Detección de la progresión de la retinopatía externa oculta zonal aguda (AZOOR) con angiografía por tomografía de coherencia óptica: a propósito de un caso / Detecting progression of acute zonal occult outer retinopathy (AZOOR) with optical coherence tomography angiography: A case report

Caso clínico Mujer de 48años con escotomas superotemporales persistentes y fotopsias de 2meses de evolución y zonas despigmentadas en retina de ambos ojos con patrón trizonal en imagen multimodal. La resonancia magnética cerebral, la tomografía por emisión de positrones, los anticuerpos antirretinianos y los marcadores inmunológicos, infecciosos y tumorales fueron negativos, por lo que se diagnosticó retinopatía externa oculta zonal aguda. La paciente fue tratada con adalimumab. Sin embargo, 19meses después los síntomas aumentaron y se detectó progresión en la angiografía por tomografía de coherencia óptica, así como también en la prueba de campo visual Humphrey y el electrorretinograma, por lo que se agregó micofenolato de mofetilo, mostrando mejoría y estabilización de la enfermedad durante un seguimiento de 4años. Discusión La angiografía por tomografía de coherencia óptica puede ser una herramienta potencial para monitorizar la progresión y la respuesta al tratamiento además de otras modalidades de imagen en la retinopatía externa oculta zonal aguda, y la combinación de adalimumab y micofenolato puede ser útil en la enfermedad recurrente (AU)

Clinical case A 48-year-old woman with persistent superotemporal scotomas and photopsias for 2months, and depigmented zones in the retina of both eyes with a trizonal pattern on multimodal imaging. Brain magnetic resonance imaging, positron emission tomography, antiretinal antibodies and immunological, infectious and tumor markers tests were negative, thus acute zonal occult outer retinopathy was diagnosed. Patient was treated with adalimumab. Nevertheless, 19months later symptoms increased, and progression was detected on optic coherence tomography angiography, as well as in Humphrey visual field test and electroretinogram, thus, mycophenolate mofetil was added showing improvement and stabilization of the disease in a 4-year follow-up. Discussion Optic coherence tomography angiography may be a potential tool to monitor progression and response to treatment in addition to other imaging modalities in acute zonal occult outer retinopathy, and the combination of adalimumab and mycophenolate may be useful in recurrent disease (AU)

AZOOR con edema discal unilateral: informe de un caso atípico / AZOOR with unilateral disc edema: An atypical case report

El diagnóstico de retinopatía aguda zonal oculta externa (AZOOR) se presenta como un desafío en la práctica del oftalmólogo. En esta enfermedad fueron descritos varios hallazgos atípicos, pero el edema del disco óptico es raro. Pretendemos describir un diagnóstico desafiador de AZOOR. Presentamos el caso de una mujer de 19 años con pérdida de visión del ojo derecho, sin dolor, con 2 semanas de evolución. El fondo de ojo presentaba edema con hiperemia del disco óptico y la campimetría demostraba una mancha ciega aumentada. Se ha considerado como diagnóstico neuritis óptica, sin causa infecciosa, por lo que ha realizado tratamiento con corticoide. Después de 4 meses, la agudeza visual había mejorado, pero persistían las alteraciones en la campimetría con hiperautofluorescencia alrededor del disco óptico. La tomografía óptica de coherencia demostró pérdida generalizada de las capas externas de la retina y disminución de la reflectancia de la región correspondiente. Se concluye que el edema de la papila no es un hallazgo frecuente de AZOOR, por lo que representa una enfermedad de difícil diagnóstico (AU)

Acute zonal occult outer retinopathy (AZOOR) diagnosis is challenging and frequently delayed. Atypical findings were described, nevertheless optic disc edema has not been consistently reported. In this study we pretend to describe a challenging diagnosis of AZOOR. In our case, a 19-year-old female presented painless vision loss in her right eye for 2 weeks. Fundus examination revealed optic disc hyperaemic edema and the visual field (VF) an enlarged blind spot. Non-infectious optic neuritis was assumed and intravenous corticotherapy administered. Four months later, VA had improved, but a VF defect persisted. Funduscopic examination showed mild peripapillary atrophy and autofluorescence zonal hyperautofluorescence around optic disc. Optical coherence tomography demonstrated diffuse loss of outer retinal layers and electroretinogram weakened signal at the corresponding region. In conclusion, unilateral optic disc edema, generally not associated with AZOOR typical presentation, hamper an early diagnosis and expresses this case relevance (AU)

Detecting progression of acute zonal occult outer retinopathy (AZOOR) with optical coherence tomography angiography: A case report.

CLINICAL CASE: A 48-year-old woman with persistent superotemporal scotomas and photopsias for 2 months, and depigmented zones in the retina of both eyes with a trizonal pattern on multimodal imaging. Brain magnetic resonance imaging, positron emission tomography, antiretinal antibodies, immunological, infectious and tumor markers tests were negative, thus acute zonal occult outer retinopathy was diagnosed. Patient was treated with adalimumab. Nevertheless, 19 months later symptoms increased, and progression was detected on optic coherence tomography angiography, as well as in Humphrey visual field test and electroretinogram, thus, mycophenolate mofetil was added showing improvement and stabilization of the disease in a 4-year follow-up. DISCUSSION: Optic coherence tomography angiography may be a potential tool to monitor progression and response to treatment in addition to other imaging modalities in acute zonal occult outer retinopathy, and the combination of adalimumab and mycophenolate may be useful in recurrent disease.

AZOOR with unilateral disc edema: An atypical case report.

Acute zonal occult outer retinopathy (AZOOR) diagnosis is challenging and frequently delayed. Atypical findings were described, nevertheless optic disc edema has not been consistently reported. In this study we pretend to describe a challenging diagnosis of AZOOR. In our case, a 19-year-old female presented painless vision loss in her right eye for 2 weeks. Fundus examination revealed optic disc hyperaemic edema and the visual field (VF) an enlarged blind spot. Non-infectious optic neuritis was assumed and intravenous corticotherapy administered. Four months later, VA had improved, but a VF defect persisted. Funduscopic examination showed mild peripapillary atrophy and autofluorescence zonal hyperautofluorescence around optic disc. Optical coherence tomography demonstrated diffuse loss of outer retinal layers and electroretinogram weakened signal at the corresponding region. In conclusion, unilateral optic disc edema, generally not associated with AZOOR typical presentation, hamper an early diagnosis and expresses this case relevance.

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.