The pathogenesis of polypoidal choroidal vasculopathy (PCV) is still controversial. More evidence of clinical and basic research is needed to distinguish PCV from an independent disease to a subtype of age-related macular degeneration. Not only that, there are also many puzzles in the diagnosis, treatment options and prognosis of PCV. In addition to these common problems, we also face a large population with risk factors, a large number of PCV patients with multiple and complex challenges in China. There is a long way to go to reduce the damage effects of PCV on visual function. To fulfil this goal, we need make full use of the huge resources of PCV patients and turn these challenges into opportunities, and contribute the improvement of diagnosis and better understanding of PCV pathogenesis.
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.
Purpose To define the morphometric characteristic s and the implication of simultaneous fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) in traumatic choroidal rupture. Methods Simultaneous FFA and ICGA were carried out in 17 patient s (17 eyes) with traumatic choroidal rupture. Results Choroidal ruptures were shown as hyperfluorescence region in the early pha se of FFA,and as hyperfluorescence in the late phase of FFA but in ICGA were shown as hypofluorescence region in both early and late phases.The rupture regions in ICG A were longer than that in FFA in 5 patients (5 eyes).The rupture regions in 6 patients (6 eyes) with hemorrhage could be shown in ICGA,but couldn't be shown in FFA . Conclusion ICGA is helpful in diagnosing minor choroidal ruptures,in defining the extent of traumatic choroidal ruptures,and in further understanding the pathological changes of choroidal ruptures. (Chin J Ocul Fundus Dis, 2001,17:30-32)
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.
Objective To investigate the clinical characteristics of patients with polypoidal choroidal vasculopathy (PCV) from Central China . Methods This was a retrospective study, and 403 eyes of 362 patients diagnosed as PCV by ocular fundus photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT) were enrolled. The general clinical data, fundus manifestation and ocular fundus examinations were statistically analyzed. Results Three hundred and sixty-two cases included 249 males (68.8%) and 113 females (31.2%). Age ranged from 45 to 91 years old, and mean age was (64.81plusmn;9.31) years old. Bilateral lesions were observed in 41 patients (11.3%) and unilateral lesions were observed in 321 patients (88.7%)． In these 403 eyes, typical orangered lesions were observed in 162 eyes (40.2%); yellowishwhite exudate could be found in 185 eyes (45.9%); 268 eyes (66.5%) showed variable degrees of subretinal hemorrhage. Drusen was found in 23 eyes (5.7%), pigment proliferation in 20 eyes (5.0%) and fiber vascular scar in 96 eyes (23.8%). The lesions of 386 eyes (95.8%) located in macular region, 53 eyes (13.2%) in peripapillary area. Lesions presented multifoci in 67 eyes (16.6%). Three hundred and four eyes (75.4%) presented typical polypoidal lesions and 152 eyes (37.7%) with abnormal branching choroidal networks. Hemorrhagic retinal pigment epithelial detachments (PED) were found in 200 eyes (49.6%) and serous PED in 96 eyes (23.8%), both existed in 25 eyes（6.2%）. OCT showed 56 eyes (13.9%) presented cystoid dark chamber between the neurosensory retina and 109 eyes (27.0%) with double-layer sign formed by the separation of retinal pigment epithelium and Bruchprime;s membrane (27.0%). Two hundred and seventy-four eyes (68.0%) were found with conelike elevation beneath the RPE layer and 151 eyes (37.6%) with neurosensory detachment. Conclusions In Central China, the majority of PCV patients were male, unilateral. Most PCV lesions were located in the macula. Subretinal hemorrhage, polypoidal lesions and abnormal choroidal vascular networks were common in the PCV patients. Hemorrhagic PED presented a higher ratio than serous PED.
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.
With the rapid development of ophthalmic imaging methods, there are many ways of examination in the diagnosis and treatment of fundus diseases, such as FFA, ICGA, FAF, OCT and emerging blood vessels by OCT angiography in recent years. Multi-model image can understand the changes of anatomical structure and function of different levels and parts of the fundus from different aspects. A variety of imaging examinations are combined and complemented each other, which makes us have a further understanding of the location and pathological changes of many fundus diseases. But at the same time, the emergence of multi-modal images also brings a series of problems. How to standardize the use of multi-modal imaging platform to better serve the clinic is a problem that ophthalmologists need to understand.
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.
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.
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.