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.
ObjectiveTo observe the clinical characterisitics of choroidal excavation in the macula. MethodsA total of 22 patients (22 eyes) with choroidal excavation diagnosed by spectral domain high definition optical coherence tomography (HD-OCT) were enrolled in this study. The patients included 12 males (54.50%) and 57 females (45.50%). The age was ranged from 21 to 82 years old, with an average of (41.44±13.17) years. All the patients were affected unilaterally, including 9 right eyes and 13 left eyes. The corrected vision, slit lamp microscope with preset lens, fundus photography, HD-OCT and fluorescence fundus angiography (FFA)were measured for all patients. The clinical characterisitics and concomitant diseases were observed. Seventeen eyes were followed for a period between 3 to 12 months. The lesions change were evaluated by HD-OCT. ResultsThere were 18 eyes (81.8%) with symptoms of micropsia and metamorphopsia, 4 eyes (18.2%) without symptoms. The corrected vision was ranged from 0.3 to 1.2, 12 eyes (54.54%) with moderate or high myopia. Fundus examination presents yellowish-white exudation in 12 eyes (54.54%), yellowish-white exudation accompanied with hemorrhage in 9 eyes (40.91%), grayish yellow reflex halo in 1 eye (4.55%). HD-OCT showed that the retinal pigment epithelium (RPE) layer was involved in the excavation, and the photoreceptor outer segment and pigment junction (OPR) layer was disappeared in all eyes. The external limiting membrane and the photoreceptor inner segment/outer segment junction layer were preserved in 13 eyes (59.09%) and disappeared in 9 eyes (40.91%). There were 10 eyes (18.18%) with a single lesion, 4 eyes (18.18%) with idiopathic choroidal neovascularization, 4 eyes (18.18%) with punctate inner choroidopathy, 1 eye (4.55%) with polypoidal choroidal vasculopathy, 1 eye (4.55%) with macular preretinal menbrance, 1 eye (4.55%) with central serous chorioretinopathy. FFA showed hypofluorescence in early phase, hyperfluorescence in late phase, without obvious leakage. There was no noticeable changes in size and morphological changes in the follow-up period. ConclusionsChoroidal excavation in the macula occurs mostly in middle-aged people with myopia. It can be associated with many fundus diseases. The excavation is located in RPE layer, and OPR layer disappeared. Choroidal excavation in the macula develops slowly.
Optic coherence tomography (OCT) is one of the most rapid developing technologies in ophthalmology. OCT angiography (OCTA) has been made possible by the development of even faster scanning and sampling techniques, which is the next milestone after stratus OCT and spectral domain OCT. Without the need of injection of the contrast agent, OCTA is capable of providing a three-dimensional reconstruction of the perfused microvasculature within the retina and choroid by detecting the motion of scattering particles such as erythrocytes within sequential OCT cross-sectional scans performed repeatedly at the same location of the eye with different analysis algorithms. Comparing to fundus fluorescein angiography and indocyanine green angiography, with improved OCT technology and understanding, OCTA has showed certain advantages to diagnose retinal and choroidal diseases, especially macular vascular diseases. It is important to establish the contributions that OCTA can make to diagnosing, managing and understanding of ocular fundus diseases.
ObjectiveTo observe the characteristics of indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA) in polypoidal choroidal vasculopathy (PCV). Methods17 patients (17 eyes) with PCV referred to Peking Union Medical College Hospital from November 2014 to February 2015 were included in this cross-sectional study. There were 9 males (9 eyes) and 8 females (8 eyes), aged from 55 to 79 years, with the mean of (68.24±6.80) years. There were 10 right eyes and 7 left eyes. All patients were examined by fundus fluorescein angiography combined with ICGA, and OCTA was performed within 1 hour. ResultsICGA showed 5 eyes with branching vascular network (BVN), 7 eyes with polyps, only 1 eye with both BVN and polyps. 4 eyes showed no positive findings, 3 of them with large hemorrhage. 5 eyes with BVN shared the similar location and range of the lesions in ICGA and OCTA. 7 eyes with polyps showed hot spot in OCTA, 5 of them shared the similar lesions with ICGA, the other 2 eyes showed slightly different in ICGA and OCTA. 1 eye showed both BVN and polyps, OCTA and ICGA were consistent for this. In the 3 eyes with large hemorrhage, 2 of them showed hot spot below pigment epithelial detachment, 1 eye show no positive findings in both ICGA and OCTA. ConclusionsPCV patients with BVN shared similar findings in ICGA and OCTA, PCV patients with polyps showed highlight spot in OCTA. OCTA can visualize BNV and polyps of choroidal capillary, and it can showed the similar site and range of lesions in ICGA.
Multicolor imaging (MCI) based on confocal scanning laser ophthalmoscopy can gather more diagnostic information than traditional fundus photographs through utilizing three wavelengths of laser to scan posterior retina, which gain different layer reflected signal since the depth of penetration into retina is different for each wavelength. Currently, it provides important information and reference value for diagnose of different layer diseases on retina or choroid combining MCI with OCT, FAF, FFA and so on. However, there are still misunderstandings in the diagnosis of retinal diseases with MCI. Careful observation of retinal details in MCI, CFP and other imaging methods is more conducive to the correct diagnosis of fundus ophthalmopathy.
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.