Acute Zonal Occult Outer Retinopathy Research Articles

A case of syphilitic outer retinopathy like acute zonal occult outer retinopathy

A case of syphilitic outer retinopathy like acute zonal occult outer retinopathy

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

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

Bilateral recurrent choroidal neovascularization in AZOOR

AbstractWe present a case of acute zonal occult outer retinopathy (AZOOR) in a 61‐year‐old man who presented associated recurrent choroidal neovascularization as a complication in both eyes; he was managed with several intravitreal anti vascular endothelial growth factor (anti‐VEGF) injections in both eyes as well as with Prednisone and Methotrexate, which in improved the disease control.

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

To report the characteristics of a case series of ocular inflammatory events following COVID-19 vaccination in Japan. Retrospective multicenter study METHODS: In this retrospective multicenter survey, a questionnaire was sent to 16 Japanese hospitals that had uveitis specialty clinics. Information on patients who developed ocular inflammatory events within 14 days of COVID-19 vaccination between February 2021 and December 2021 was collected. Thirty-seven patients were diagnosed with ocular inflammatory events following COVID-19 vaccination. The mean age was 53.4 ± 16.4 years (range, 26-86 years), and the mean time to onset after vaccination was 6.3 ± 4.2 days (range, 1-14 days). Vogt-Koyanagi-Harada disease (VKH) was the most common event (n = 17 patients, 46%), followed by anterior uveitis (n = 6), infectious uveitis (n = 3), acute zonal occult outer retinopathy (AZOOR) (n = 2), sarcoidosis-associated uveitis (n = 1), acute posterior multifocal placoid pigment epitheliopathy (APMPPE) (n = 1), optic neuritis (n = 1), multiple evanescent white dot syndrome (MEWDS) (n = 1), Posner-Schlossman syndrome (n = 1), and unclassified uveitis (n = 4). Twenty-eight cases occurred after BNT162b2 vaccination (Pfizer-BioNTech) and 8 after mRNA-1273 vaccination (Moderna), whilst 1 patient had no information about vaccine type. COVID-19 vaccination can be related to various types of ocular inflammatory events. When we encounter patients with ocular inflammatory disease, we should consider that it may be an adverse effect of COVID-19 vaccination.

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

A worldwide mass vaccination campaign against the coronavirus disease 2019 (COVID-19) pandemic is currently underway. Although the safety data of the clinical trials did not report specific concerns regarding neuro-ophthalmological adverse events, they involved a limited number of individuals and were conducted over a relatively short time. The aim of the current review is to summarize the available postmarketing data regarding the occurrence of neuro-ophthalmological and other ocular complications of the COVID-19 vaccines. Electronic searches for published literature were conducted using Ovid MEDLINE, Embase, Web of Science, Google Scholar, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and ClinicalTrials.gov. The search strategy incorporated controlled vocabulary and free-text synonyms for the concepts of COVID, vaccines, and visual and neuro-ophthalmologic diseases and symptoms. A total of 14 case reports and 2 case series have been selected for inclusion in the final report, reporting 76 cases of post-COVID-vaccination adverse events. The most common adverse event was optic neuritis (n = 61), followed by uveitis (n = 3), herpes zoster ophthalmicus (n = 2), acute macular neuroretinopathy (n = 2), optic disc edema as an atypical presentation of Guillain-Barré syndrome (n = 1), (arteritic anterior ischemic optic neuropathy; n = 1), abducens nerve palsy (n = 1), oculomotor nerve palsy (n = 1), Tolosa-Hunt syndrome (n = 1), central serous retinopathy (n = 1), acute zonal occult outer retinopathy (n = 1), and bilateral choroiditis (n = 1). Most cases were treated with high-dose steroids and had a favorable clinical outcome. Since the implementation of the COVID-19 vaccination campaign in the past year, several post-COVID-vaccination neuro-ophthalmological complications have been described. However, considering the number of individuals that have been exposed to the vaccines, the risk seems very low, and the clinical outcome in most cases is favorable. Therefore, on a population level, the benefits of the vaccines far outweigh the risk of neuro-ophthalmological complications.

A case of acute zonal occult outer retinopathy which was initially diagnosed as retrobulbar optic neuritis

A case of acute zonal occult outer retinopathy which was initially diagnosed as retrobulbar optic neuritis

A Case of Acute Syphilitic Posterior Chorioretinitis That Have Been Followed Up for A Long Time as Acute Zonal Occult Outer Retinopathy

A Case of Acute Syphilitic Posterior Chorioretinitis That Have Been Followed Up for A Long Time as Acute Zonal Occult Outer Retinopathy

Resident Perspectives 56-5

Resident Perspectives 56-5

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

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. A case report and systematic literature review are presented. 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. 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.

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

To evaluate the clinical characteristics of patients with acute zonal occult outer retinopathy (AZOOR), according to the presence or absence of anti-retinal antibodies (ARAs) that are frequently detected in autoimmune retinopathy. Retrospective observational case series. This study included 33 patients with acute-stage AZOOR who had been followed up for more than 6months after the initial visit. The median follow-up period was 26months. Immunoblot analyses were used to detect autoantibodies for recoverin, carbonic anhydrase II, and α-enolase in serum from these patients. Main outcome measures comprised clinical factors at the initial and final visits, including best-corrected visual acuity, mean deviation on Humphrey perimetry, and retinal morphology, which were statistically compared between patients with AZOOR who exhibited ARAs and those who did not. At least one serum ARA was detected in 42% of patients with AZOOR. There were no significant differences in clinical factors between the two groups, including follow-up period, best-corrected visual acuity and mean deviation at the initial and final visits, a-wave amplitude on single-flash electroretinography at the initial visit, and frequencies of improvement of the macular ellipsoid zone and AZOOR recurrence. Our findings suggest that the presence of ARAs did not influence visual outcomes or outer retinal morphology in patients with AZOOR.

Automated Cone Cell Identification on Adaptive Optics Scanning Laser Ophthalmoscope Images Based on TV-L1 Optical Flow Registration and K-Means Clustering

Cone cell identification is essential for diagnosing and studying eye diseases. In this paper, we propose an automated cone cell identification method that involves TV-L1 optical flow estimation and K-means clustering. The proposed algorithm consists of the following steps: image denoising based on TV-L1 optical flow registration, bias field correction, cone cell identification based on K-means clustering, duplicate identification removal, identification based on threshold segmentation, and merging of closed identified cone cells. Compared with manually labelled ground-truth images, the proposed method shows high effectiveness with precision, recall, and F1 scores of 93.10%, 94.97%, and 94.03%, respectively. The method performance is further evaluated on adaptive optics scanning laser ophthalmoscope images obtained from a healthy subject with low cone cell density and subjects with either diabetic retinopathy or acute zonal occult outer retinopathy. The evaluation results demonstrate that the proposed method can accurately identify cone cells in subjects with healthy retinas and retinal diseases.

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

Acute zonal occult outer retinopathy (AZOOR) is a rare syndrome characterized by sudden onset of photopsia, scotomas, and abnormal electrophysiological tests, predominantly affecting young women. Although its pathogenesis remains unknown, auto-reactivity to retinal components is thought to mediate tissue damage. A 42-year-old woman presented with symptoms and examination consistent with the diagnosis of AZOOR. She was treated with azathioprine for 5 years. In spite of the immunosuppressive treatment, clear progression in the visual field, autofluorescence, electrophysiological tests and optical coherence tomography was observed. Treatment with intravenous immunoglobulins (IVIg) and subcutaneous Abatacept was subsequently started with little efficacy. Hereby, we present a case of progressive AZOOR despite aggressive immunosuppression with 10-year follow up. Currently, there is no consensus regarding management of AZOOR, and the convenience of administering aggressive immunosuppression remains uncertain.

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

This review aims to discuss retinal diseases that may masquerade as neurological causes of vision loss and highlights modern ophthalmic ancillary testing that can help to establish these diagnoses. Retinal diseases with signs and symptoms overlapping with neurological causes of vision loss include central serous chorioretinopathy, retinal ischemia, acute macular neuroretinopathy, Acute zonal occult outer retinopathy (AZOOR) complex diseases, paraneoplastic retinopathy, retinal dystrophy, and toxic retinopathy. Diagnosis is facilitated by electrophysiologic studies and multimodal ophthalmic imaging including optical coherence tomography and fundus autofluorescence imaging. Looking into the future, translation of adaptive optics ophthalmoscopy into clinical practice may facilitate early detection of microscopic retinal abnormalities that characterize these conditions. With conventional methods of physical examination, diagnosis of retinal diseases that may masquerade as neurological causes of vision loss can be challenging. Current advance in multimodal ophthalmic imaging along with electrophysiologic studies enhances the provider's ability to make early diagnosis and monitor progression of these conditions.

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

In 1988, William Hoyt, MD, et al described "acute idiopathic blind spot enlargement" (AIBSE) in 7 symptomatic patients who had no apparent abnormalities of the optic disc or surrounding retina. With the use of multifocal electroretinography, they showed that the scotoma was caused by occult retinal dysfunction. In 1992, J. Donald Gass, MD, described "acute zonal occult outer retinopathy" (AZOOR) in 13 patients who had sudden loss of often large zones of visual field without fundus abnormalities. Most patients developed zonal atrophy of retinal pigment epithelium and had no improvement in vision. Gass believed that AZOOR, multiple evanescent white dot syndrome, multifocal choroiditis, and AIBSE were all variants of the same disorder. Despite over 3 decades of numerous reports, the classification of these entities, their pathogenesis, and treatment remain controversial. AIBSE and AZOOR may be mistaken for an acute optic neuropathy, so it behooves the neuro-ophthalmologist to be familiar with these disorders. This review describes the initial recognition of AIBSE and its relationship to AZOOR.

Photopsia

‘Photopsia’ describes the symptom of visual disturbances that are typically flash-like, sudden in onset and brief, and occurring without light entering the eye. Patients reporting photopsia often pose a diagnostic...

Diffuse Phlebitis in Patients with Syphilitic Outer Retinopathy

ABSTRACT Purpose: To report two cases of syphilitic outer retinopathy that showed diffuse phlebitis on fluorescein angiography (FA). Methods: Retrospective chart review. Results: The two patients had presentation similar to acute zonal occult outer retinopathy (AZOOR), including hyperautofluorescence on fundus autofluorescence and ellipsoid zone disruption on optical coherence tomography. The main difference from AZOOR was the finding of diffuse phlebitis on FA. Both patients recovered well after antibiotic and steroid treatment. Conclusion: FA is especially important for differentiating syphilitic outer retinopathy from AZOOR. Timely diagnosis and treatment with penicillin and cautious use of steroid usually lead to favorable prognosis.

Progressive Enlarging Blind Spot in a Young Asian Lady: A Case Report on Acute Zonal Occult Outer Retinopathy

Acute Zonal Occult Outer Retinopathy (AZOOR) is a very rare retinal disease that predominantly affect the Caucasians. It is usually present in a young myopic woman with an acute onset of photopsia and enlarging blind spots. Here, we report a young Asian lady who presented with a typical presentation of AZOOR and later confirmed by electrophysiological test of the retina. She was wrongly diagnosed as retrobulbar optic neuritis due to low index of suspicion for AZOOR among the treating physicians in the hospital. Fortunately, she responded to systemic corticosteroid that was given as a treatment of optic neuritis. AZOOR was only diagnosed later by a visiting European neuro-ophthalmologist in a neighboring country. The objective of this case report is to remind the reader that the condition exists in Asian population and should be kept as a differential diagnosis in a young lady who presented with progressive visual field defect.

Masqueraders of multiple evanescent white dot syndrome (MEWDS).

To describe disorders that can masquerade as multiple evanescent white dot syndrome (MEWDS). Retrospective, multicenter case series. Patients who presented with clinical findings compatible with a diagnosis of MEWDS but were ultimately diagnosed with an alternative inflammatory, infectious, or neoplastic disorder. Clinical records and multimodal imaging findings including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) were analyzed. Inclusion criteria to be defined as a masquerade syndrome for MEWDS included the presence of disseminated grayish-white outer retinal spots that were hyperautofluorescent on FAF and associated with ellipsoid zone (EZ) disruption on OCT. Twenty-two eyes of 13 patients were identified. All patients presented with the classic findings of MEWDS listed above. A MEWDS-like presentation was bilateral in nine of 13 patients (69%). Final diagnosis was determined on the basis of additional investigations including serologies and biopsy. These diagnoses included syphilis (three patients), lymphoma (three patients), idiopathic multifocal choroiditis (two patients), idiopathic retinal phlebitis (one patient), idiopathic acute zonal occult outer retinopathy (one patient), sarcoidosis (one patient), tuberculosis (one patient), and cancer-associated retinopathy (one patient). The outer retinal lesions and imaging findings resolved with treatment for the associated systemic disorders. Widespread grayish-white outer retinal spots associated with hyperautofluorescence on FAF and disruption of the EZ on OCT are not pathognomonic for MEWDS. A high index of suspicion must be maintained for masqueraders of MEWDS, which can include serious inflammatory, infectious, and neoplastic disorders.

White dot syndromes : Principles, diagnostics, and treatment

The white dot syndromes include a group of diseases which are characterized by multiple yellowish-white foci in the outer retina, retinal pigment epithelium, and choroid. For clinicians and researchers alike they present significant diagnostic and therapeutic challenges. White dot syndromes include primary inflammatory choriocapillaropathies, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE)/acute multifocal ischemic choriocapillaropathy (AMIC), multiple evanescent white dot syndrome (MEWDS)/acute idiopathic blind spot enlargement (AIBSE), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), serpiginous choroiditis (SC), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Among the primary stromal choroiditis is birdshot retinochoroidopathy (BSRC); however, the pathogenesis of these disorders is largely unknown. Immunological reactions to previous viral infections with a genetic disposition seem to be acommon denominator.

The status in the application of optical coherence tomography angiography in noninfectious uveitis

Noninfectious uveitis refers to a category of inflammatory diseases involving the uvea, vitreous, optic disk and retina, with the exception of infectious factors or masquerade syndrome. These kind of blinding diseases are frequently recurrent, and the diagnosis and follow-up require fundus imaging techniques. OCT angiography (OCTA) is a rapid, noninvasive and quantifiable blood flow imaging modality that provides a depiction of the microvasculature morphology of the retinal and choroidal through different segmentation and detects the abnormal blood perfusion as well as the neovascularization. OCTA plays an important role in the diagnosis, assessment and follow-up for anterior uveitis, posterior uveitis and pan-uveitis such as Vogt-Koyanagi-Harada disease, Behcet’s disease, ocular sarcoidosis, birdshot chorioretinopathy, serpiginous choroiditis, multifocal choroiditis, punctate inner choroidopathy, acute zonal occult outer retinopathy, acute posterior multifocal placoid pigment epitheliopathy, multiple evanescent white dot syndrome, and also provides clue about their pathophysiologic mechanisms. Key words: Uveitis; Tomography, optical coherence; Review