Complejo anómalo vascular exudativo perifoveal (PEVAC) / Perifoveal exudative vascular anomalous complex (PEVAC)

Se presentan 3 casos de pacientes, con 66, 80 y 23años de edad, que presentaron una pérdida de visión unilateral. La tomografía de coherencia óptica (OCT) mostró edema macular junto con una lesión redondeada de pared hiperreflectiva y la angiografía con fluoresceína (AFG) de dos de ellos, dilataciones aneurismáticas perifoveales hiperfluorescentes con exudación. Ninguno de los casos mostró respuesta al tratamiento tras un año de seguimiento, diagnosticándose finalmente de complejo anómalo vascular exudativo perifoveal (PEVAC) (AU)

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyper-reflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with perifoveal exudative vascular anomalous complex (PEVAC) (AU)

Perifoveal exudative vascular anomalous complex (PEVAC).

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyperreflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with Perifoveal Exudative Vascular Anomalous Complex (PEVAC).

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.

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)

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.

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)