Dos casos de retinopatía externa oculta zonal aguda: importancia del diagnóstico multimodal / Two case reports of acute zonal occult outer retinopathy (AZOOR): importance of multimodal diagnosis

Presentamos dos casos de retinopatía externa oculta zonal aguda (AZOOR). Un varón joven de 19 años de edad y una mujer de 47 años. El varón joven presentó inicialmente clínica unilateral de escotoma centrocecal y fotopsias. La sintomatología inicial de la mujer fue disminución de visión y fotopsias bilaterales. Los estudios realizados mediante imagen multimodal con fotografías del fondo de ojo, angiografía fluoresceínica, autofluorescencia de fondo, tomografía de coherencia óptica de dominio espectral y pruebas de campo visual fueron consistentes con el diagnóstico de AZOOR. El diagnóstico diferencial de esta patología es complicado, ya que comparte datos clínicos con otras retinopatías, por lo que resulta imprescindible apoyarse en las pruebas de imagen disponibles hoy en día, prestando especial atención a aquellas donde puede verse el patrón trizonal característico, como son la autofluorescencia y la OCT (AU)

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); a 19-year-old man, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT (AU)

Two case reports of acute zonal occult outer retinopathy (AZOOR): Importance of multimodal diagnosis.

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); one a young man of 19 years, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT.

Retinitis necrosante crónica por citomegalovirus en una paciente con esclerodermia y enfermedad mixta del tejido conectivo / Chronic cytomegalovirus necrotising retinitis in a patient with scleroderma and mixed connective tissue disease

Mujer de 52 años de edad con esclerodermia, enfermedad mixta del tejido conectivo, enfermedad pulmonar intersticial y malnutrición severa a causa de la afectación esofágica de su enfermedad, en tratamiento con prednisona, micofenolato e hidroxicloroquina, que desarrolló una necrosis retiniana crónica por citomegalovirus. Inicialmente diagnosticada de agujero macular, se realizó una vitrectomía para su corrección. No fue hasta 2meses después de la intervención, como consecuencia del empeoramiento progresivo, cuando se realizó el diagnóstico y el comienzo del tratamiento con ganciclovir por vía intravenosa e intravítrea. La agudeza visual final fue de contar dedos, como consecuencia de una grave atrofia macular. En pacientes VIH negativos con inmunodeficiencia parcial por otras causas, puede desarrollarse una necrosis retiniana crónica por citomegalovirus, caracterizada por una necrosis granular lentamente progresiva acompañada de una vasculitis retiniana oclusiva (AU)

The case is presented of a 52-year-old woman with scleroderma, mixed connective tissue disease, and interstitial lung disease, who developed chronic cytomegalovirus necrotising retinitis while on treatment with prednisone, mycophenolate, and hydroxychloroquine. Initially diagnosed as macular hole, the patient underwent a pars plana vitrectomy. Two months after surgery, due to progressive worsening, the diagnosis was made and treatment started (intravenous and intravitreal ganciclovir). The patient developed severe macular atrophy with final visual acuity of counting fingers. A chronic retinal necrosis can be caused by cytomegalovirus infection in non-HIV patients with partial immune dysfunction from other causes, characterised by a slowly progressive granular retinitis with occlusive vasculitis (AU)

Chronic cytomegalovirus necrotizing retinitis in a patient with scleroderma and mixed connective tissue disease.

The case is presented of a 52-year-old woman with scleroderma, mixed connective tissue disease, and interstitial lung disease, who developed chronic cytomegalovirus necrotizing retinitis while on treatment with prednisone, mycophenolate, and hydroxychloroquine. Initially diagnosed as macular hole, the patient underwent a pars plana vitrectomy. Two months after surgery, due to progressive worsening, the diagnosis was made and treatment started (intravenous and intravitreal ganciclovir). The patient developed severe macular atrophy with final visual acuity of counting fingers. A chronic retinal necrosis can be caused by cytomegalovirus infection in non-HIV patients with partial immune dysfunction from other causes, characterized by a slowly progressive granular retinitis with occlusive vasculitis.

Presentación multifocal en 2 casos de maculopatía unilateral aguda idiopática / Multifocal presentation in 2 cases of unilateral acute idiopathic maculopathy

Presentamos 2 casos de maculopatía idiopática aguda unilateral en 2adultos jóvenes, un varón de 24 años y una mujer de 37 años. Ambos sufrieron una pérdida aguda de visión acompañada de manifestaciones clínicas compatibles con la maculopatía idiopática unilateral aguda. Como hallazgo excepcional, presentaron lesiones multifocales alrededor de una lesión central de mayor tamaño. Con el tiempo, se produjo la recuperación espontánea de la pérdida visual. La maculopatía idiopática aguda unilateral puede presentarse de formas distintas de la convencional, por lo que deberíamos considerar la posibilidad de encontrarnos ante estas formas atípicas (AU)

Two cases of multifocal unilateral acute idiopathic maculopathy are presented, one in a 24 year-old man, and another in a 37 year-old woman. Both of them presented with acute vision loss and clinical findings compatible with unilateral acute idiopathic maculopathy. As a relatively uncommon finding, they had multifocal lesions around a larger central lesion. They experienced a spontaneous improvement of their vision. Atypical presentations of unilateral acute idiopathic maculopathy like multifocal lesions are possible. Ophthalmologists should be aware of this rare form of presentation (AU)

Multifocal presentation in 2 cases of unilateral acute idiopathic maculopathy. / Presentación multifocal en 2 casos de maculopatía unilateral aguda idiopática.

Two cases of multifocal unilateral acute idiopathic maculopathy are presented, one in a 24 year-old man, and another in a 37 year-old woman. Both of them presented with acute vision loss and clinical findings compatible with unilateral acute idiopathic maculopathy. As a relatively uncommon finding, they had multifocal lesions around a larger central lesion. They experienced a spontaneous improvement of their vision. Atypical presentations of unilateral acute idiopathic maculopathy like multifocal lesions are possible. Ophthalmologists should be aware of this rare form of presentation.

Flujo venoso trilaminar: más allá del fenómeno angiofluoresceingráfico / Trilaminar venous flow: Beyond the fluorescein angiography phenomenon

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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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Análisis estructural macular bilateral tras síndrome de embolismo graso / Bilateral macular structural analysis after fat embolism síndrome

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Respuesta similar a la uveítis de recuperación inmune en un paciente con retinitis herpética como complicación de una leucemia de células peludas / Immune recovery uveitis in a patient with herpes retinitis as a complication of hairy cell leukaemia

Paciente de 51 años de edad con leucemia de células peludas tratado con pentostatina. Mientras recibía el tratamiento, desarrolló una retinitis herpética en el ojo derecho. Tras finalizar el tratamiento con pentostatina, presentó un cuadro de vitritis y edema macular quístico. No había signos de reactivación de la retinitis herpética. Tras excluir otras causas de inflamación intraocular, se estableció el diagnóstico de uveítis de recuperación inmune. El paciente fue tratado con triamcinolona intravítrea, corticoides orales, implantes de dexametasona intravítreos y, finalmente, vitrectomía. La uveítis de recuperación inmune puede aparecer en pacientes VIH negativos. La reconstitución inmune tras el tratamiento podría dar lugar a una inflamación intraocular. El manejo de esta puede ser bastante complicado, pudiendo ser necesaria la realización de una vitrectomía. La posibilidad de una uveítis de recuperación inmune en pacientes VIH negativos debería ser tenida en cuenta

A 51 year-old man with hairy cell leukaemia was treated with pentostatin. While receiving the treatment, he was diagnosed with herpes retinitis in his right eye. After the last cycle of pentostatin the patient developed a mild vitritis and cystoid macular oedema. There were no signs of herpes retinitis reactivation. After excluding other possible causes of intraocular inflammation, a diagnosis of immune recovery uveitis was made. The patient was treated with 2-monthly retro-septal injections of triamcinolone, oral corticosteroids, intravitreal dexamethasone implants and, finally, pars plana vitrectomy. An immune recovery uveitis-like response is possible in HIV negative individuals. The immune reconstitution after the treatment of hairy cell leukaemia may have led to intraocular inflammation. Management of immune recovery uveitis is challenging and difficult. Pars plana vitrectomy may be necessary. Ophthalmologists should be alert to the possibility of immune recovery uveitis in HIV negative patients

Diagnóstico de enfermedad de Eales a partir de una membrana epirretiniana / Diagnosis of Eales disease from a macular epiretinal membrane

Presentamos el caso de un varón de 38 años, remitido a nuestro servicio por tracción vitreorretiniana y membrana epirretiniana asociada a pérdida de visión de 3 meses de evolución. Tras una exploración oftalmológica que incluyó el examen de la periferia retiniana, tomografía de coherencia óptica, prueba de tuberculina, interferon gamma release assay (IGRA) y estudio sistémico se llegó al diagnóstico de enfermedad de Eales. La afectación macular en pacientes con enfermedad de Eales es un hallazgo común, ya sea en forma de edema macular o membrana epirretiniana. Por ello, es aconsejable realizar un estudio macular mediante OCT. Por otra parte, el hallazgo de una membrana epirretiniana en un paciente joven debe hacernos pensar en una posible etiología no idiopática

Macular involvement is a common finding in patients with Eales disease. The purpose of this communication is to describe the diagnosis of Eales disease from the finding of a macular epiretinal membrane in a young patient. The case is presented of a 38-year-old man referred to this medical service unit with blurred vision developed over the past 3 months, and was associated with vitreoretinal traction and a macular epiretinal membrane. After an ophthalmological examination including the retinal periphery, optical coherence tomography, tuberculin test, interferon gamma release assay (IGRA), and a systemic study, the patient was diagnosed with Eales disease. Macular oedema or epiretinal membranes due to Eales disease are relatively common. Sd-OCT is recommended in all patients with Eales disease. On the other hand, the presence of epiretinal membranes in young patients usually suggests a non-idiopathic aetiology

Immune recovery uveitis in a patient with herpes retinitis as a complication of hairy cell leukaemia. / Respuesta similar a la uveítis de recuperación inmune en un paciente con retinitis herpética como complicación de una leucemia de células peludas.

A 51 year-old man with hairy cell leukaemia was treated with pentostatin. While receiving the treatment, he was diagnosed with herpes retinitis in his right eye. After the last cycle of pentostatin the patient developed a mild vitritis and cystoid macular oedema. There were no signs of herpes retinitis reactivation. After excluding other possible causes of intraocular inflammation, a diagnosis of immune recovery uveitis was made. The patient was treated with 2-monthly retro-septal injections of triamcinolone, oral corticosteroids, intravitreal dexamethasone implants and, finally, pars plana vitrectomy. An immune recovery uveitis-like response is possible in HIV negative individuals. The immune reconstitution after the treatment of hairy cell leukaemia may have led to intraocular inflammation. Management of immune recovery uveitis is challenging and difficult. Pars plana vitrectomy may be necessary. Ophthalmologists should be alert to the possibility of immune recovery uveitis in HIV negative patients.

Estudio del ángulo iridocorneal mediante OCT Swept-Source en paciente con uveítis anterior granulomatosa / Iridocorneal angle assessment using Swept-Source OCT in a patient with anterior granulomatous uveítis

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Diagnosis of Eales disease from a macular epiretinal membrane. / Diagnóstico de enfermedad de Eales a partir de una membrana epirretiniana.

Macular involvement is a common finding in patients with Eales disease. The purpose of this communication is to describe the diagnosis of Eales disease from the finding of a macular epiretinal membrane in a young patient. The case is presented of a 38-year-old man referred to this medical service unit with blurred vision developed over the past 3 months, and was associated with vitreoretinal traction and a macular epiretinal membrane. After an ophthalmological examination including the retinal periphery, optical coherence tomography, tuberculin test, interferon gamma release assay (IGRA), and a systemic study, the patient was diagnosed with Eales disease. Macular oedema or epiretinal membranes due to Eales disease are relatively common. Sd-OCT is recommended in all patients with Eales disease. On the other hand, the presence of epiretinal membranes in young patients usually suggests a non-idiopathic aetiology.

Síndrome de despigmentación aguda bilateral de iris y transiluminación aguda bilateral de iris / Bilateral acute iris depigmentation and bilateral acute iris transillumination síndrome

Varón de 32 años que acude con un cuadro agudo bilateral caracterizado por visión borrosa, ojo rojo, fotofobia severa y dolor ocular tras un cuadro seudogripal. El paciente presentaba un cuadro con afectación bilateral caracterizado por pupilas en midriasis media, escasamente reactivas a la luz, transiluminación del iris, despigmentación difusa del estroma iridiano, dispersión de pigmento en la cámara anterior e hipertensión ocular. Tras el examen ocular se descartó un cuadro inflamatorio y un glaucoma pigmentario. El paciente presentaba características tanto de la despigmentación como de la transiluminación bilateral de iris. Ambas entidades podrían formar parte del espectro de la misma enfermedad

The case is presented of a 32 year-old male who arrived with acute bilateral symptoms with blurred vision, red eye, severe photophobia and severe ocular pain after suffering from a flu-like syndrome. The patient presented with a clinical picture of bilateral involvement characterised by pupils in mid-mydriasis, scarcely reactive to light, iris transillumination, diffuse depigmentation of the iridian stroma, pigment dispersion in the anterior chamber, and ocular hypertension. After the eye examination an inflammatory syndrome and pigmentary glaucoma were ruled out. The patient showed depigmentation characteristics as well as bilateral iris transillumination. Both conditions could form part of the spectrum of the same disease

Necrosis retiniana como manifestación de aspergilosis invasiva en un paciente tratado con ruxolitinib / Invasive aspergillosis manifesting as retinal necrosis in a patient treated with ruxolitinib

Varón de 30 años de edad con leucemia mieloblástica aguda y síndrome mielodisplásico secundario que desarrolló una enfermedad injerto contra huésped. El paciente fue tratado con ruxolitinib, un inhibidor de la Janus quinasa. A los 3 meses de haber iniciado el tratamiento se produjo una necrosis retiniana por Aspergillus, sin respuesta al tratamiento. El tratamiento con inhibidores de la Janus quinasa favorecería un aumento en la incidencia de infecciones oportunistas. El uso de estos fármacos podría dar lugar a una menor eficacia de los tratamientos empleados

A 30 year-old man with acute myeloblastic leukaemia and secondary myelodysplastic syndrome developed graft-versus-host disease. The patient was treated with ruxolitinib. After being treated for 3 months with ruxolitinib, an inhibitor of Janus kinase, he developed Aspergillus retinal necrosis resistant to common treatment. Treatment with Janus kinase inhibitors may lead to an increased incidence of opportunistic infections. Janus kinase inhibitor administration may result in poor treatment efficacy