, por lo que es fundamental identificar el agente etiológico. Presentamos un caso de endocarditis por Bartonella henselae asociada a glomerulonefritis y neurorretinitis, con hemocultivo negativo, anticuerpos anticitoplasma de neutrófilos y antiproteinasa 3 positivos, y serología positiva para Bartonella con reacción cruzada para Coxiella burnetti. El diagnóstico etiológico fue confirmado a posteriori mediante amplificación y secuenciación parcial del gen ribC a partir de tejido de la válvula cardíaca. El paciente recibió tratamiento antibiótico e inmunosupresor seguido de recambio valvular aórtico y presentó evolución favorable.

Blood-culture negative endocarditis is a diagnostic challenge. Both Bartonella and Coxiella can cause it with similar clinical presentations mimicking a systemic vasculitis. The identification of the etiologic agent is essential because they differ in treatment type and duration. We present a case of blood-culture negative endocarditis caused by Bartonella henselae, associated with glomerulonephritis and neuroretinitis, with negative blood culture, positive anti-neutrophil cytoplasmic and antiproteinase 3 antibodies. The serology was positive for Bartonella with cross-reactivity to Coxiella burnetti. The etiological diagnosis was achieved by polymerase chain reaction amplification and sequencing of a ribC gene fragment. The patient received antibiotic and immunosuppressive treatment followed by replacement of the aortic valve with favorable medium-term evolution.

Presumed ocular histoplasmosis syndrome in a commercially insured population, United States.

PURPOSE: To describe epidemiologic features of patients with presumed ocular histoplasmosis syndrome (POHS) in the United States using insurance claims data and compare POHS patients with and without choroidal neovascularization (CNV). DESIGN: Retrospective cohort study. METHODS: Patients with International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for histoplasmosis retinitis on an outpatient claim in the 2014 IBM® MarketScan® Commercial Database and the Medicare Supplemental Database who were enrolled for at least 2 years after the POHS code. MAIN OUTCOME MEASURES: Data related to testing, treatment, and direct medical costs. RESULTS: Among >50 million total MarketScan enrollees, 6,678 (13 per 100,000) had a POHS diagnosis code. Of those, 2,718 were enrolled for 2 years; 698 (25%) of whom had a CNV code. Eleven of the 13 states with the highest POHS rates bordered the Mississippi and Ohio rivers. CNV patients had significantly more eye care provider visits (mean 8.8 vs. 3.2, p<0.0001), more ophthalmic imaging tests, higher rates of treatment with anti-vascular endothelial growth factor injections (45% vs. 4%, p<0.0001), and incurred higher mean total yearly costs ($1,251.83 vs. $251.36, p<0.0001) than POHS patients without CNV. CONCLUSIONS: Although the relationship between Histoplasma and POHS remains controversial, geographic patterns of POHS patient residence were consistent with the traditionally reported range of the fungus. CNV in the context of POHS was associated with additional healthcare use and costs. Further research to understand POHS etiology, risk factors, prevalence, and complications is needed, along with early diagnosis and treatment strategies.

Is Retinal Metabolic Dysfunction at the Center of the Pathogenesis of Age-related Macular Degeneration?

The retinal pigment epithelium (RPE) forms the outer blood⁻retina barrier and facilitates the transepithelial transport of glucose into the outer retina via GLUT1. Glucose is metabolized in photoreceptors via the tricarboxylic acid cycle (TCA) and oxidative phosphorylation (OXPHOS) but also by aerobic glycolysis to generate glycerol for the synthesis of phospholipids for the renewal of their outer segments. Aerobic glycolysis in the photoreceptors also leads to a high rate of production of lactate which is transported out of the subretinal space to the choroidal circulation by the RPE. Lactate taken up by the RPE is converted to pyruvate and metabolized via OXPHOS. Excess lactate in the RPE is transported across the basolateral membrane to the choroid. The uptake of glucose by cone photoreceptor cells is enhanced by rod-derived cone viability factor (RdCVF) secreted by rods and by insulin signaling. Together, the three cells act as symbiotes: the RPE supplies the glucose from the choroidal circulation to the photoreceptors, the rods help the cones, and both produce lactate to feed the RPE. In age-related macular degeneration this delicate ménage à trois is disturbed by the chronic infiltration of inflammatory macrophages. These immune cells also rely on aerobic glycolysis and compete for glucose and produce lactate. We here review the glucose metabolism in the homeostasis of the outer retina and in macrophages and hypothesize what happens when the metabolism of photoreceptors and the RPE is disturbed by chronic inflammation.

Retinal inflammation diagnosed as an idiopathic macular hole with multiple recurrences and spontaneous closures: A case report.

RATIONALE: An idiopathic macular hole that causes substantial reduction in central visual acuity is believed to involve no obvious underlying diseases; thus, it is suspected to form due to the presence of idiopathic tractional forces at the vitreoretinal interface. Importantly, it is effectively treated with pars plana vitrectomy (PPV), which removes the mechanical forces. However, while it is exceedingly rare, a macular hole can develop in eyes after PPV; fresh or postoperative macular holes can close spontaneously without surgical removal of traction. Thus, another mechanism might be involved, although it remains obscure. PATIENTS CONCERNS: A 67-year-old woman experienced 4 episodes of distorted and/or blurred vision. DIAGNOSIS: She was diagnosed with recurrent macular hole formation. INTERVENTIONS: For each episode, she either underwent surgery or was placed under observation. OUTCOMES: The macular hole was twice closed with PPV and twice without. The 2nd PPV procedure, which was performed at the time of 2nd recurrence, confirmed the absence of the epiretinal membrane and internal limiting membrane that cause tractional forces at the vitreoretinal interface in the macular area. At the time of the 3rd recurrence, fluorescein angiographies (FAs) revealed the presence of mild and diffuse inflammation throughout the peripheral retina, although there were no other findings indicative of ocular inflammation during the general eye examination conducted for every episode of macular hole formation. After the initiation of topical steroid treatment, inflammation (as recorded on FA) was reduced, and the macular hole subsequently closed. Development and resolution of perifoveal cystoid change and retinal protrusion were observed in every episode in optical coherence tomography (OCT) images. A bridging element in an OCT image was observed during the 4th closure of the macular hole. LESSONS: Dynamic changes in FA and OCT images unraveled the pathogenesis of a macular hole that was originally diagnosed as idiopathic; mild inflammation was involved. The FA is typically not used for the diagnosis and management of macular hole formation; however, its use in this case helped determine a new mechanism in an otherwise idiopathic disease.

Manifestaciones oftalmológicas en pacientes con virus de inmunodeficiencia humana. Anomalía de peters: reporte de caso / Ophthalmological manifestations in patients with human inmmunodeficiencyvirus. Peters anomaly: case report

El virus de inmunodeficiencia humana (VIH)/ síndrome de inmunodeficiencia adquirida (SIDA), afecta a millones de personas alre-dedor del mundo. En Latinoamérica y el Caribe, casi 1.9 millones de personas viven con VIH/SIDA. Se caracteriza por ser una enfermedad multisistémica en donde las enfermedades oftalmológicas se presentan en un 70% a 80% de los pacientes infecta-dos. En estos pacientes las infecciones oportunistas son la principal causa de enfer-medad ocular sin embargo, es también causa de anomalías visuales, como la ano-malía de Peter que solo se presenta 1 en un millón de personas y es la causa de más del 40% de las opacidades corneales congéni-tas. Se presenta caso de femenina de 21 años de edad, procedente de San Pedro Sula, con antecedente de virus de inmuno-deficiencia adquirida en estadio B2, por transmisión vertical, en tratamiento con anti-rretrovirales, sin antecedentes maternos infecciosos sumados al VIH. Con historia de visión borrosa, lejana y cercana en ambos ojos con mayor severidad en ojo derecho. Además, refiere cefalea, frontal, pulsátil, de predominio vespertino que se irradia hacia parietal y occipital. Al exámen físico se encuentra agudeza visual disminuida, presión intraocular en límite superior, leuco-mas corneales y gonioscopía alterada en ambos ojos. Actualmente ambos ojos esta-bles y agudeza visual mejora con gradua-ción lo que es indicativo de un pronóstico favorable...(AU)

Bilateral arterial occlusions masking retinitis in a HIV-positive male.

We report an interesting case of 36-year-old HIV-positive male with uveitis, cilioretinal artery occlusion in OD, and superotemporal branch retinal artery occlusion in OS. Hypercoagulability, cardiovascular, and rheumatologic workups were unremarkable. Aqueous taps were negative for toxoplasma, viruses, and MTb by multiplex polymerase chain reaction. Patches of retinitis were seen on clearing of retinal edema. Serology was positive for toxoplasma and rickettsia. Management included doxycycline, azithromycin, bactrim DS, and oral steroids. Vision improvement to 6/60 and 6/24 in OD and OS refer to the right eye and left eye, respectively, were noted at 4-month follow-up. Infections should be considered in arterial occlusions associated with inflammation in HIV-positive individuals.

Recurrent idiopathic neuroretinitis as a spectrum of atypical optic neuritis: a case report and literature review / Neurorretinite recorrente idiopática como espectro da neurite óptica atípica: relato de caso e revisão da literatura

ABSTRACT Recurrent idiopathic neuroretinitis is an inflammatory optic neuropathy characterized by optic nerve edema and macular star, associated with painless and recurrent episodes of visual loss, poor visual prognosis, and visual field defects related to nerve fiber layer injury. The disorder is sometimes mistaken for atypical optic neuritis. However, early diagnosis is important for visual recovery. Long-term immunosuppression has been shown to reduce the rate of recurrence and protect against severe and irreversible vision loss.

RESUMO Neurorretinite recorrente idiopática é uma neuropatia óptica inflamatória caracterizada por edema do nervo óptico e estrela macular associada a episódios recorrentes de perda visual indolor, baixo prognóstico visual e desfeitos de campo visual relacionados a injúria da camada de fibras nervosas. Essa condição pode ser confundida com neurite óptica atípica e seu correto diagnóstico é importante para o prognóstico visual, uma vez que a imunossupressão continua previne episódios recorrentes que podem levar a perda visual severa e irreversível.

OCTA Imaging of Choroidal Neovascular Membrane Secondary to Toxoplasma Retinochoroiditis.

Two patients (a 37-year-old man and a 28-year-old woman) who had choroidal neovascular membrane (CNVM) secondary to inactive toxoplasma retinochoroiditis scarring were evaluated. Multimodal imaging including fluorescein angiography, optical coherence tomography (OCT), and OCT angiography (OCTA) was used. CNVM secondary to inactive toxoplasma retinochoroiditis scarring was detected. Representative images of CNVM were demonstrated in the outer retinal layer and choriocapillary layer on OCTA. OCTA, a relatively new technique, is useful in the diagnosis of the CNVMs secondary to retinochoroiditis. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:509-511.].

Full-thickness macular hole: a rare complication of Borrelia burgdorferi neuroretinitis.

Borrelia burgdorferi is a known infective cause of neuroretinitis. We present a case of B burgdorferi neuroretinitis complicated by macular hole in a 22-year-old man. The neuroretinitis was managed with early high-dose intravenous corticosteroid and oral antibiotic. The macular hole was managed with macular hole surgery after intraocular inflammation had resolved.

In vivo multi-modal imaging of experimental autoimmune uveoretinitis in transgenic reporter mice reveals the dynamic nature of inflammatory changes during disease progression.

BACKGROUND: Experimental autoimmune uveoretinitis (EAU) is a widely used experimental animal model of human endogenous posterior uveoretinitis. In the present study, we performed in vivo imaging of the retina in transgenic reporter mice to investigate dynamic changes in exogenous inflammatory cells and endogenous immune cells during the disease process. METHODS: Transgenic mice (C57Bl/6 J Cx 3 cr1 (GFP/+) , C57Bl/6 N CD11c-eYFP, and C57Bl/6 J LysM-eGFP) were used to visualize the dynamic changes of myeloid-derived cells, putative dendritic cells and neutrophils during EAU. Transgenic mice were monitored with multi-modal fundus imaging camera over five time points following disease induction with the retinal auto-antigen, interphotoreceptor retinoid binding protein (IRBP1-20). Disease severity was quantified with both clinical and histopathological grading. RESULTS: In the normal C57Bl/6 J Cx 3 cr1 (GFP/+) mouse Cx3cr1-expressing microglia were evenly distributed in the retina. In C57Bl/6 N CD11c-eYFP mice clusters of CD11c-expressing cells were noted in the retina and in C57Bl/6 J LysM-eGFP mice very low numbers of LysM-expressing neutrophils were observed in the fundus. Following immunization with IRBP1-20, fundus examination revealed accumulations of Cx3cr1-GFP(+) myeloid cells, CD11c-eYFP(+) cells and LysM-eGFP(+) myelomonocytic cells around the optic nerve head and along retinal vessels as early as day 14 post-immunization. CD11c-eYFP(+) cells appear to resolve marginally earlier (day 21 post-immunization) than Cx3cr1-GFP(+) and LysM-eGFP(+) cells. The clinical grading of EAU in transgenic mice correlated closely with histopathological grading. CONCLUSIONS: These results illustrate that in vivo fundus imaging of transgenic reporter mice allows direct visualization of various exogenously and endogenously derived leukocyte types during EAU progression. This approach acts as a valuable adjunct to other methods of studying the clinical course of EAU.

Identification of a novel polyomavirus in a pancreatic transplant recipient with retinal blindness and vasculitic myopathy.

BACKGROUND: A 33 year-old pancreatic transplant recipient developed weakness, retinal blindness, and necrotic plaques on her face, scalp, and hands. METHODS: A muscle biopsy was analyzed by light and electron microscopy and high-throughput nucleic acid sequencing. RESULTS: The biopsy revealed microthrombosis and viral particles in swollen endothelial cell nuclei. High-throughput sequencing of nucleic acid revealed a novel polyomavirus. In situ hybridization confirmed the presence of the polyomavirus in endothelial cells at sites of myositis and cutaneous necrosis. CONCLUSIONS: New Jersey polyomavirus (NJPyV-2013) is a novel polyomavirus that may have tropism for vascular endothelial cells.

Intraocular cysts of toxoplasma gondii in patients with necrotizing retinitis following periocular/intraocular triamcinolone injection.

PURPOSE: To report the detection of Toxoplasma gondii cysts in intraocular aspirates of patients with necrotizing retinitis following periocular/intraocular corticosteroid injection. DESIGN: Case report. METHODS: Two patients (2 eyes) with widespread necrotizing retinitis in a steroid-exposed eye posed a diagnostic challenge and underwent pars plana vitrectomy (PPV). Intraocular samples (vitreous fluid, retinal tissue, and subretinal aspirate in case 1, and vitreous fluid in case 2) were subjected to cytological examination. RESULTS: The subretinal aspirate (case 1) revealed encysted bradyzoites of Toxoplasma gondii. Vitreous fluid (case 2) tested positive for anti-toxoplasma antibodies and the smear showed encysted forms of Toxoplasma gondii on cytology. CONCLUSION. Toxoplasma gondii cysts were detected in eyes with necrotizing retinitis that developed secondary to injudicious use of corticosteroids.

Cytomegalovirus ventriculoencephalitis with compartmentalization of antiviral-resistant cytomegalovirus in a T cell-depleted haploidentical peripheral blood stem cell transplant recipient.

We describe a case of cytomegalovirus (CMV) ventriculoencephalitis and retinitis with compartmentalization of antiviral drug-resistant CMV across the blood-brain barrier after T cell-depleted haploidentical peripheral blood stem cell transplantation.

Visual acuity recovery in a case of idiopathic retinal vasculitis aneurysms and neuroretinitis.

PURPOSE: To describe the visual recovery after intravitreal injections of the antivascular endothelial growth factor, bevacizumab, in a case of vaso obliteration from idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN). The name IRVAN was given to the condition to highlight the key findings present in the disease. IRVAN is a severe, sight threatening condition that can lead to peripheral capillary non-perfusion and vision loss from the ischemic sequelae of vascular occlusion. Panretinal photocoagulation (PRP) is the current standard of care for IRVAN but visual outcome is poor if PRP is initiated after neovascularization develops. Intravitreal bevacizumab has success at treating neovascularization from other ischemic retinopathies and inflammatory retinal conditions that have similar characteristics to IRVAN. CASE REPORT: This case report describes a patient with decreased vision in the OS. The patient presented with best-corrected visual acuity of 20/20 in the OD and count fingers at 4 ft in the OS. Evaluation revealed findings consistent with an advanced stage of IRVAN. Anterior and posterior neovascularization had developed from extensive capillary non-perfusion in both retinas. A dense vitreous hemorrhage blocked vision OS. Bilateral intravitreal injections of bevacizumab and extensive PRP were given in the area of retinal ischemia for treatment. After 4 months, the patient's vision had improved from count fingers in the OS to 20/40. CONCLUSIONS: IRVAN has favorable outcomes when treated with a combination of PRP and intravitreal injections of antivascular endothelial growth factor. This case demonstrates the effectiveness of this combination treatment in a case of IRVAN with both posterior and anterior neovascularization.