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.
Maculopathy caused by various fundus diseases in the late stage is a common cause of low vision. Medical technology is difficult to reverse the loss of macular function currently, so interventions that help improve the visual system, utilize residual visual function, and improve quality of life deserve attention. Damage to the fovea of the macula does not mean that the entire retinal function is impaired. There may be one or more retinal regions adjacent to the fovea that can serve as a fixation center. It is possible to form stable paracentral fixation, complete functional remodeling of the visual system, and effectively utilize residual visual function by taking appropriate training on these potential paracentral fixation points for most patients. In 2021, a clinical guideline has been published for low vision rehabilitation in China. In order to strengthen the precise management of diseases and develop a standard operating procedure for visual training specifically for patients with low vision due to macular disease, the National Clinical Research Center for Eye Diseases initiated and organized relevant domestic experts, utilizing the latest research experience at home and abroad, and through repeated discussions, this consensus (International Practice Guideline Registration Number: PREPARE-2023CN199) was formed as a reference for ophthalmologists, optometrists and rehabilitation physicians in their clinical research and practice.
Objective To observe the imaging features of ultra-wide field short wave fundus autofluorescence (SW-FAF) in eyes with multiple evanescent white dot syndrome (MEWDS), and analysis the correspondence to conventional images. Methods It was a retrospective case series study. Thirteen patients (14 eyes) diagnosed with MEWDS were enrolled. There were 12 females and 1 male, aged from 22 to 57 years, mean age was 34.5 years. All the eyes underwent fundus color photography, optical coherence tomography (OCT) and ultra-wide field autofluorescence (FAF). Simultaneous fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were performed in 6 eyes. The characteristic changes of SW-FAF in studied eyes were observed and compared with the images of FFA and ICGA. All the eyes were followed up every 1 to 2 weeks, with an average of 16.7 weeks. The characteristic images of SW-FAF and corresponding OCT were studied during follow up. Results MEWDS presented with numerous multiple hyper-autofluorescent spots, sized from 50-500 μm, with a vague boundary in ultra-wide field SW-FAF. These spots located mainly at the peripapillary area and the posterior pole with a confluent pattern. The lesions extended to the mid-peripheral retina as well and became more scattered. The distribution of the hyper-autofluorescent lesions in SW-FAF corresponded roughly to that of the greyish-white spots seen in color photograph and the hyper-fluorescent spots detected by FFA. It was consistent with the distribution of hypo-fluorescent spots in late-phase ICGA as well. But the number of the spot showed in FAF is much more than that in FFA, and slightly less than that in ICGA. The OCT scans through the hyper-autofluorescent lesions in SW-FAF showed impairment of outer retina. After the recovery, the hyper-autofluorescent spots disappeared with the outer retina structure repaired completely. Conclusions MEWDS presented with numerous multiple hyper-autofluorescent spots which located mainly at the peripapillary area in ultra-wide field SW-FAF. The distribution of the hyper-autofluorescent lesions in SW-FAF corresponded roughly to color photograph, FFA and ICGA in late-phase. The OCT scans through the hyper-autofluorescent lesions in SW-FAF showed impairment of outer retina.
Multiple evanescent white dot syndrome (MEWDS) is an acute retinal disease characterized by multifocal white spots in the fundus often seen in the unilateral eye. The lesions mainly involve the retinal pigment epithelium and the outer retinal structure. Typical ocular manifestations of MEWDS include grayish-white outer retinal spots with a clear borderline identified on the fundus, findings of hyper-autofluorescence in the early stage consistent with the spots identified on the fundus, and the optical coherence tomography manifestation of multifocal disruption of the ellipsoid zone. With the rapid development of multimodal imaging technology, some scholars found that these manifestations are not exclusive to MEWDS as some types of chorioretinopathy can also show MEWDS-like changes. The etiology of these diseases may be inflammation, infection, immunity, or tumor-related, misdiagnosed by masquerading as MEWDS. Here we summarized the clinical manifestations and imaging features of MEWDS and reviewed the fundus lesions changes that can be misdiagnosed as MEWDS.
Objective To observe the microperimetry performance of macular function in pathologic myopia patients. Methods The clinical data of 90 patients (142 eyes) with pathologic myopia were retrospectively analyzed. All patients were asked in details about history, and take examinations of best corrected visual acuity (BCVA), refractive dioptre, eye axis, fluorescent fundus angiography (FFA), indirect ophthalmoscopy and optical coherence tomography (OCT). According to the test results, patients were divided into non-pathological macular group (20 patients, 24 eyes) and pathological macular group (70 patients, 118 eyes). Retinal imaging and macular microperimetry were measure by MP-1 Microperimeter.The mean retinal sensitivities (MS) and fixation stability in the central 10deg;, fixation rate and fixation position in the central 2deg; and 4deg;were determined.Results The MS of pathological and non-pathological macular group were(16.39plusmn;2.12), (10.80plusmn;4.53) dB respectively, the difference was statistically significant(F=15.044,t=-9.314;P=0.000). Among 24 eyes of non-pathological macular group, fixation was stable in 19 eyes (79.17%), relative unstable in five eyes (20.83%); among 118 eyes of pathological macular group, fixation was stable in 45 eyes (38.14%), relative unstable in 52 eyes (44.07%), unstable in 21 eyes (17.79%), the difference was statistically significant(chi;2=13.56, P=0.000). The differences of 2 deg;and 4 deg;fixation rate between those two groups are statistically significant (F=5.773, 13.230; t=-4.110,-5.465;P=0.000) . Among 24 eyes of non-pathological macular group, center fixation occurred in 23 eyes (95.83%), weak center fixation occurred in one eye (4.17%); among 118 eyes of pathological macular group, fixation center occurred in 81 eyes (68.64%), weak center fixation occurred in 16 eyes (13.56%)，eccentric fixation occurred in 21 eyes (17.80%), the difference was statistically significant (F=9.618,t=-5.773；P=0.000).Conclusion Pathological myopia patients with pathological macular changes have decreased retinal sensitivity, decreased fixation stability and eccentric fixation points.
Objective To observe the fundus imaging characteristics of different stages of syphilitic posterior uveitis. Methods Retrospective cases series. Forty-six eyes of 32 patients with syphilitic posterior uveitis were included. There were 14 patients (16 eyes) and 18 patients (30 eyes) were assigned to acute stage group (with the course ＜2 months) and chronic stage group (with the course ≥2 months) respectively. All eyes received the examination of indirect ophthalmoscopy, color fundus photography, fundus fluorescein angiography (FFA) and optical coherence tomography (OCT). All patients received regular anti-syphilitic treatment. Color fundus photography and OCT were followed after treatment. The fundus imaging characteristics of different stages of syphilitic posterior uveitis were observed. Results Indirect ophthalmoscopy and fundus color photography showed that in the acute stage group, there were 3/16 eyes with optic disc edema; 4/16 eyes with a yellowish, placoid lesion involving the macular. There were only some pigment alterations on the fundus after treatment. In the chronic stage group, there were 4/30 eyes with optic disc hyperemia, 3/30 eyes with cystoid macular edema. After treatment, the optic hyperemia vanished gradually, but there were still some pigment alterations. The FFA images of two groups showed various vascular leakages. In the chronic stage group, patients also showed hyper-fluorescence with cystoid macular edema. The patients with course 2 – 3 years have more transmitted fluorescence on FFA. OCT showed that all eyes in the acute stage group had lost the ellipsoid zone, with irregular granular reflectivity of the retinal pigment epithelium (RPE) layer, 6 eyes with subretinal fluid in the macular. After treatment, the ellipsoid zone and RPE layer structure recovered gradually. In the chronic stage group, all eyes showed widespread loss of the ellipsoid zone, pigment migration and (or) cystoid macular edema. After treatment, the ellipsoid zone showed partial recovery. The outer ellipsoid zone was still discontinuous in patients with long duration. Conclusions Syphilitic posterior uveitis patients generally had normal fundus, but some cases had a yellowish, placoid lesion involving the macular. FFA showed various vascular leakages, and the chronic stage group showed more transmitted fluorescence. The major OCT change was loss of the ellipsoid zone or with subretinal fluid. After treatment, fundus showed no abnormal manifestations except some pigment alterations; the ellipsoid zone structure recovered gradually in acute stage eyes, partially recovered in chronic stage eyes.