Acute zonal occult outer retinopathy (AZOOR) is an acquired retinal diseases. The majority of patients who develop AZOOR are women characterized by an acute onset of visual blurred and scotoma with photopsias. The fundus examination is often normal or appeared mild abnormal. The RPE atrophy of fundus is similar with white syndrome. Although FFA and ICGA features are either unremarkable or unrelated to AZOOR, there are still important in differential diagnosis. The characteristic abnormalities appearance of FAF (complicated and varied), OCT (regional anomaly of ellipsoid zone), visual field (visual field defect) and ERG (decreased amplitude and prolonged latency of rod reaction, maximum reaction, cone reaction and scintillation reaction) are considered critical examinations to the diagnosis of AZOOR. Although there is no effective therapy for AZOOR, it has some self-limitation.
Retinal vein occlusion (RVO) is one of the most common retinal vascular diseases causing blindness, macular edema (ME) is often secondary to it, which causes serious visual impairment to patients. Imaging biomarkers in the changes of retina and choroid of ME secondary to RVO (RVO-ME) have important clinical value in the evaluation of condition, curative effect and visual acuity prediction of patients with RVO-ME. Among them, the disorganization of the retinal inner layers, the integrity of external limiting membrane and ellipsoid zone, and the change of central macular thickness are reliable indexes to evaluate the prognosis of visual acuity; hyperreflective foci, subretinal fluid and intraretinal fluid can be used as important parameters to reflect the level of inflammation; prominent middle limiting membrane and paracentral acute middle maculopathy are the objective basis for judging the degree of retinal ischemia; the changes of choroidal vascular index and choroidal thickness also have potential advantages in evaluating the progress of the disease. Accurately grasp the characteristics of biological markers of RVO-ME related optical coherence tomography is conducive to its reasonable and accurate use in the clinical diagnosis and treatment of RVO-ME, and helpful to further explore the pathogenesis of the disease.
ObjectiveTo observe the clinical features of nine foveal hypoplasia (FVH) patients in a family. MethodsA retrospective clinical study. In August 2018, nine patients with FVH from a family diagnosed in Qilu Hospital of Shandong University (Qingdao) were included in this study. Detailed medical history of the proband was collected. Best corrected visual acuity (BCVA), slit-lamp, cycloplegic refraction, fundus color photography, optical coherence tomography (OCT) and OCT angiography (OCTA) were performed on the proband. The peripheral venous blood of V7 (family member), the proband and the proband's parents were collected for DNA extraction, and gene detection was performed. ResultsThe proband, a four-year-old girl, had poor vision with BCVA of 0.4 in both eyes. OCT showed absence of foveal pit, absence of outer segment lengthening, absence of outer nuclear layer widening and incursion of inner retinal layers. The proband's mother was 32 years old, and macular foveal reflection was not observed in her eyes. OCT and OCTA examination showed no foveal pit and foveal avascular zone in both eyes. Both eyes of the proband and her mother were diagnosed with Thomas grade 4 FVH. The other seven patients also had no foveal pit, and could be categorized into Thomas grade 3 or 4. No significant pathogenic genes and mutation sites were detected in the proband through whole genome sequencing, and no copy number variation or chromosomal abnormality associated with the phenotype of the proband was detected. After seven months of amblyopia treatment, the proband's BCVA had improved to 0.5 in the right eye and 0.6 in the left eye, while the BCVA did not change after 2 years of follow-up. ConclusionNine FVH patients in this family had no foveal pit with similar OCT images, and their visual acuity was affected from lightly to severely. Early amblyopia training is helpful to improve the visual acuity of child patients.
Peripapillary intrachoroidal cavitation (PICC) is a common pathological change observed in high myopia. The exact pathogenesis of PICC is still unclear. Expansion and mechanical stretching of the peripapillary sclera, breakage and defect in the retina near the border of the myopic conus and communication between intrachoroidal cavity and the vitreous space may be important segments during the development of PICC. Color fundus photography shows a localized and well-circumscribed peripapillary lesion with yellow-orange colour, often accompanied by fundus changes, such as myopic conus excavation, optic disc tilting and inferotemporal retinal vein bending at the transition from the PICC to the myopic conus. However, the PICC lesion is not easy to be recognized in the fundus photography. Fluorescein angiography shows early hypofluorescence and later progressively staining in the lesion. Indocyanine green angiography shows hypofluorescence throughout the examination. Optical coherence tomography (OCT) is vital in diagnosing PICC. Hyporeflective cavities inside the choroid, sometimes communicating with the vitreous chamber, can be observed in OCT images. OCT angiography indicates lower vessel density or even absence of choriocapillary network inside or around PICC lesions.
Objective To observe multimodality imaging features of different properties in multifocal choroiditis (MFC). Methods Twenty-eight patients (51 eyes) with MFC were enrolled in this study. There were 10 males and 18 females. The patients aged from 31 to 49 years, with the mean age of (41.5±0.8) years. There were 23 bilateral patients and 5 unilateral patients. All patients underwent best corrected visual acuity (BCVA), slit-lamp biomicroscopy, indirect ophthalmoscopy, fundus colorized photography, infrared fundus photography, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA) and optical coherence tomography (OCT) examinations. The lesions were classified as active inflammatory lesion, inactive inflammatory lesion, active choroidal neovascularization (CNV) and inactive CNV. The multimodality imaging features of different properties in MFC was observed. Results In fundus colour photography, the boundaries of active inflammatory lesions were blurry, while inactive inflammatory lesions had relatively clear boundaries. Secondary active CNV showed mild uplift and surrounding retinal edema; Secondary active CNV lesions showed mild uplift, retinal edema around the lesion; Secondary non-active CNV had no retinal exudate edema lesions, but had lesions fibrosis and varying degrees of pigmentation. Infrared fundus examination revealed that both active and inactive inflammatory lesions showed a uniform punctate or sheet-like fluorescence. The fluorescence of CNV lesions was not uniform; there was a bright ring around the strong fluorescence. FAF found that active inflammatory lesions showed weak autofluorescence (AF), surrounded by a strong fluorescence ring; inactive inflammatory lesions showed AF loss. Secondary active CNV lesions showed strong AF with a bright ring along the edge, and obscured fluorescence for co-occurred hemorrhagic edema; secondary non-active CNV lesions were strong AF, surrounded by a weak AF ring. FFA revealed that active inflammatory lesions showed weak fluorescence in the early stage, and fluorescence gradually increased in the late stage with slight leakage. Inactive inflammatory lesions showed typical transmitted fluorescence. Fluorescein leakage secondary to active CNV was significant; lesions secondary to inactive CNV showed scar staining. In OCT, the active inflammatory lesions showed moderately weak reflex signals in the protruding lesions under the retinal pigment epithelium (RPE). The inactive inflammatory lesions showed penetrable RPE defects or choroidal scar, it also showed clear RPE uplift lesions with a strong reflection signal. Secondary active CNV showed subretinal fluid retention; secondary non-active CNV showed RPE defects and choroidal scarring. Conclusions Active inflammatory lesions in MFC have blurred boundary, retinal edema and fluorescein leakage in FFA; inactive inflammatory lesions have clear boundary and typical transmitted fluorescence in FFA, and no retinal edema. Secondary active CNV showed subretinal fluid in OCT; and secondary non-active CNV showed RPE defects and choroidal scarring.
ObjectiveTo determine the retinal nerve fiber layer thickness (RNFLT) of chronic progressive external ophthalmoplegia (CPEO) and kearns-sayre syndrome (KSS) patients using spectral-domain optical coherence tomography (SD-OCT) and to analyze the potential influence factors for RNFLT of these patients. Methods18 CPEO and 4 KSS (CPEO with retinitis pigmentosa and cardiac block) patients, all were muscle biopsy confirmed, were included in this study. There were 7 males and 15 females, the average age was (29.09±13.40) years, the average onset age was (16.4±10.7) years and the average disease duration was (11.30±7.30) years. All the patients underwent SD-OCT examination for the left eye, the peripapillary RNFL thickness was measured using the Spectralis 3.45 mm circle scan protocol. 8 quadrants were scanned including superior temporal (ST), temporal upper (TU), temporal lower (TL), inferior temporal (IT), inferior nasal (IN), nasal lower (NL), nasal upper (NU) and superior nasal (SN). The relationship between RNFLT and onset time, disease duration was analyzed by Pearson correlation analysis. ResultsThe average RNFLT of ST, TU, TL, IT, IN, NL, NU, SN in the 22 patients were (136.3±24.1), (85.4±25.7), (68.2±11.7), (128.2±28.7), (127.3±29.5), (66.7±16.8), (70.1±17.6) μm, respectively. The circumferential average RNFLT was (101.5±14.4) μm. There was no significant difference between the KSS group and CPEO group (P>0.05). The decrease of the circumferential RNFLT had no relationship with the onset age (r=-0.306, P=0.11), but a negative relationship with the disease course (r=-0.518, P=0.03). There were negative relationships between the disease course and RNFLT of 4 nasal quadrants (IN:r=-0.555, P=0.01, NL: r=-0.630, P=0.00, NU: r=-0.559, P=0.01, SN: r=-0.557, P=0.01). ConclusionThere is no difference in RNFLT of patients with CPEO and KSS. There is a negative relation between RNFLT (especially RNFLT of 4 nasal quadrants) and disease course.
ObjectiveTo evaluate macular microvessel changes in familial exudative vitreoretinopathy (FEVR) by optical coherence tomography angiography. MethodsCross-sectional clinical case-control study. From November 2019 to November 2020, 21 FEVR patients (41 eyes) from Weifang Eye Hospital were selected; 17 healthy volunteers (28 eyes) with the same age and gender as FEVR group were selected as normal control group. According to the best corrected visual acuity (BCVA) 1.0 and ＜1.0, FEVR group was divided into normal visual acuity group (27 eyes) and visual acuity decreased group (14 eyes). All enrollees received BCVA and OCTA. BCVA was performed with an international standard visual acuity chart, which was converted to logarithm of the minimum angle of resolution (logMAR) vision. The OCTA instrument was used to scan the macular area of all the examined eyes in the range of 3 mm×3 mm, 6 mm×6 mm, and the blood vessel density (VD) and blood perfusion density (PD) within the range of 3 mm×3 mm, 6 mm×6 mm were measured and the area, circumference, and morphological index of the foveal avascular zone (FAZ) within the range of 6 mm×6 mm. Quantitative data were compared between groups by independent sample t test. Statistical data were compared by χ2 test. The area under curve (AUC) of each index was determined according to receiver operating characteristic curve (ROC curve), and the predictive value of each index was evaluated. ResultsIn the macular area of 6 mm×6 mm, VD, PD, FAZ area and FAZ perimeter of FEVR group were all lower than those of normal control group, and the differences were statistically significant (t=−3.350, −2.387, −3.519, −3.029; P＜0.05). In macular area of 3 mm×3 mm and 6 mm×6 mm, compared with normal vision group and vision loss group, both VD and PD decreased. The differences were statistically significant (t=2.088, 2.114, 2.160, 2.545; P＜0.05). In the macular area of 6 mm×6 mm , the FAZ morphological index of the two groups was significantly different (t=2.409, P＜0.05). ROC curve analysis showed that all the indicators had low diagnostic value for FEVR (AUC＜0.5). ConclusionThere are microvascular abnormalities in macular area in FEVR patients, and the decrease of blood vessels and the change of FAZ shape may be related to the loss of visual acuity.
OCT angiography (OCTA) is a fast, noninvasive and quantifiable new technique, which is especially suitable for long-term follow-up in patients with hereditary retinochoroidal degeneration, such as retinitis pigmentosa, Best vitelliform macular dystrophy, adult onset foveomacular vitelliform dystrophy, doyne honeycomb retinal dystrophy, choroideremia and Stargardt disease. During the follow-up, clinicians can find the subtle signs that explain disease development from the blood flow imaging, quantitatively describe the vascular density, timely detect and treat choroidal neovascularization. It is significant to explore the etiology and monitor the course of these diseases. With the development of more treatments for these diseases, OCTA parameters can also be used as indicators to evaluate and compare different therapeutic effects. In the future, more quantitative indicators of OCTA will be applied to evaluate the course of hereditary retinochoroidal degeneration, and provide valuable basis for early diagnosis and treatment.
ObjectiveTo observe the clinical evolution process and imaging characteristics of choroidal lesions in different subtypes of serpiginous choroiditis (SC), and to explore the clinical significance of subtype classification. MethodsA retrospective, uncontrolled and observational study. A total of 45 eyes of 25 SC patients diagnosed in Yunnan Eye Hospital from May 2009 to September 2021 were included in the study. According to the initial location of the lesion and fundus images, including fundus color photography, fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and other examination results. SC was divided into peripapillary serpiginous choroiditis, macular serpiginous choroiditis and ampiginous choroiditis. According to the shape of the lesions at the first diagnosis, it can be divided into new lesions with only infiltrating edema, old lesions with only atrophy and recurrent lesions with coexistence of edema and atrophy. the imaging features, development and complications of different subtypes of ocular lesion were observed. ResultsAmong the 45 eyes of 25 cases, 15 cases were male and 10 cases were female, 20 cases of binocular and 5 cases of monocular, age was 42.3±5.7 years old. There were 21 eyes with active lesions, of which 5 eyes were new lesions and 16 eyes with recurrent lesions; 24 eyes were old lesions. Concurrent optic disc edema occurred in 3 eyes; mild vitreitis occurred in 5 eyes; retinal occurred vasculitis in 3 eyes; choroidal neovascularization occurred in 3 eyes. Among the 16 cases (64%, 16/25) of the peripapillary serpiginous choroiditis, 2 cases (2 eyes) were monocular, and 14 cases (28 eyes) were binocular. Active lesions were found in 16 eyes, of which patients with binocular lesions only one had active lesions. The choroidal lesions that were close to the optic disc or around the optic disc, expanded outwards centrifugally with the prolongation of the disease course, and can progress to the macula. The edge of the lesion was tortuous, with a geographic-like, amoeboid-like and finger-like, polypoid or propeller-like shape. Active lesions in FFA showed weak fluorescence in the early stage and strong fluorescence in the late stage; the old lesions showed weak fluorescence in the early stage and mottled fluorescence in the late stage, and mostly strong fluorescence on the edge. OCT showed thickening of active lesions and thinning of old lesions. Among the 4 cases (16.0%, 4/25) of macular type, 2 cases (2 monocular eyes) had active lesions; 2 cases (4 eyes) had lesion in both eyes, among them, 1 case (2 eyes) had old lesion, and the other case had alternate active lesions. The initial lesions were all located in the off-center of the macula, and most of them were disk-shaped and progressing centrifugally to the periphery. The FFA and OCT imaging findings of the lesions were similar to those of the peridisc type. Among the 5 cases (20.0%, 5/25) of ampiginous choroiditis, 1 case (1 eye) was monocular and 4 cases (8 eyes) were binocular. These lesions were multiple old lesions of varying sizes, gray-white with pigmentation, with clear borders in the posterior pole. Among them 4 eyes have new active lesions appeared near the old lesions. The old lesions showed weak fluorescence with clear borders, and the fluorescein leakage at the late edge formed a strong fluorescence ring; the active lesions showed weak fluorescent spots with blurred edges, and the fluorescence was slightly enhanced in the late stage. In old lesions, atrophy of the photoreceptor layer, RPE and choroid can be seen, and RPE hyperplasia in some areas. ConclusionsSC subtype is a classification of the location of the first lesion, but the characteristics of the repeated attack of this disease can lead to the annihilation of each subtype due to the continuous expansion of the lesion. The phenomenon that the fundus active lesions only occur in one eye that can explain the clinical manifestations of asymmetric morphology of binocular lesions. The characteristics of binocular subtype warn that the predilection site of the healthy eye should be paid attention to.
ObjectiveTo observe the characteristics of macular optical coherence tomography (OCT) changes before and after silicone oil removal in patients who had undergone pars plana vitrectomy with silicone oil tamponade for macula-off rhegmatogenous retinal detachment (RRD). MethodsThirty-nine eyes that underwent silicone oil removal were enrolled in this retrospective study. The patients included 24 males and 15 females, with an average age of (53.05±4.03) years, the duration of silicone oil tamponade ranged from 3 to 7 months. Best-corrected visual acuity, intraocular pressure, slit lamp microscope and pre-lens, indirect ophthalmoscopy and fourier domain OCT were measured for all patients before and at months 1, 3 and 6 after silicone oil removal. The macular microstructure were observed before and after silicone oil removal. ResultsSubmacular fluid was detected in 6 eyes (15.38%), at the last time of follow-up, submacular fluid resolved completely in 2 eyes with disrupted ellipsoid zone, and resolved partly in 2 eyes. Disrupted ellipsoid zone were observed before silicone oil removal in 16 eyes (41.02%), 6 eyes showed simultaneous disrupted ellipsoid zone and disrupted external limiting membrane, and there were 2 eyes that external limiting membrane was not identified, at the last time of follow-up, disrupted ellipsoid zone restored in 2 eyes and the extent of disrupted ellipsoid zone became reduced in 4 eyes. Cystoids macular edema were found in 2 eyes (5.12%), it resolved completely in 1 eye and resolved partly in 1 eye at the last time of follow-up. Macular epiretinal membrane was detected in 10 eyes (25.64%), and macular epiretinal membrane was found before silicone oil removal in 5 eyes, at the last time of follow-up, the membrane became thickened in 2 eye; 5 eyes developed macular epiretinal membrane after silicone oil removal, at the last time of follow-up, the membrane became thickened in 1 eye. Secondary macular hole were noted in 2 eyes. Microcystic macular changes were observed in 9 eyes (23.07%), it was observed in 7 eyes before silicone oil removal, and was observed in 2 eyes after silicone oil removal, at the last time of follow-up, the cysts reduced in 1 eye. ConclusionSubmacular fluid, disrupted ellipsoid zone and microcystic macular are the main macular ultrastructural changes that developed in patients with RRD before and after silicone oil removal.