A brief talk about the diagnosis of "outer retinopathy" — based on the recognition and understanding of optical coherence tomography images / 中华眼底病杂志

Outer retinopathy does not refer to a specific type of retinal disease. Patients with outer retinopathy often have abnormal vision symptoms, however, no positive signs can be found with conventional routine eye examination. And the diseases are often labeled "occult" . In recent years, optical coherence tomography (OCT) has been widely used in clinical practice. It has been found that many so-called "occult" diseases are actually caused by structural abnormalities of the outer retina. The causes of structural abnormalities are diverse, and the treatments and disease outcomes are also different. Therefore, it is necessary for clinical ophthalmologists to get detailed medical history, make diagnosis and differential diagnosis based on multi-model imaging, rather than roughly name it as "outer retinopathy" . With the development of OCT imaging technology, higher resolution images reveal the finer structure of retinal tissue, allowing us to have a deep understanding of the disease, thus improving diagnosis and treatment in clinical practice.

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

A 44-year-old female presented with central vision loss and photopsia in both eyes since 2 months. Multimodal imaging, field defects, electroretinogram abnormalities, and spectral domain optical coherence tomography changes were all suggestive of acute zonal occult outer retinopathy. En-face optical coherence tomography angiography (OCTA) images demonstrated hyper-reflective dots at the level of ellipsoid zone in both eyes in the presence of normal retinochoroidal vasculature. The patient was started on oral azathioprine and prednisolone. On two consecutive monthly follow-ups, en-face OCTA images demonstrated serial changes in the hyper-reflective dot morphology at ellipsoid zone level that have not been previously reported in the literature.

Clinical Characteristics of Acute Zonal Occult Outer Retinopathy

PURPOSE: To investigate the clinical characteristics of patients with acute zonal occult outer retinopathy (AZOOR). METHODS: Medical records of 13 patients who visited Seoul National University Bundang Hospital from May, 2003 to May, 2015 and diagnosed with AZOOR were retrospectively reviewed. RESULTS: Thirteen patients (11 women and 2 men), with a mean age of 28.5 ± 11.4 years were followed for 42.8 ± 30.3 months. Visual field loss, photopsia, and blurred vision were common complaints. Initially, four patients had bilateral disease and seven patients showed bilateral involvement at the last visit. Mean best corrected visual acuity of involved eyes (BCVA) was 0.75 ± 0.32 (log MAR). Among 20 eyes with AZOOR, BCVA was 20/40 or better in 17 eyes (85.0%). The mean spherical equivalent was -4.59 ± 3.23 diopters (D), and 15 eyes (75.0%) had myopia less than -2.00 D. Nine eyes of seven patients (34.6%) had abnormal fundus findings. All patients underwent full field electroretinogram (ERG) or multifocal ERG and a visual field test. Thirteen patients (100.0%) showed a decreased response in ERG and visual field defects presented in every patient. With respect to the visual field test, 10 eyes (50.0%) showed improvement, 6 eyes (30.0%) had stationary status, and the progression of the visual field defect was observed in 4 eyes (20.0%). Among 13 patients, 4 (30.8%) patients showed flu-like symptom, 4 (30.8%) patients had fatigue, 2 (15.4%) patients had nausea, and 2 (15.4%) patients showed headache. CONCLUSIONS: AZOOR should be considered as one of the differential diagnoses, especially in female patients with myopia who show photopsia or visual field defects. ERG and visual field tests are necessary to confirm a decrease in retinal function and visual field loss. Central vision is preserved in most cases and recovery of visual field defect occurs often.

Current research of acute zonal occult outer retinopathy / 中华实验眼科杂志

Acute zonal occult outer retinopathy (AZOOR) is a relatively rare ocular disease that is characterized by the rapid loss of one or more zones of outer retinal function.AZOOR usually occurs in young women.AZOOR patients typically present with the sudden onset of photopsia and acute scotomas related to loss of sectors of outer retinal function.Early in the course of AZOOR,funduscopic appearance was often normal;however,most patients developed zones of retinal pigment epithelial atrophy or pigment clumping over time.Changes in electroretinogram (ERG) and persistent visual field defects were usually observed.Optical coherence tomography (OCT) showed that inner segment (IS)/outer segment (OS) regional abnormal changes corresponding to the visual field defect location area,at the same time the morphology and position of the visual field defect and multifocal electroretinogram (mfERG) shown in retinal amplitude density decreased regions coincide.The visual function of some patients can permanently damage.Fundus examination,mfERG and fluorescein angiography (FA),indocyanine green angiography (ICGA),autofluorescence (AF) and frequency domain OCT imaging techniques has an important significance for the diagnosis of AZOOR.Ever since the lack of understanding,AZOOR is often missed or misdiagnosed.In order to improve the understanding of the disease,this paper reviewed the definition,etiology,the epidemiological characteristics,clinical manifestation,classification,diagnosis,differential diagnosis,treatment and prognosis of AZOOR.

Ocular Syphilis Presented as Occult Outer Retinopathy

PURPOSE: To report a case of ocular syphilis presenting as occult outer retinopathy. CASE SUMMARY: A 42-year-old female presented with decreased visual acuity for one month in duration in the left eye. Her best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/250 in the left eye. There was no sign of inflammation in the anterior segment. Fundus examination showed no distinct abnormal findings including vitreous cell or haziness except mild diabetic retinopathy and subtle opacity of the macula. Spectral domain optical coherence tomography (OCT) showed a marked distortion of the inner segment-outer segment (IS-OS) junction in the photoreceptor layer without macular edema. Fluorescent angiography revealed diffuse subtle hyperfluorescence with microvasculitis in late phase. Full-field electroretinography (ERG) and multifocal ERG showed decreased amplitude; thus, acute zonal occult outer retinopathy (AZOOR) was considered as the diagnosis. Laboratory work-ups before steroid therapy revealed positive serology for active syphilis. One month after treatment with penicillin G (6 million international units per day for 14 days), best-corrected VA improved to 20/30, and restoration of the IS-OS junction was observed on OCT. CONCLUSIONS: Ocular syphilis may present as occult outer retinopathy resembling AZOOR, and serologic work-up is required to avoid misdiagnosis. In the present case, anatomical and functional recovery were obtained after antibiotic therapy.

A Case Report of Acute Zonal Occult Outer Retinopathy Showing Acute Idiopathic Blind Spot Enlargement

PURPOSE: To present a case of acute zonal occult outer retinopathy (AZOOR) with acute idiopathic blind spot enlargement (AIBSE) investigated using indocyanine green angiography (ICGA), multifocal electroretinography (multifocal ERG) and spectral domain-OCT (SD-OCT). CASE SUMMARY: A healthy 31-year-old female with photopsia and a five-day visual field defect in her left eye showed no abnormal findings in ocular examinations such as slit lamp and fundus examination, fluorescein angiography (FAG), or full field ERG as well as in the systemic neurologic examination. In the late phase of ICGA, there were multiple hypofluorescent spots around the optic disc and scattered through the posterior pole. The patient showed an amplitude decrease of multifocal ERG and the destruction of the boundary between the inner and outer segments of the photoreceptors in the SD-OCT examined in the retinal area corresponding to the visual field defect. The patient was diagnosed with AZOOR and was followed-up without treatment. After three months, no abnormal SD-OCT or visual field test findings were observed and no symptoms remained; however, a slightly depressed response on multifocal ERG was present. CONCLUSIONS: ICGA, multifocal ERG and SD-OCT could be useful methods to detect AZOOR with EBS.

Acute Zonal Occult Outer Retinopathy, Responsive to an Immunosuppressive Agent: A Case Report

PURPOSE: To report a case of acute zonal occult outer retinopathy (AZOOR), initially misdiagnosed as retrobulbar optic neuritis, which was responsive to an immunosuppressive agent. CASE SUMMARY: A 28-year-old female with photopsia and a visual field defect in the left eye was referred to a hospital. There were no fundus abnormalities to explain her left visual field defect. Neurologic examination and brain MRI were normal. The patient was diagnosed with retrobulbar optic neuritis and treated with high-dose steroids. Four months after the onset of symptoms, she visited our hospital. Visual acuity was hand motion in the left eye. No relative afferent pupillary defect in the left eye and no anterior segment or fundus abnormalities were observed. A visual field examination of the right eye was normal and revealed field defect in the left eye. No abnormality was noted in the visual evoked potential test or fluorescein angiography. All examinations of the right eye were normal. In the left eye, fundus autofluorescence showed a hyperautofluorescent spot at the posterior pole, there was a decreased response in electroretinography and spectral domain optic coherence tomography showed that the junction between the photoreceptor inner and outer segments (IS/OS) was faintly visible only in the fovea. With the presumptive diagnosis of AZOOR, the patient was treated with an immunosuppressive agent. Visual acuity improved to 20/80 in the left eye at 10 months after the onset of symptoms. CONCLUSIONS: Electroretinogaphy is essential to diagnose AZOOR in patients with photopsia, visual field defect and minimal or no fundus changes, especially in a young women. There is currently no proven standard treatment, however immunosuppressive agents may be helpful.

Acute Zonal Occult Outer Retinopathy Mimicking Retrobulbar Optic Neuritis

A 27-year-old woman suffered a sudden visual disturbance in the right eye. The right eye had a visual acuity of 0.02 and a relative afferent pupillary defect. A visual-field examination revealed an inferotemporal field defect in the right eye. A fundus examination revealed no abnormalities that were responsible for the visual-field defect. Multifocal electroretinography showed decreased responses in the right eye at the corresponding area with the visual-field defect. She was diagnosed as acute zonal occult outer retinopathy.

A Case Report of Acute Zonal Occult Outer Retinopathy

PURPOSE: We present a case of acute zonal occult outer retinopathy (AZOOR) that has not been reported in Korea. METHODS: A 26-year-old female with photopsia and visual field defect in the left eye was referred to our hospital. There were no fundus abnormalities to explain the visual field defect. Electroretinography, indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging were performed for further evaluation. RESULTS: Visual acuity was 20/20 in both eyes. Visual field examination revealed a peripheral scotoma in the right eye and marked constriction of visual field in the left eye. Electroretinography showed normal response in the right eye and no response in the left eye. There was an afferent pupillary defect in the left eye and vitreous cells in both eyes. Indocyanine green angiography, neurologic examination, cerebrospinal fluid tapping and brain magnetic resonance imaging showed no abnormal findings. CONCLUSIONS: The possibility of AZOOR should be considered in a patient with photopsia, visual field defect, acute loss of outer retinal function confirmed with electroretinography, and minimal or no fundus and angiographic changes, especially in a young woman.