Neurorretinitis bilateral y nefritis lúpica membranosa: dos manifestaciones infrecuentes en el lupus juvenil / Bilateral neuroretinitis and membranous lupus nephritis: 2 infrequent manifestations in juvenile lupus

Joven de 15 años, previamente sana, se presentó con dolor abdominal, vómitos, diarrea, eritema malar, edema palpebral y en miembros inferiores, artralgias, rigidez matinal y visión borrosa bilateral. Estudios de laboratorio y por imágenes junto con la clínica permitieron realizar el diagnóstico de síndrome nefrótico secundario a lupus eritematoso sistémico. Al examen oftalmológico se constató 8/10 de visión en ambos ojos y edema de papila bilateral con estrella macular parcial, hallazgos compatibles con una neurorretinitis bilateral. La biopsia renal estableció el diagnóstico de nefritis lúpica membranosa. Se inició tratamiento inmunosupresor, con mejoría clínica gradual. Si bien el lupus eritematoso sistémico con nefritis lúpica membranosa y neurorretinitis es una asociación muy infrecuente, frente a un paciente con neurorretinitis bilateral debemos considerar el lupus eritematoso sistémico dentro de los diagnósticos diferenciales (AU)

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 (AU)

Use of Ultra-Widefield Fluorescein Angiography to Guide the Treatment to Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis-Case Report and Literature Review.

PURPOSE: To review the clinical features, diagnosis, and treatment of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) and to report a case with the use of ultra-widefield fluorescein angiography (UWFA) for confirming the precise staging of IRVAN and aid in early treatment. The patient improved after being treated with intravitreal aflibercept injection. RESULTS: A 26-year-old female complained of progressive blurred vision OD for one week. Her BCVA was 0.6 OD and 1.0 OS. Fundus examination showed vitritis, retinal hemorrhage, and vasculitis over bilateral eyes. Fluorescein angiography (FA) with a 55 degree of view revealed aneurysmal dilations of the peripapillary arteriole, peripapillary focal leakage, venous leakage, and capillary nonperfusion area. Stage 2 IRVAN was impressed OU. Oral prednisolone was administered. After four months, she experienced decreased visual acuity OS. Optical coherence tomography (OCT) revealed subretinal and intraretinal fluid with hyperreflective material. One posterior subtenon triamcinolone and one intravitreal aflibercept injection were performed OS, and macular edema subsided. A 105-degree ultra-widefield fluorescein angiography (UWFA) showed multiple peripheral background hypofluorescence areas corresponding to capillary nonperfusion. Retinal neovascularization (NV) was found OS, which had not been revealed by the previous 55-degree FA. Stage 3 IRVAN was made OS and panretinal laser photocoagulation (PRP) was performed. Oral prednisone and cyclosporine were prescribed. Her vision improved to 1.0 OU. CONCLUSION: UWFA provides visualization of peripheral retinal pathology and for precise staging. It also had direct implications in the follow-up and treatment strategy.

Epitelitis pigmentaria retiniana aguda (EPRA). A propósito de un caso / Acute retinal pigment epitheliitis (ARPE). A case report

Mujer de 35 años de edad que acudió a urgencias por pérdida de visión en ojo izquierdo. Presentaba una lesión de aspecto amarillento subfoveal que, en la tomografía de coherencia óptica (OCT), se correspondía con una lesión hiperreflectiva desde la capa nuclear externa hasta el epitelio pigmentario de la retina. La lesión se reabsorbió a las 6 semanas dejando una discontinuidad en la línea de los fotorreceptores y del epitelio pigmentario de la retina. La paciente fue diagnosticada de epitelitis pigmentaria retiniana aguda (EPRA). Debido a que la EPRA es una enfermedad autolimitada en el tiempo y de buen pronóstico, queremos recalcar la importancia del diagnóstico correcto mediante la funduscopia, OCT y angiofluoresceingrafía para evitar tratamientos innecesarios (AU)

A 35-year-old woman arrived in the emergency department due to loss of vision in the left eye. She had a subfoveal yellowish-looking lesion that, on optical coherence tomography (OCT), corresponded to a hyper-reflective lesion from the external nuclear layer to the retinal pigment epithelium. The lesion was reabsorbed at 6 weeks, leaving a discontinuity in the photoreceptor and retinal pigment epithelium lines. The patient was diagnosed with acute retinal pigmentary epitheliitis (ARPE). ARPE is a self-limiting disease with a good prognosis. Emphasis is placed on the importance of a correct diagnosis using funduscopy, OCT, and fluorescent angiography, in order to avoid unnecessary treatments (AU)

Optical coherence tomography angiography (OCTA) of retinal vasculature in patients with post fever retinitis: a qualitative and quantitative analysis.

Post fever retinitis is a heterogenous entity that is seen 2-4 weeks after a systemic febrile illness in an immunocompetent individual. It may occur following bacterial, viruses, or protozoal infection. Optical coherence angiography (OCTA) is a newer non-invasive modality that is an alternative to fundus fluorescein angiography to image the retinal microvasculature. We hereby describe the vascular changes during the acute phase of post fever retinitis on OCTA. Imaging on OCTA was done for all patients with post fever retinitis at presentation with 3 × 3 mm and 8 × 8 mm scans centred on the macula and corresponding enface optical coherence tomography (OCT) scans obtained. A qualitative and quantitative analysis was done for all images. 46 eyes of 33 patients were included in the study. Salient features noted were changes in the superficial (SCP) and deep capillary plexus (DCP) with capillary rarefaction and irregularity of larger vessels in the SCP. The DCP had more capillary rarefaction when compared to the SCP. The foveal avascular zone (FAZ) was altered with an irregular perifoveal network. Our series of post fever retinitis describes the salient vascular features on OCTA. Although the presumed aetiology was different in all our patients, they developed similar changes on OCTA. While OCTA is not useful if there is gross macular oedema, the altered FAZ can be indicative of macular ischemia.

Multimodal Imaging in Idiopathic Neuroretinitis with Localized Choroidal Insufficiency: A Case Report.

Neuroretinitis is a rare clinical entity, characterized by optic nerve edema and star-shape hard exudate around fovea. The clinical features include acute unilateral visual loss, dyschromatopsia, relative afferent pupillary defect and visual field abnormalities. Increased vascular permeability of the optic disc is the main pathophysiology. As it is a not fully known clinical entity, diagnosis is challenging. In this case, we use multimodal imaging to reveal pathophysiology and anatomical change of early mild neuroretinitis. Case presentation: A 28-year-old healthy woman presented to the clinic with mild blurred vision in her left eye. After complete ophthalmic examination, outer retinal thickening of the temporal peripapillary area and optic disc edema were observed. Two days after diagnosis, the retinal edema and visual symptoms were aggravated. A hard exudate, maybe a part of macular star, was observed. Multimodal imaging including optical coherence tomography (OCT), swept-source OCT angiography (SS-OCTA), fluorescein angiography, and indocyanine green angiography visualized choroidal thinning and insufficient circulation beneath the outer retinal edema. Following steroid pulse therapy, the retinal edema and blurred vision were completely resolved. Conclusions: Multimodal imaging suggested that unilateral optic disc edema and early macular star help the diagnosis of neuroretinitis. In SS-OCTA, we found focal choroidal insufficiency. The focal insufficient choroidal circulation might be a contribution factor for idiopathic neuroretinitis. Multimodal imaging including SS-OCTA may be a valuable tool for detecting and monitoring disease progression.

Multimodal imaging-guided diagnosis, management and follow-up of a case of diffuse unilateral subacute neuroretinitis.

A 45-year-old woman presented with diminished vision in the left eye. Visual acuity was 6/9 and fundus showed a very large live nematode near the macula along with inflammatory outer retinal lesions in the periphery. We diagnosed the case as diffuse unilateral subacute neuroretinitis and treated with immediate focal photocoagulation of the worm along with oral antihelminthic drugs and corticosteroids. The report highlights the importance of prompt laser and steroids in achieving good structural as well as the functional outcome, and the added significance of advanced imaging techniques in prognosticating such patients.

Macroaneurisma arterial de disco óptico como entidad simuladora de neurorretinitis aguda / Arterial macroaneurysm of the optic disc as an entity simulating acute neuroretinitis

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Neurorretinitis estrellada idiopática de Leber: conflictos diagnósticos y terapéuticos / Leber's idiopathic stellate neuroretinitis: diagnostic and therapeutic conflicts

Presentamos 3 pacientes con clínica de disminución de agudeza visual, papilitis y desarrollo de estrella macular al cabo de unas semanas. En todos ellos, las pruebas complementarias arrojaron resultados irrelevantes. Dado este contexto, fueron diagnosticados de neurorretinitis estrellada idiopática de Leber (NEIL) y se pautó tratamiento con corticoides, así como antibióticos en 2de ellos. Evolucionaron favorablemente, aunque con signos de atrofia papilar en los ojos afectos. El diagnóstico de la neurorretinitis estrellada idiopática de Leber puede resultar complejo, dado el amplio espectro de enfermedades que descartar. A esto se suma un período de tiempo en el que puede no haber aparecido la estrella macular, motivo que no debe hacer excluir su diagnóstico. El uso de antibióticos o corticoides como medida terapéutica es controvertido dado su buen curso clínico, aunque parece razonable en casos severos. Otro punto controvertido es el uso rutinario de pruebas complementarias, principalmente aquellas que pueden resultar agresivas o caras, que deben ser utilizadas de forma racional

Three cases of patients with decreased visual acuity and papillitis at onset with subsequent macular star development after a few weeks are presented. Complementary tests were unremarkable in all included patients. Based on this clinical context, they were diagnosed with Leber's idiopathic stellate neuroretinitis, were treated with corticosteroids, as well as with antibiotics in 2 cases. All patients showed favourable outcomes, although signs of papillary atrophy were observed in the affected eyes. Leber's idiopathic stellate neuroretinitis diagnosis can be challenging due to the wide spectrum of conditions that have to be ruled out. In addition, macular star may appear later on, which should not exclude its diagnosis. Use of antibiotics and/or corticosteroids is controversial considering its benign nature, but should be considered in selected severe cases. Moreover, the routine use of complementary tests should be carefully evaluated, mainly those that can be aggressive and/or expensive, which should be rationally used

Leber's idiopathic stellate neuroretinitis: diagnostic and therapeutic conflicts. / Neurorretinitis estrellada idiopática de Leber: conflictos diagnósticos y terapéuticos.

Three cases of patients with decreased visual acuity and papillitis at onset with subsequent macular star development after a few weeks are presented. Complementary tests were unremarkable in all included patients. Based on this clinical context, they were diagnosed with Leber's idiopathic stellate neuroretinitis, were treated with corticosteroids, as well as with antibiotics in 2cases. All patients showed favourable outcomes, although signs of papillary atrophy were observed in the affected eyes. Leber's idiopathic stellate neuroretinitis diagnosis can be challenging due to the wide spectrum of conditions that have to be ruled out. In addition, macular star may appear later on, which should not exclude its diagnosis. Use of antibiotics and/or corticosteroids is controversial considering its benign nature, but should be considered in selected severe cases. Moreover, the routine use of complementary tests should be carefully evaluated, mainly those that can be aggressive and/or expensive, which should be rationally used.

Bartonella presenting as branch retinal artery occlusion in a child.

Branch retinal artery occlusion (BRAO) is rare in children. Bartonella is a known cause of branch retinal artery occlusion in adults, but it is typically not considered in the differential diagnosis for pediatric BRAO. We present the case of a 12-year old boy with a BRAO caused by a Bartonella henselae infection. This is the youngest such case reported in the literature. Although rare, Bartonella infection may be an important and underrecognized cause of pediatric BRAO.

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.