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.
Polypoidal choroidal vasculopathy (PCV) is a fundus disease characterized by choroidal anomalous branch vascular network and terminal polypoidal dilatation. According to its fundus feature, lesion location, imaging feature and disease progression, PCV can be divided into different types or stages. It can be divided into hemorrhage and exudation PCV according to the fundus features, into macular, peripapillary, periphery and mixed types according to the lesion locations. It can also be divided into type 1 and 2 according to the ICGA (indocyanine green angiography) manifestations, and can be classified as early stage and late stage according to disease progression. There were different correlations between different types of PCV and some risk genetic loci, such as ARMS2 (age-related macular degeneration factor 2)/ HTRA1 (high temperature essential protein A1) , C2, complement factor B, complement factor H, and elastin genes. The response to therapy and prognosis are also different between different types. It is important to further study the clinical classification of PCV, to explore the genetic characteristics, influencing factors and treatment or prognosis features of different types of PCV. The results will improve the differential diagnosis of PCV, and the effectiveness of individualized treatment.
Objective To cpmpare the assessment of retinal and choroidal disease using confocal scanning laser ophthalmoscope (cSLO) imaging and color fundus camera. Methods Sixty-seven patients (90 eyes) with fundus diseases were included in this study. There were 35 males (51 eyes) and 32 female (39 eyes), mean age was 51.32 years. All subjects underwent fundus imaging using cSLO technology and traditional color fundus camera, positive numbers of every retinal pathological change were calculated and compared. Spectral domain-optical coherence tomography (SD-OCT) was also done to compare the accordance rate between two modes of fundus imaging (cSLO technology and traditional color fundus camera) and SD-OCT in choroidal changes. Results The positive numbers of retinal microaneurysm (χ2=4.157, P < 0.05) and epiretinal membrane (χ2=5.428, P < 0.05) using cSLO fundus imaging were significantly higher than traditional color fundus camera, while the positive numbers of cotton wool spots (χ2=0.523), retinal hemorrhage (χ2=0.117), hard exudates (χ2=0.325) and macular hole (χ2=0.070) were no significant different (P > 0.05). The SD-OCT accordance rate of choroidal pathological changes using cSLO technology was higher than traditional color fundus camera (χ2=9.143, P=0.007). Conclusion In retinal and choroidal diseases, the imaging quality of cSLO fundus imaging technology is better than the traditional color fundus camera technology.
Ultra-wide field fundus autofluorescence (FAF) imaging is a new noninvasive technique with an imaging range of about 200 °. It can detect peripheral retinal lesions that cannot be found in previous FAFs and more objectively reflect intracellular content and distribution of lipofuscin in the retinal pigment epithelium (RPE) and RPE cell metabolic status. The ultra-wide field FAF can find the abnormal autofluorescence (AF) in the peripheral retina of the eyes of age-related macular degeneration (AMD), and different AF manifestations may have an impact on the diagnosis and treatment of the different AMD subtypes. It is helpful to evaluate subretinal fluid in the eyes of central serous choroidal retinopathy and can accurately detect the changes in the outer retina of the eyes without subretinal fluid. It can help to determine the type of uveitis and fully display the evolution of the disease. It can also assess the peripheral photoreceptor cell layer and RPE in patients with retinal dystrophy and retinitis pigmentosa, and comprehensively evaluate their retinal function and monitor the progress of disease. It can also assist in the evaluation of the short-term efficacy and RPE cell function after the scleral buckling surgery for patients with rhegmatogenous retinal detachment. In the future, ultra-wide field FAF may change the knowledge and intervention strategy of ocular fundus diseases and promote the clinical and scientific research in this field.
Optical coherence tomography angiography (OCTA) is a new and noninvasive imaging technique that generates real-time blood flow pattern on chorioretinal vasculature. In order to apply this novel technology in the practice to diagnose and treat ocular fundus diseases, we need to further strengthen the quality of OCTA image acquisition and reporting specifications. We need to understand its technical principle, and multiple factors affecting the OCTA image acquisition and interpretation. Furthermore, In the process of image acquisition, as well as analysis and interpretation, we need to pay attention to the stratification, interpretation of blood flow signals and identification of artifacts of OCTA images.
Objective To observe the clinical features, phenotypes and genotypes in a Chinese family with choroideremia (CHM). Methods A Chinese four-generation family (15 members) with CHM, including 5 patients (4 males/1 female), 2 female carriers and 8 healthy members, was enrolled in this study. Initially all family members underwent best corrected visual acuity (BCVA), indirect ophthalmoscopy, fundus fluorescein angiography, optical coherence tomography (OCT), visual field and full view electroretinogram (ERG). BCVA was followed up for 3 years. Venous blood samples were collected, and all of the 15 coding exons and flanking intron regions were amplified in the proband by polymerase chain reaction followed by direct sequencing. Protein structure was modeled based on the protein data bank and mutations in DeepView v4.0.1 to predict the effect of the mutations. A total of 180 healthy volunteers were enrolled as control group to matching CHM gene sequences. Results The visual acuity (VA) of 3/4 adult male patients began to decrease at less than 10, 10 and 30 years old, the average BCVA was 0.43. There were characteristic signs and symptoms of CHM including narrow visual field, extinguished rod and cone response in ERG, disappeared junction line and intermediate line of photoreceptor inner segment/outer segment on OCT. After 3 years, the mean BCVA decreased to 0.11. The BCVA of one young male patient was 1.0 in both eyes with minor changes fundus and visual field. The VA of the female patient began to decrease at 50 years old, her BCVA of two eyes were 0.5 and 0.25, respectively. The fundus changes were typical of CHM, with relative scotomas in the peripheral visual field of OD, and big scotomas in the OS. After 3 years, her mean BCVA decreased to 0.2. Of 2 female carriers, one had minor fundus changes (patches of pigmentary deposits, atrophy spots of retinal pigment epithelium cells), and the other was normal. A novel heterozygous c.1837G＞A mutation in exon 15 of CHM was detected in the proband, which resulted in the substitution of serine by proline at codon 613 (p.D613N). Based on molecular modeling, the misfolded protein caused by the mutation might destabilize the structure of the helix that potentially could affect the global stability of the Rep-1 protein. Conclusions A novel c.1837G＞A (p.D613N) mutation may be the causative mutation for CHM in this family. Female CHM carriers may have some signs and symptoms.
Optical coherence tomography angiography (OCTA) is a new diagnostic technique in recent years based on the optical coherence tomography. It is one of the fastest developing imaging examinations in ophthalmology. Compared with the classic diagnostic methods of fundus fluorescein angiography and indocyanine green angiography, OCTA show the ability to reveal blood flow non-invasively. With the development of modern medical detection technology, the requirement for ophthalmic diagnosis is raised, and many new measurement methods begin to apply in research and clinical, which makes the detection methods in the field of ophthalmology more accurate and comfortable. OCTA is a novel and noninvasive flow imaging technique, and it has the advantages of high resolution, fast scanning, as w ell as quantifying blood flow. Meanwhile, this technique can not only qualitatively analyze the shape of ocular blood vessels, but also be able to measure the ocular blood vessels and blood flow non-invasively, as well as to assess the depth of lesions. At present, with a wide clinical application in ophthalmology, OCTA still has its own superiority and weakness, but with the development of technology. It is believed that the OCTA will be expected to replace the relevant invasive examination methods and become a new tool for ophthalmic imaging.
Objective To observe the characteristics of spectraldomain optical coherence tomography (SD-OCT) and fundus autofluorescence (FAF) in acute and chronic central serous chorioretinopathy (CSC).Methods Seven-three eyes of 67 patients with CSC diagnosed by slit-lamp microscopy, fundus photochromy, fundus fluorescein angiography (FFA) and indocyanine green angiography were enrolled in this study. All the patients were examined for FAF and SD-OCT. The patients were divided into acute CSC group (37 patients, 37 eyes) and chronic CSC group (30 patients,36 eyes) according to the clinical features and FFA images. According to the OCT feature in retinal detachment area, they were divided into three categories, which including intact, non-intact and atrophy outer segment, respectively. According to the FAF characteristics, they were divided into hyper-FAF, hypo-FAF and mixed type, respectively. The characteristics of SD-OCT and FAF of both acute and chronic CSC patients were evaluated and analyzed. Results In acute CSC group, 19 eyes (51.35%) were hypo-FAF, 18 eyes (48.65%) were hyper-FAF. In chronic CSC group, two eyes (5.56%) were hypo-FAF, 16 eyes (44.44%) were hyper-FAF, and 18 eyes (50.00%) were mixed type. There was significant difference between both groups (chi;2=31.872,P=0.000). The SD-OCT results showed that in acute group, 15 eyes (40.54%) were intact outer segment, 18 eyes (48.65%) were non-intact outer segment, and four eyes (10.81%) were atrophy outer segment. In chronic group, five eyes (13.89%) were intact outer segment, 17 eyes (47.22%) were non-intact outer segment, and 14 eyes (38.89%) were atrophy outer segment. There was significant difference between both groups (chi;2=10.572,P=0.005). Conclusions The FAF characteristics of acute and chronic CSC mainly manifests hypo-FAF and mixed type, respectively. The OCT characteristics of acute CSC mainly manifests intact outer segment and non-intact outer segment, but non-intact outer segment and atrophy outer segment in chronic CSC.
Objective To investigate the characteristics of indocyanine green angiography(ICGA) in central serous chorioretinopathy(CSC) Methods The simultanous fundus fluorescein angiography(FFA) and ICGA were performed on 79 eyes of 70 consecutive patients with Heidelberg Retina Angiography. Results Seventy-nine eyes in FFA revealed RPE leakages.The changes of ICGA showed a small localized delay of filling of choroid vessels during the early phase of angiography in 23 eyes,choroidal capillary congestion in 79 eyes,the choriodal capillary hyperpermeability in the area of RPE leakage in 78 eyes,pigment epithelial detachment in 25 eyes and RPE atrophy in 21 eyes． Conclusion The findings in this research indicate that the choroidal abnormalities are the basic characteristics of ICGA in CSC. (Chin J Ocul Fundus Dis,20000,16:14-16)
ObjectiveTo observe the imaging features of branching vascular network (BVN) in polypoidal choroidal vasculopathy (PCV). MethodsEighty PCV patients (90 eyes) were enrolled in this study. The patients included 58 males and 22 females. The age was ranged from 49 to 85 years, with a mean age of 61.4 years. All the patients were examined for fundus photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT). The fibrovascular retinal pigment epithelium detachment (PED) was defined as a well-demarcated subretinal heterogeneous plaque with increasing fluorescence on FFA. The late lichenoid hyperfluorescent plaque was defined as a well-demarcated lichenoid hyperfluorescent plaque on late phase ICGA. The double-layer sign on OCT was defined as a wide range of shallow PED from Bruch membrane. ResultsBVN were found on early ICGA in 76 eyes among the 90 eyes (84.4%). Among these 76 eyes, 18 eyes (23.7%) demonstrated the subretinal reddish-orange branches corresponding to BVN. Fifty-six eyes (73.7%) demonstrated all or part of the BVN on early FFA. Three eyes (3.9%) demonstrated branching transmitted fluorescence corresponding to BVN throughout the FFA. Seventy-three eyes (96.1%) were manifested by occult choroidal vascularization on FFA, and 21 eyes (27.6%) of them were fibrovascular PED. Among the 76 eyes with BVN, all BVN appeared earlier than polypoidal lesions on ICGA. Polypoidal lesions located on the terminal of BVN in 62 eyes (81.6%). Sixty-nine eyes (90.8%) on ICGA demonstrated the late lichenoid hyperfluorescent plaque, whose area was equal to or greater than the area of BVN shown on early ICGA. Seventy-two eyes (94.7%) had the double-layer sign. Among these 72 eyes, 15 eyes (20.8%) had lumen-like structure within the double-layer sign. Sixty-five eyes (90.3%) had punctate and linear hyper-reflectance within the double-layer sign. Two eyes (2.8%) demonstrated a hyporeflective short segment and a gap of Bruch membrane on OCT corresponding to the origin of the BVN. Sixty-three eyes (87.5%) had an area of double-layer sign that matched the area of late lichenoid hyperfluorescent plaque on ICGA. ConclusionsBVN in PCV can be noted as reddish-orange branches on fundus examination. Most of the BVN are shown as early branching transmitted fluorescence but collectively an occult choroidal vascularization on FFA, as lichenoid hyperfluorescent plaque on late ICGA, and as double-layer sign on OCT whose area matches late lichenoid hyperfluorescent plaque.