Raras
Buscar doenças, sintomas, genes...
Retinopatia oculta externa zonal aguda
ORPHA:284454CID-10 · H35.0CID-11 · 9B65.0DOENÇA RARA

AZOOR (Retinopatia Externa Oculta Zonal Aguda) é uma doença rara que afeta os olhos. Pessoas com esta condição podem apresentar um início súbito de clarões de luz e uma área de perda parcial da visão (um ponto cego). Outros sintomas podem incluir "visão esbranquiçada" ou visão embaçada. Embora qualquer pessoa possa ser afetada, a condição é mais frequentemente diagnosticada em mulheres jovens (com uma idade média de 36,7 anos). A causa exata do AZOOR é atualmente desconhecida; no entanto, alguns pesquisadores sugerem que agentes infecciosos (como vírus) ou a autoimunidade podem desempenhar um papel no desenvolvimento da doença. Não há tratamento comprovado que melhore a visão de quem tem AZOOR; no entanto, corticosteroides que agem em todo o corpo são o tratamento mais utilizado.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

AZOOR (Retinopatia Externa Oculta Zonal Aguda) é uma doença rara que afeta os olhos. Pessoas com esta condição podem apresentar um início súbito de clarões de luz e uma área de perda parcial da visão (um ponto cego). Outros sintomas podem incluir "visão esbranquiçada" ou visão embaçada. Embora qualquer pessoa possa ser afetada, a condição é mais frequentemente diagnosticada em mulheres jovens (com uma idade média de 36,7 anos). A causa exata do AZOOR é atualmente desconhecida; no entanto, alguns pesquisadores sugerem que agentes infecciosos (como vírus) ou a autoimunidade podem desempenhar um papel no desenvolvimento da doença. Não há tratamento comprovado que melhore a visão de quem tem AZOOR; no entanto, corticosteroides que agem em todo o corpo são o tratamento mais utilizado.

Pesquisas ativas
1 ensaio
1 total registrados no ClinicalTrials.gov
Publicações científicas
213 artigos
Último publicado: 2026 Mar 24

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
150
pacientes catalogados
Início
Adolescent
+ adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H35.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

👁️
Olhos
11 sintomas
💪
Músculos
1 sintomas
❤️
Coração
1 sintomas

+ 11 sintomas em outras categorias

Características mais comuns

55%prev.
Visão turva
Frequente (79-30%)
55%prev.
Angiografia fluoresceínica anormal do fundo de olho
Frequente (79-30%)
55%prev.
Fotopsia
Frequente (79-30%)
55%prev.
Anormalidade da refração
Frequente (79-30%)
55%prev.
Defeito do campo visual
Frequente (79-30%)
55%prev.
Pupila de Marcus Gunn
Frequente (79-30%)
24sintomas
Frequente (10)
Ocasional (10)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

Visão turvaBlurred vision
Frequente (79-30%)55%
Angiografia fluoresceínica anormal do fundo de olhoAbnormal fundus fluorescein angiography
Frequente (79-30%)55%
FotopsiaPhotopsia
Frequente (79-30%)55%
Anormalidade da refraçãoAbnormality of refraction
Frequente (79-30%)55%
Defeito do campo visualVisual field defect
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico213PubMed
Últimos 10 anos127publicações
Pico201715 papers
Linha do tempo
2026Hoje · 2026📈 2017Ano de pico🧪 2019Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Retinopatia oculta externa zonal aguda

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
122 papers (10 anos)
#1

Unmasking neurosarcoidosis in a patient with acute zonal occult outer retinopathy: a case report.

BMC ophthalmology2026 Jan 29

We report a case of neurosarcoidosis in a patient with a prior history of acute zonal occult outer retinopathy (AZOOR), using multimodal imaging, including color fundus photography, spectral-domain optical coherence tomography, fluorescein and indocyanine green (ICG) angiography. A 37-year-old female with a history of AZOOR presented with new-onset bilateral photopsia and a paracentral scotoma in the left eye. Examination revealed bilateral anterior chamber reaction, optic disc edema, vitreous cells, and choroiditis on ICG angiography. Given the presentation, AZOOR recurrence was deemed unlikely, and further workup for panuveitis was initiated. A week later, she developed multiple cranial neuropathies, and further systemic evaluation confirmed neurosarcoidosis. High-dose corticosteroids and methotrexate resolved both neurological and ophthalmic symptoms. This case highlights the importance of carefully evaluating patients with inflammatory eye conditions, as new symptoms might suggest a different diagnosis rather than a recurrence of the preexisting condition. Prompt recognition and adequate treatment can significantly impact patient outcomes.

#2

En Face Optical Coherence Tomography and OCT Angiography in the Pathoanatomy of Inflammatory Macular Disease.

American journal of ophthalmology2026 Apr

To review the pivotal role of en face optical coherence tomography (OCT) and OCT angiography (OCTA) in elucidating the pathoanatomy, diagnostic markers, and clinical management of inflammatory macular diseases. These noninvasive modalities provide depth-resolved, layer-specific visualization of structural and vascular alterations, enhancing our understanding of disease mechanisms and facilitating monitoring of disease activity and therapeutic response. This perspective article synthesizes recent advances in multimodal imaging (MMI) with emphasis on en face OCT and OCTA across the spectrum of posterior noninfectious and infectious uveitic disorders. Representative diseases include multiple evanescent white dot syndrome (MEWDS), punctate inner choroidopathy (PIC), and idiopathic multifocal choroiditis (iMFC), acute zonal occult outer retinopathy (AZOOR), acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and its chronic variants, serpiginous choroiditis (SC), acute syphilitic posterior placoid chorioretinopathy (ASPPC), and Vogt-Koyanagi-Harada (VKH) disease. Imaging signatures were reviewed in relation to pathophysiology, differential diagnosis, and clinical management. En face OCT delineates disease-specific topographic patterns: "dots and spots" in MEWDS, hyperreflective plaques in PIC/iMFC, trizonal maps in AZOOR, sharply demarcated placoid lesions in APMPPE, geographic patterns in SC, and concentric hyperreflective "fingerprint" rings in VKH. OCTA provides complementary vascular insights, distinguishing preserved vs impaired choriocapillaris (CC) flow and revealing disease-driven ischemia. MEWDS typically demonstrates preserved CC perfusion, in contrast to the flow deficits of APMPPE, relentless placoid chorioretinitis, and SC. In PIC/iMFC, OCTA enables detection of inflammatory choroidal neovascularization (iCNV), while in ASPPC and VKH, it captures reversible or persistent CC flow voids following appropriate systemic therapy. Structural-vascular correlation permits differentiation between ischemic and nonischemic disorders, recognition of masquerades, and monitoring of subclinical disease activity. En face OCT and OCTA have redefined the diagnostic and management landscape of inflammatory macular diseases by providing rapid, reproducible, and layer-specific structural-vascular information. Their disease-specific signatures enhance accuracy, support treatment decisions, and improve monitoring of progression and complications such as inflammatory CNV. Integration of these tools into routine multimodal imaging protocols is essential, while future research should prioritize standardization of acquisition and interpretation criteria, quantitative biomarkers, and expanded use of widefield technologies to capture peripheral and longitudinal disease dynamics.

#3

New Insights into Imaging Patterns of Autoimmune Retinopathies: A Cluster-Based Update.

American journal of ophthalmology2026 Apr

To describe multimodal imaging (MMI) features in autoimmune retinopathies (AIR) and characterize distinct phenotypic clusters. Retrospective observational case series. Ten patients diagnosed with AIR between November 2017 and August 2025 were evaluated at the Ocular Immunology Unit, Azienda Unità Sanitaria Locale-IRCCS, Reggio Emilia, Italy. Patients with paraneoplastic and nonparaneoplastic AIR underwent full-field electroretinography and MMI, including retinography, fundus autofluorescence (FAF), optical coherence tomography (OCT), fluorescein angiography (FA), and indocyanine green angiography. Qualitative pattern analysis was performed to identify recurring imaging features. MMI characteristics of AIR. Of 30 referred patients, 10 (14 eyes) met inclusion criteria (median age 42 years, range 28 to 65; 70% female; median follow-up 40 months). Three recurring imaging clusters were identified. One cluster showed features within the acute zonal occult outer retinopathy spectrum, including monozonal, bizonal, and trizonal configurations on FAF and OCT. A second cluster was characterized by predominant posterior pole photoreceptoritis with subtle FAF changes and diffuse outer retinal alterations on OCT. A third cluster showed unilateral, peripheral, retinitis pigmentosa-like degeneration with centripetal progression on FAF and mild capillary leakage on fluorescein angiography. In this small case series, MMI revealed recurring phenotypic clusters in AIR that may help contextualize the heterogeneity of this condition. These observations are exploratory and hypothesis-generating, and warrant validation in larger, multicenter studies.

#4

Chronic changes in acute zonal occult outer retinopathy mimicking non-progressive retinitis pigmentosa: clinical and laboratory characteristics.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie2026 Mar 24
#5

[Correction: Acute zonal occult outer retinopathy after cataract surgery].

Klinische Monatsblatter fur Augenheilkunde2026 Mar 04

Publicações recentes

Ver todas no PubMed

📚 EuropePMC137 artigos no totalmostrando 126

2026

Chronic changes in acute zonal occult outer retinopathy mimicking non-progressive retinitis pigmentosa: clinical and laboratory characteristics.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2026

[Correction: Acute zonal occult outer retinopathy after cataract surgery].

Klinische Monatsblatter fur Augenheilkunde
2026

Unmasking neurosarcoidosis in a patient with acute zonal occult outer retinopathy: a case report.

BMC ophthalmology
2026

Acute zonal occult outer retinopathy complex and angioid streaks: a case report.

International journal of ophthalmology
2026

En Face Optical Coherence Tomography and OCT Angiography in the Pathoanatomy of Inflammatory Macular Disease.

American journal of ophthalmology
2026

New Insights into Imaging Patterns of Autoimmune Retinopathies: A Cluster-Based Update.

American journal of ophthalmology
2025

Acute Zonal Occult Outer Retinopathy after Cataract Surgery.

Klinische Monatsblatter fur Augenheilkunde
2025

Usefulness of Color Laser Scanning Ophthalmoscopy and Optical Coherence Tomography Angiography in the Diagnosis of Acute Zonal Occult Outer Retinopathy Complex: A Report of Two Cases.

Case reports in ophthalmology
2025

Clinical manifestation and multimodal images of Chinese acute zonal occult outer retinopathy patients.

International journal of ophthalmology
2025

Noninfectious Uveitis Syndromes.

Advances in experimental medicine and biology
2025

Structural, Functional, and Cellular Analysis of a Case of Acute Zonal Occult Outer Retinopathy (AZOOR).

Biomedicines
2025

Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR) with a chronologically divergent presentation- a case report.

Journal of ophthalmic inflammation and infection
2025

Autoimmune Retinopathy in a Patient with Common Variable Immunodeficiency.

Ocular immunology and inflammation
2025

A Case of Concomitant Acute Zonal Occult Outer Retinopathy and Secondary Nonparaneoplastic Autoimmune Retinopathy.

Case reports in ophthalmology
2025

Acute zonal occult outer retinopathy (AZOOR) complex associated with torpedo retinopathy following COVID-19 recombinant mRNA vaccination: a case report of a rare clinical presentation.

BMC ophthalmology
2025

The importance of genetic and electrophysiological studies in the differential diagnosis of acute zonal occult outer retinopathy (AZOOR).

Indian journal of ophthalmology
2024

A case documenting distinct natural history of multizonal outer retinopathy and retinal pigment epitheliopathy (MORR) with longitudinal multi-modal documentation of progression.

American journal of ophthalmology case reports
2024

An update of multimodal imaging in white dot syndrome.

Oman journal of ophthalmology
2024

DIAGNOSTIC YIELD OF AN INHERITED RETINAL DISEASE GENE PANEL IN RETINOPATHY OF UNKNOWN ORIGIN.

Retina (Philadelphia, Pa.)
2024

Incidence and Clinical Characteristics of COVID-19 Ophthalmopathy Following the Termination of Dynamic Zero COVID-19 Strategy in China.

Neuro-ophthalmology (Aeolus Press)
2024

Chorioretinopathy presenting as bitemporal hemianopia.

Journal of the neurological sciences
2024

[Bilateral acute regional occult outer retinopathy: a case report].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2025

A CASE OF ACUTE ZONAL OCCULT OUTER RETINOPATHY UNDER IMMUNOMODULATORY TREATMENT.

Retinal cases &amp; brief reports
2024

Application of Ophthalmic Electrophysiology in Inflammatory Disorders of Retina and Optic Nerve.

Journal of clinical medicine
2025

Long-Term Clinical Course and Treatment Outcomes in Occult and Overt Acute Zonal Outer Retinopathy.

Ocular immunology and inflammation
2024

A Case of an Acute Zonal Occult Outer Retinopathy Variant Characterized With an Insidious Peripheral Onset and Centripetal Progression.

Cureus
2024

Bilateral symmetric acute zonal occult outer retinopathy.

BMJ case reports
2024

Clinical features and possible pathogenesis of multiple evanescent white dot syndrome with different retinal diseases and events: a narrative review.

International journal of ophthalmology
2024

Current understanding of acute zonal occult outer retinopathy (AZOOR).

Indian journal of ophthalmology
2023

MULTIZONAL OUTER RETINOPATHY AND RETINAL PIGMENT EPITHELIOPATHY (MORR): A Newly Recognized Entity or an Unusual Variant of AZOOR?

Retina (Philadelphia, Pa.)
2024

Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES) in the Era of OCT - a Review.

Klinische Monatsblatter fur Augenheilkunde
2024

Acute zonal occult outer retinopathy (AZOOR): Case report with confocal scanning laser ophthalmoscopy retromode imaging.

Journal francais d'ophtalmologie
2023

A Case of Acute Zonal Occult Outer Retinopathy in which Oct en Face Imaging Was Useful for Diagnosis and Follow-up.

Acta medica Okayama
2023

Latest advances in white spot syndromes: New findings and interpretations.

Progress in retinal and eye research
2023

Primary versus Secondary Elevations in Fundus Autofluorescence.

International journal of molecular sciences
2023

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Diagnostics (Basel, Switzerland)
2023

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

Archivos de la Sociedad Espanola de Oftalmologia
2023

AZOOR with unilateral disc edema: An atypical case report.

Archivos de la Sociedad Espanola de Oftalmologia
2022

A systematic review of acute zonal occult outer retinopathy with a focus on attempted treatment modalities.

Current ophthalmology reports
2022

Acute annular outer retinopathy preceded by invasive ductal breast carcinoma: a case report.

BMC ophthalmology
2023

A multicenter study of ocular inflammation after COVID-19 vaccination.

Japanese journal of ophthalmology
2022

Acute zonal occult outer retinopathy in a patient with retinitis pigmentosa.

Digital journal of ophthalmology : DJO
2022

Acute zonal occult outer retinopathy-like presentation secondary to scleral buckle.

American journal of ophthalmology case reports
2022

Acute zonal occult outer retinopathy: optical coherence tomography angiography findings and treatment response.

GMS ophthalmology cases
2022

Neuro-Ophthalmological Complications of the COVID-19 Vaccines: A Systematic Review.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2022

Monocular Temporal Hemianopia due to Acute Zonal Occult Outer Retinopathy.

Case reports in ophthalmology
2022

En Face OCT in Acute Zonal Occult Outer Retinopathy.

Ophthalmology
2022

Infliximab recovers central cone dysfunction with normal fundus in a patient with ulcerative colitis.

American journal of ophthalmology case reports
2021

Use of multimodal analysis in the diagnosis and follow-up of a case of Acute Zonal Occult Outer Retinopathy (AZOOR).

Archivos de la Sociedad Espanola de Oftalmologia
2021

Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up.

Medicina (Kaunas, Lithuania)
2021

Neurosyphilis Presenting as an Atypical Case of Posterior Placoid Chorioretinitis in a Young, HIV-Negative Male.

Cureus
2021

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

Archivos de la Sociedad Espanola de Oftalmologia
2021

COVID-19 Recombinant mRNA Vaccines and Serious Ocular Inflammatory Side Effects: Real or Coincidence?

Journal of ophthalmic &amp; vision research
2022

Paraneoplastic ocular syndrome: a pandora's box of underlying malignancies.

Eye (London, England)
2021

Multimodal imaging in acute idiopathic blind spot enlargement syndrome.

Archivos de la Sociedad Espanola de Oftalmologia
2022

Association of Acute Zonal Occult Outer Retinopathy With Multiple Sclerosis: A Report of 2 Cases.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2021

Acute Zonal Occult Outer Retinopathy (AZOOR) Results from a Clinicopathological Mechanism Different from Choriocapillaritis Diseases: A Multimodal Imaging Analysis.

Diagnostics (Basel, Switzerland)
2022

Syphilitic Outer Retinopathy: A Case Report and Review of the Literature.

Journal of vitreoretinal diseases
2021

A Patient-Centered Integrative Approach Improves Visual Field Defect: A Case Report.

Global advances in health and medicine
2021

Comparison of clinical characteristics in patients with acute zonal occult outer retinopathy according to anti-retinal antibody status.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2021

Bitemporal Hemianopia Secondary to Acute Zonal Occult Outer Retinopathy.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2021

Acute zonal occult outer retinopathy complex disease: Lessons learned about choroid, photoreceptors, and retinal function.

European journal of ophthalmology
2020

Long term follow-up and effect of immunosuppression in acute zonal occult outer retinopathy.

European journal of ophthalmology
2021

Diagnostic and Therapeutic Challenges.

Retina (Philadelphia, Pa.)
2020

Acute zonal occult outer retinopathy presenting as optic neuritis.

Taiwan journal of ophthalmology
2020

Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss.

Current neurology and neuroscience reports
2020

Acute Idiopathic Blind Spot Enlargement and Acute Zonal Occult Outer Retinopathy: Potential Mimics of Neuro-Ophthalmic Disease.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2022

Atypical Syphilitic Outer Retinitis and Severe Retinal Vasculitis as Onset Manifestations in a Patient with Concurrent HIV and Syphilis Infection.

Ocular immunology and inflammation
2021

Unilaterally extinguished electroretinograms in an eye with normal visual acuity and visual field.

Documenta ophthalmologica. Advances in ophthalmology
2022

ACUTE ZONAL OCCULT OUTER RETINOPATHY: A CASE REPORT OF REGRESSION AFTER AN INTRAVITREAL DEXAMETHASONE (OZURDEX) IMPLANT.

Retinal cases &amp; brief reports
2021

Diffuse Phlebitis in Patients with Syphilitic Outer Retinopathy.

Ocular immunology and inflammation
2020

Ocular Syphilis mimicking Acute Zonal Occult Outer Retinopathy (AZOOR).

Archivos de la Sociedad Espanola de Oftalmologia
2022

ADAPTIVE OPTICS OPTICAL COHERENCE TOMOGRAPHY IN A CASE OF ACUTE ZONAL OCCULT OUTER RETINOPATHY.

Retinal cases &amp; brief reports
2021

Long-term Outcome of Zonal Outer Retinopathy in Punctate Inner Choroidopathy or Multifocal Choroiditis.

Ocular immunology and inflammation
2020

Masqueraders of multiple evanescent white dot syndrome (MEWDS).

International ophthalmology
2019

[White dot syndromes : Principles, diagnostics, and treatment].

Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
2020

Lessons learned from quantitative fundus autofluorescence.

Progress in retinal and eye research
2019

Optical Coherence Tomography Angiography (OCT-A) in a Patient with Occult Retinal Dysfunction.

Case reports in ophthalmological medicine
2019

A case report of acute zonal occult outer retinopathy.

European journal of ophthalmology
2019

Bilateral acute zonal occult outer retinopathy (AZOOR) in a young adult Indian male.

GMS ophthalmology cases
2019

Unilateral pigmentary retinopathy: a retrospective case series.

Acta ophthalmologica
2018

Acute Zonal Occult Outer Retinopathy (AZOOR) and Related Diseases.

Advances in experimental medicine and biology
2019

Acute annular outer retinopathy associated with human immunodeficiency virus.

Archivos de la Sociedad Espanola de Oftalmologia
2018

Multimodal imaging of acute zonal occult outer retinopathy in a Indian male.

Indian journal of ophthalmology
2018

Panoramic optical coherence tomography angiography features in acute zonal occult outer retinopathy.

Indian journal of ophthalmology
2018

Acute zonal occult outer retinopathy: Is optical coherence tomography angiography useful?

Indian journal of ophthalmology
2018

Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization.

International journal of retina and vitreous
2018

Multimodal imaging signatures in a case of acute zonal occult outer retinopathy.

Indian journal of ophthalmology
2018

From A… to… Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy.

Neuro-ophthalmology (Aeolus Press)
2018

Treatment of Acute Zonal Occult Outer Retinopathy With Intravitreal Steroids.

Ophthalmic surgery, lasers &amp; imaging retina
2019

Multimodal Imaging of Post-Infectious Unilateral Outer Retinopathy and Choroiditis.

Ocular immunology and inflammation
2017

Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex.

BMC ophthalmology
2019

CHOROIDAL THICKNESS CHANGES IN ACUTE ZONAL OCCULT OUTER RETINOPATHY.

Retina (Philadelphia, Pa.)
2017

Quantitative Autofluorescence Intensities in Acute Zonal Occult Outer Retinopathy vs Healthy Eyes.

JAMA ophthalmology
2017

Relationship between Choroidal Thickness and Visual Field Impairment in Acute Zonal Occult Outer Retinopathy.

Journal of ophthalmology
2019

Acquired unilateral scotoma.

Survey of ophthalmology
2018

ACUTE ZONAL OCCULT OUTER RETINOPATHY: Structural and Functional Analysis Across the Transition Zone Between Healthy and Diseased Retina.

Retina (Philadelphia, Pa.)
2018

The clinical characteristics and prognosis of acute zonal occult outer retinopathy.

International ophthalmology
2017

Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases.

American journal of ophthalmology
2017

Acute Zonal Cone Photoreceptor Outer Segment Loss.

JAMA ophthalmology
2017

Ultra-Wide-Field Fundus Autofluorescence and Spectral-Domain Optical Coherence Tomography Findings in Syphilitic Outer Retinitis.

Ophthalmic surgery, lasers &amp; imaging retina
2017

Observation and Clinical Pattern in Patients with White Dot Syndromes: The Role of Color Photography in Monitoring Ocular Changes in Long-Term Observation.

Medical science monitor : international medical journal of experimental and clinical research
2017

Acute Zonal Occult Outer Retinopathy Associated With Retrobulbar Optic Neuritis.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2017

[Multimodal Approaches for the Analysis of Retinal Functional Disorders―Focusing on Retinal Detachment].

Nippon Ganka Gakkai zasshi
2017

Fundus Autofluorescence Imaging in the Assessment of Acute Zonal Occult Outer Retinopathy.

Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde
2017

Rapid Loss of Vision With the Onset of a White Ring Surrounding the Optic Nerve.

JAMA ophthalmology
2017

Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases.

American journal of ophthalmology
2017

MULTIPLE EVANESCENT WHITE DOT SYNDROME WITH CENTRAL VISUAL LOSS.

Retinal cases &amp; brief reports
2017

ULTRA-WIDE-FIELD FUNDUS AUTOFLUORESCENCE FINDINGS IN PATIENTS WITH ACUTE ZONAL OCCULT OUTER RETINOPATHY.

Retina (Philadelphia, Pa.)
2016

Pure small cell carcinoma of the prostate preceded by acute zonal occult outer retinopathy: A case report.

International journal of surgery case reports
2016

[Acute zonal occult outer retinopathy].

Journal francais d'ophtalmologie
2017

ACUTE ZONAL OCCULT OUTER RETINOPATHY AFFECTING THE PERIPHERAL RETINA WITH CENTRIPETAL PROGRESSION.

Retinal cases &amp; brief reports
2016

Autoimmunity in visual loss.

Handbook of clinical neurology
2016

Recovery of outer retina in acute idiopathic blind spot enlargement (AIBSE).

American journal of ophthalmology case reports
2016

OCT Angiography Identification of Choroidal Neovascularization Secondary to Acute Zonal Occult Outer Retinopathy.

Ophthalmic surgery, lasers &amp; imaging retina
2015

Acute Zonal Occult Outer Retinopathy; Revisited.

Journal of ophthalmic &amp; vision research
2016

Occult hypertensive choroidopathy: novel finding of suprachoroidal fluid.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2016

AZOOR - A 10-year follow-up.

Acta ophthalmologica
2015

The Fundus Autofluorescence Spectrum of Punctate Inner Choroidopathy.

Journal of ophthalmology
2015

Improved Photoreceptor Function in Male Acute Zonal Occult Outer Retinopathy.

Optometry and vision science : official publication of the American Academy of Optometry
2015

Is Acute Zonal Occult Outer Retinopathy an Autoimmune Condition? A Case Report and Literature Review.

Ophthalmic surgery, lasers &amp; imaging retina
2015

[Acute zonal occult outer retinopathy - a case with rapid return of visual functions in type 3 disease].

Ceska a slovenska oftalmologie : casopis Ceske oftalmologicke spolecnosti a Slovenske oftalmologicke spolecnosti
2016

[Progressive scotoma with perception of photopsia].

Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
2015

NEAR-INFRARED REFLECTANCE IMAGING IN EYES WITH ACUTE ZONAL OCCULT OUTER RETINOPATHY.

Retina (Philadelphia, Pa.)
2015

Acute zonal occult outer retinopathy in Japanese patients: clinical features, visual function, and factors affecting visual function.

PloS one
2015

Systemic corticosteroids therapy in the management of acute zonal occult outer retinopathy.

Journal of ophthalmology
Ver todos os 137 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Retinopatia oculta externa zonal aguda

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Unmasking neurosarcoidosis in a patient with acute zonal occult outer retinopathy: a case report.
    BMC ophthalmology· 2026· PMID 41612300mais citado
  2. En Face Optical Coherence Tomography and OCT Angiography in the Pathoanatomy of Inflammatory Macular Disease.
    American journal of ophthalmology· 2026· PMID 41490810mais citado
  3. New Insights into Imaging Patterns of Autoimmune Retinopathies: A Cluster-Based Update.
    American journal of ophthalmology· 2026· PMID 41483864mais citado
  4. Chronic changes in acute zonal occult outer retinopathy mimicking non-progressive retinitis pigmentosa: clinical and laboratory characteristics.
    Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie· 2026· PMID 41874676mais citado
  5. [Correction: Acute zonal occult outer retinopathy after cataract surgery].
    Klinische Monatsblatter fur Augenheilkunde· 2026· PMID 41780542mais citado
  6. Acute zonal occult outer retinopathy complex and angioid streaks: a case report.
    Int J Ophthalmol· 2026· PMID 41524022recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:284454(Orphanet)
  2. MONDO:0017298(MONDO)
  3. GARD:8640(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q794002(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Retinopatia oculta externa zonal aguda
Compêndio · Raras BR

Retinopatia oculta externa zonal aguda

ORPHA:284454 · MONDO:0017298
Prevalência
Unknown
Casos
150 casos conhecidos
Herança
Not applicable
CID-10
H35.0 · Retinopatias de fundo e alterações vasculares da retina
CID-11
Ensaios
1 ativos
Início
Adolescent, Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0730298
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso
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