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Ocular involvement is not uncommon in patients with COVID-19. However, the incidence of COVID-19 ophthalmopathy in COVID-19 patients is still not clear. In this prospective case series study, we recruited 2445 consecutive cases presenting at Neuro-ophthalmology clinic of our Eye Center during the last resurgence of SARS-CoV-2 infection from 8 December 2022 to 15 March 2023 in China, 149 cases were diagnosed as COVID-19 ophthalmopathy, 87 cases were female, with a mean age of 43.2 years, and the mean follow-up time was 15.4 weeks. One hundred and twenty of 149 cases suffered from systemic symptoms mostly manifesting as fever, cough and muscle pain prior to or soon after ocular involvement. The most common COVID-19 ophthalmopathy was optic neuritis (51/149), followed by acute zonal occult outer retinopathy complex disease (31/149), uveitis (17/149), ocular mobility disorder-related (third, fourth, or sixth) cranial nerve neuritis (15/149), anterior ischaemic optic neuropathy (9/149), retinal artery occlusion (8/149), retinal microangiopathy including retinal haemorrhage and cotton wool spot (8/149), viral conjunctivitis (7/149), retinal vein occlusion (3/149), viral keratitis (2/149), ptosis (2/149), and other rare ocular diseases. Except 5 cases with central retinal artery occlusion, other 144 COVID-19 ophthalmopathy cases showed good response to steroid therapy. Our study revealed an incidence of 6.09% for COVID-19 ophthalmopathy in outpatients at our Neuro-ophthalmology clinic during last resurgence of COVID-19 in China, and demonstrated that SARS-CoV-2 infection could induce an initial onset or a relapse of ophthalmic diseases, and that ocular involvement might manifest as the initial or even the only presentation of COVID-19.

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INTRODUCTION: Bitemporal hemianopia is usually caused by chiasmal pathology. Rarely, chorioretinal lesions may develop symmetrically in both eyes and mimic chiasmopathy. METHODS: This case series included three patients who presented to a tertiary neuro-ophthalmology centre with bitemporal hemianopic defects between 2021 and 2023 and were subsequently diagnosed with bilateral chorioretinopathy. All patients received comprehensive examinations from a fellowship-trained neuro-ophthalmologist and uveitis specialist to rule out other causes of visual dysfunction. RESULTS: Three males aged 64, 62, and 72 years were included. All patients showed bitemporal hemianopic defects crossing the vertical midline on automated perimetry and binasal thinning of the macular ganglion cell complex on spectral-domain optical coherence tomography (OCT). Fundus autofluorescence (FAF) showed classical features of acute zonal occult outer retinopathy (AZOOR) in two patients and central serous chorioretinopathy (CSCR) in another. AZOOR diagnosis was preceded by neuroimaging in both cases, whereas the patient with CSCR had longstanding, electroretinography-confirmed lesions and did not require neuroimaging. Fundus appearance and visual field defects remained stable in all patients across 3-6 months of follow-up. CONCLUSIONS: Bilateral chorioretinopathy should be considered in the differential diagnosis of bitemporal hemianopia in specific cases, including when visual field defects cross the vertical midline and when neuroimaging fails to reveal chiasmal pathology. FAF and macular OCT have high diagnostic yield as initial investigations.

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This review covers the utility of electrophysiological studies relevant to inflammatory diseases of the retina in conditions such as acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, Adamantiades-Behçet disease, autoimmune retinopathy and neuro-retinopathy, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and Vogt-Koyanagi-Harada disease. Electrophysiological studies can help with the diagnosis, prognostication, evaluation of treatment effects, and follow-up for these conditions.

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PURPOSE: To report the longitudinal outcomes for AZOOR patients including treatment response, imaging evolution, and overlap with multiple evanescent white dot syndrome (MEWDS). METHODS: Visual acuity (VA) and visual field (VF) outcomes of occult and overt AZOOR patients were retrospectively compared between the first and final visits as well as between the two AZOOR subtypes. For treated patients, rates of VA change and fundus lesion area were compared before and after treatment. Analyses were performed using STATA 17. RESULTS: Seventeen eyes from 11 occult AZOOR patients and 45 eyes from 29 overt AZOOR patients were included. In a composite VA/VF primary outcome, clinical improvement was noted in five occult AZOOR and three overt AZOOR patients. The decline of logarithm of the Minimal Angle of Resolution (logMAR) VA was minimal in both groups: 0.00016 units/month in occult AZOOR patients and 0.009 units/month in overt AZOOR patients (p = 0.94). Occult AZOOR patients were more likely to have improved or stable VF than overt AZOOR patients (p = 0.04). One occult AZOOR and two overt AZOOR patients developed MEWDS at subsequent visits. Treatment with steroids or immunomodulatory therapy (IMT) was initiated in one occult AZOOR patient and nine overt AZOOR patients. Treated patients had overall VA stability. Fundus lesion area in treated patients changed by a mean of 0.2831 mm2/month, with 40% of patients showing decreased lesion area. CONCLUSION: AZOOR patients generally maintained their VA. Overt AZOOR patients were more likely to receive steroids or IMT; treatment was associated with stabilization of VA.

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Acute zonal occult outer retinopathy (AZOOR) manifests as the rapid loss of one or multiple large zones of the outer retinal layers, often with a distinct sectoral distribution. Subtle fundus changes, such as pigmentary alterations around the optic nerve, are typically present in the early stages. Disease progression is characterized by the appearance of well-defined atrophic zones involving the outer retina, retinal pigment epithelium, and choroid. AZOOR lesions typically begin in the peripapillary region and then spread centrifugally toward the peripheral fundus. In this case report, we present the clinical and multimodal imaging characteristics of a 63-year-old woman with a symmetrical, peripheral-onset AZOOR variant with a very slow centrifugal progression. Most notably, the posterior pole was unaffected bilaterally.

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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)

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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)

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A 23-year-old woman presented with a 1-month history of visual abnormalities in her right eye. A visual field test revealed temporal abnormalities in the right eye. Optical coherence tomography revealed an indistinct ellipsoid zone (EZ) on the B-scan image and hyporeflective areas in the EZ layer on the en face image in the right eye. We diagnosed the patient with acute zonal occult outer retinopathy. Visual field tests and B-scan images improved to almost normal at 6 months, but hyporeflective areas remained on the en face images. Thus, en face images may be more sensitive at detecting abnormalities in the outer retina than other modalities.

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White spot syndromes (WSS) pose challenges in the field of ophthalmology, particularly in terms of accurate diagnosis and effective management. However, recent advancements in multimodal imaging (MMI) have significantly contributed to our understanding of WSS, allowing for improved characterization of these inflammatory chorioretinopathies. By employing various imaging modalities, including fundus fluorescein angiography, indocyanine green angiography, fundus autofluorescence, optical coherence tomography (OCT), ultra-widefield imaging, and OCT angiography, researchers and clinicians have gained valuable insights into the underlying pathophysiological changes and clinical progression of WSS. Furthermore, MMI has unveiled novel and atypical variants within the spectrum of WSS, expanding our knowledge in this field. Notably, the identification of secondary forms of WSS occurring concurrently with unrelated chorioretinal disorders has suggested a potential autoimmune mechanism underlying these conditions. The introduction of MMI has also facilitated a more comprehensive evaluation of previously ill-defined entities, such as acute zonal occult outer retinopathy, leading to improved diagnostic criteria and enhanced recognition of distinct features. This review paper provides a comprehensive overview of the latest advances and interpretations in WSS. By integrating MMI into the diagnosis and management of these conditions, this review aims to enhance patient outcomes and provide valuable insights into the complexities surrounding WSS.

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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.

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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.

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BACKGROUND: Acute annular outer retinopathy (AAOR) is an uncommon disease. To date, there are few documented cases in the literature. Our case report is the first to describe a case of acute annular outer retinopathy in a patient with invasive ductal breast carcinoma. CASE PRESENTATION: The patient presented with photopsias and visual loss approximately 3 weeks prior to a diagnosis of invasive ductal breast carcinoma. We have documented the outer annular white ring seen in the acute phase of this disease and correlate it anatomically with Spectral-domain optical coherence tomography (SD-OCT) imaging. We identified RPE atrophy with nodular hyperreflectivity and loss of ellipsoid layer within the white annular ring with corresponding visual field loss. Fundus autofluorescence correlated with structural alterations seen on SD-OCT and showed both presumed active hyperautofluorescent zones with patchy hypoautofluorescent zones of atrophy and a classic annular hyperautofluorescent border. This case provides additional information about the natural history of this rare entity and its prognosis and varied presentation. CONCLUSIONS: The authors report a single case of acute annular outer retinopathy in a patient with invasive ductal breast carcinoma with the corresponding SD-OCT, fundus autofluorescence and visual field findings, during the acute phase of the disease. These findings provide new insight into the characteristic features, etiology and progression of this rare disease.

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Purpose: To describe a case of acute zonal occult outer retinopathy-like (AZOOR-like) presentation following scleral buckle surgery for rhegmatogenous retinal detachment. Observations: A 48-year-old man underwent successful scleral buckle with cryotherapy for repair of a left eye inferior macula-on rhegmatogenous retinal detachment. Five years later he presented with a six-month history of left peripheral field restriction. Fundus autofluorescence and optical coherence tomography demonstrated degeneration of the photoreceptors in a ring pattern around the left macula. Humphrey visual fields showed functional loss corelating with the imaging, with a paracentral ring scotoma. Electrophysiology demonstrated a delayed 30 Hz flicker latency in the left eye confirming cone system dysfunction. Conclusion and Importance: Scleral buckling surgery for repair of a rhegmatogenous retinal detachment may be associated with a late AZOOR-like presentation.

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Acute zonal occult outer retinopathy (AZOOR) is a rare condition primarily affecting the outer retina based on electrophysiologic studies. In addition to characteristic fundus appearance, spectral domain optical coherence tomography and fundus autofluorescence are unique in diagnosing AZOOR. There are a few reports on optical coherence tomography anjiography (OCTA) in AZOOR disease. In this report, we present a case using multimodal imaging including OCTA and treatment response to combined systemic antiviral, antiinflammatory, and immunosuppressive drugs. En-face OCTA outer retinal slab may provide useful tips for diagnosis and treatment response.

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Retinal disease may mimic an optic neuropathy since both may result in a relative afferent pupillary defect (RAPD), and retinal abnormalities may not be evident on a clinical exam. We report a case of a young woman with a monocular temporal hemianopia respecting the vertical meridian due to acute zonal occult outer retinopathy (AZOOR). This 34-year-old woman presented with a 10-day history of left eye vision loss and was found to have a visual acuity of 20/20 in both eyes, a left RAPD, and left temporal hemianopia on Humphrey 24-2 SITA-Fast visual field testing. Dilated fundus examination showed a normal-appearing optic nerve and retina in both eyes. She had already had a normal magnetic resonance imaging of the orbits with contrast and retinal disease was suspected. Optical coherence tomography showed dropout of the ellipsoid zone in the peripapillary retina, and fundus autofluorescence showed hyper-autoflourescence in the peripapillary region of the left eye. A diagnosis of AZOOR was made, and no improvement with prednisone occurred at final follow-up. This case demonstrates the importance of multimodal imaging in patients referred for optic neuropathies since retinal disease such as AZOOR can produce visual field defects characteristic of optic nerve disease.

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PURPOSE: To report the efficacy of anti-tumor necrosis factor α (anti-TNFα) on autoimmune-mediated macular cone dysfunction in a Japanese woman with ulcerative colitis (UC). OBSERVATIONS: A 41-year-old woman presented with bilateral visual acuity loss and photophobia. She suffered from UC, and had been treated with prednisolone and 5-aminosalicylate since age 37. Although fundus photographs and optic coherence tomography images were unremarkable, electroretinograms (ERGs) were abnormal. A full-field electroretinogram (full-field ERG) revealed mildly decreased cone responses and oscillatory potential responses bilaterally. Importantly, focal-macular ERG (fmERG) and a multifocal electroretinogram (mfERG) revealed severe macular cone dysfunction in both eyes. Infliximab, a chimeric monoclonal anti-TNFα antibody, was administrated to treat recurrent abdominal symptoms and continued at 8-week intervals. Almost 6 months after infliximab therapy, the mfERG response (especially in the central retina), the fmERG response, and visual acuity recovered bilaterally. Abdominal symptoms also improved after infliximab therapy. CONCLUSIONS AND IMPORTANCE: Bilateral cone dysfunction with normal fundus were observed in a UC patient, resulting in loss of visual acuity and photophobia. This retinopathy may have been caused by an autoimmune mechanism, such as an autoimmune retinopathy or acute zonal occult outer retinopathy, which is most identifiable by ERG changes. This is the first report demonstrating the efficacy of infliximab in autoimmune retinal dysfunction.

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Purpose: This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity. Methods: A case report and systematic literature review are presented. Results: A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit. Conclusions: Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.

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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)

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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.

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Presentamos dos casos clínicos ilustrativos con imagen multimodal de síndrome de aumento idiopático de mancha ciega (SAIMC), que debutan con puntos blancos peripapilares en el fondo de ojo. El SAIMC se engloba dentro del complejo de retinopatía zonal aguda oculta externa (AZOOR), solapándose en muchas ocasiones diferentes entidades dentro de este complejo. La hiperautofluorescencia en estos casos es típicamente peripapilar. En el en-face de la angiografía por OCT (A-OCT), a nivel de la capa de los elipsoides, se observan puntos hiperreflectivos peripapilares en ambos casos. En uno de ellos disponemos del campo visual que muestra aumento de mancha ciega reversible. El complejo AZOOR tiene en común la afectación de la retina externa que actualmente podemos demostrar con el OCT estructural. En ambos casos se observa una afectación de las capas externas que se recuperan progresivamente (AU)

Two cases are reported of acute idiopathic blind spot enlargement syndrome (AIBSE) that presented with peripapillary white spots in the fundus. AIBSE belongs to the acute zonal occult outer retinopathy (AZOOR) complex. Conditions of this AZOOR complex may overlap. Typically, in AIBSE, a peripapillary hyperautofluorescence is seen in the autofluorescence. En-face OCT angiography at the ellipsoid level showed hyper-reflective spots around the optic disk in both cases. One case showed a reversible enlargement of the blind spot in visual field. AZOOR complex is an inflammatory disorder that affects the outer retina, and can now be confirmed with structural optical coherence tomography. In the cases presented, there was a reversible severe loss of the outer retina (AU)

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