Chorioretinopathy presenting as bitemporal hemianopia.

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.

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

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.

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

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.

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

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.

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

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.

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

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.

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

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.

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.

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

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.

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

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.

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

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.

Monocular Temporal Hemianopia due to Acute Zonal Occult Outer Retinopathy.

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.

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

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.

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

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.

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.

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

PURPOSE: To report two cases; bilateral arteritic anterior ischemic optic neuropathy (AAION) and bilateral acute zonal occult outer retinopathy (AZOOR) after COVID-19 mRNA vaccination. CASE REPORT: The first patient was a 79-year-old female was presented to us 35 days after a sudden bilateral loss of vision, which occurred two days after receiving the second recombinant mRNA vaccine (Pfizer) injection. Temporal artery biopsy was compatible with AAION. At presentation, the best-corrected visual acuity was 20/1250 and 20/40 in the right and left eyes on the Snellen acuity chart, respectively. There was 3+ afferent pupillary defect in the right eye. The anterior segment and posterior segment exams were normal except for pallor of the optic nerve head in both eyes. Intraocular pressure was normal in both eyes. She was diagnosed with bilateral AAION and Subcutaneous tocilizumab 162 mg weekly was recommended with monitoring her ESR, CRP, and IL-6.The second patient was a 33-year-old healthy female who was referred to us for a progressive nasal field defect in her left eye, and for flashes in both eyes. Her symptoms started 10 days after receiving the second recombinant mRNA vaccine (Moderna) injection. Complete bloodwork performed by a uveitis specialist demonstrated high ESR (25) and CRP (19) levels. As a result, she was diagnosed with unilateral AZOOR in her left eye and was subsequently treated with an intravitreal dexamethasone implant in the same eye. At presentation, vision was20/20 in both eyes. The anterior segment and posterior segment exams were completely normal except for the presence of abnormal white reflex in the temporal macula of her left eye. We diagnosed her with bilateral AZOOR. Since she was nursing, intravitreal dexamethasone implant was recommended for the right eye. CONCLUSION: There may be a correlation between ocular inflammatory diseases with autoimmune mechanism and the mRNA COVID-19 vaccination.

Multimodal imaging in acute idiopathic blind spot enlargement syndrome.

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.

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

BACKGROUND AND AIM: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction. Evolution, response to immunosuppressive treatment and outcome are difficult to predict. The aim of this small case series was to identify the morphological changes and sequence of events in AZOOR thanks to multimodal imaging. METHODS: Charts of AZOOR patients seen in the Centre for Ophthalmic Specialized care (COS, Lausanne, Switzerland) were analyzed by multimodal imaging including fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), blue light fundus autofluorescence (BL-FAF) and spectral domain optical coherence tomography (SD-OCT) in addition to a complete ophthalmological examination including visual field testing and microperimetry, as well as OCT angiography (OCT-A) and ganglion-cell complex analysis when available. Cases and Results: Three AZOOR patients with a mean follow-up of 47 ± 25.5 months were included following the clinical definitions laid down by J Donald Gass. The primary damage was identified at the level of the photoreceptor outer segments with an intact choriocapillaris and retinal pigment epithelium (RPE) layer, these structures being only secondarily involved with progression of the disease. CONCLUSION: Although AZOOR has often been included within white dot syndromes, some of which are now known to be choriocapillaris diseases (choriocapillaritis entities), our findings clearly commend to differentiate AZOOR from entities such as MEWDS (Multiple evanescent white dot syndrome), APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy), MFC (Multifocal Choroiditis) and others, as the damage to photoreceptors is primary in AZOOR (a retinopathy) and secondary in choriocapillaritis (a choriocapillaropathy).

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

PURPOSE: 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. METHODS: Retrospective observational case series. This study included 33 patients with acute-stage AZOOR who had been followed up for more than 6 months after the initial visit. The median follow-up period was 26 months. 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. RESULTS: 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. CONCLUSIONS: Our findings suggest that the presence of ARAs did not influence visual outcomes or outer retinal morphology in patients with AZOOR.