Background:Leber’s congenital amaurosis (LCA) is a severe hereditary retinopathy disease that is characterized by early and severe reduction of vision,nystagmus,sluggish or absent pupillary responses. To date, the pathogenesis of LCA remains unclear,and the majority cases are caused by autosomal recessive inheritance. In this study, we explored the mutation in the Crumbs homologue 1(CRB1) gene in a Chinese family with LCA. Methods:We conducted comprehensive ocular examinations and collected 5 ml of blood samples from members of a Chinese family with LCA. The pathogenic gene was identified by capturing and sequencing the related genes of ocular diseases.Results: We found a nonsense mutation(c.1499C>G) in the 6th exon of CRB1 in a Chinese family with LCA,which predicted a change of the protein p.S500X,may lead to loss of gene function.Conclusions: This study reported a novel mutation(c.1499C>G,p.S500X) of the CRB1 gene occurred in a Chinese family with LCA, thus expanding the spectrum of CRB1 mutations causing LCA. And we summarize the 76 mutations reported so far in CRB1 that caused LCA8.

AIM: To explore the multimodal imaging features of butterfly-like retinal pigment epithelial dystrophy(BPD)patients.METHODS: Retrospective analysis of the multimodal imaging of 18 BPD patients(36 eyes)from January 2016 to July 2019, including fundus color photography, infrared photography, autofluorescence, fundus fluorescein angiography, indocyanine green angiography, optical coherence tomography(OCT), and optical coherence tomography angiography(OCTA). RESULTS: A typical fundus color photography showed the appearance of a butterfly-like lesion caused by abnormal pigmentation. The appearance of the butterfly was not obvious after the lesion progressed, and the corresponding region shrinks; Infrared photography showed the yellow lesions clearly in the BPD patients, which are white highlight images; Autofluorescence showed patchy, spotted butterfly wing performance. When the lesion progressed, autofluorescence did not show typical butterfly changes, but it showed the damage of pigment epithelial cells; Fundus fluorescein angiography and choroidal angiography, in addition to showed butterfly lesions, can more accurately display vascular lesions, especially choroidal neovascularization(CNV); OCT showed lesions located between the retinal pigment epithelial(RPE)layer and the photoreceptors. As the lesion progressed, the pigment epithelium showed enlarged lesions correspondingly. Secondary CNV patients can be seen to break RPE; OCTA showed that the lesions were not obvious at the deep and superficial layers of the retina. But the choroidal blood flow signals were lost in some degrees in OCTA, and the blood flow images of CNV can be detected sensitively.CONCLUTION: Multimode imaging technology can provide imaging features of progression in BPD patients, which helps clinicians to understand the disease more deeply.

Familial exudative vitreoretinopathy(FEVR)is a severe clinically and genetically heterogeneous retinal disease which characterized by abnormal development of the peripheral retinal vessels. FEVR presents many clinical phenotypes, the main and typical feature is retinal folds. There are various inheritance modes with high genetic heterogeneity of FEVR including autosomal recessive, X-recessive, autosomal dominant recessive, and other scattered inheritance modes. So far, nine FEVR pathogenic genes have been reported: NDP, FZD4, LRP5, CTNNA1, TSPAN12, ZNF408, KIF11, CTNNB1, and JAG1 genes. These genes are mainly involved in signaling pathways such as Wnt, Notch, and Norrin-β-catenin. This article reviews the above nine FEVR pathogenic genes and their signaling pathways.

目的 评估经结膜入路前路开眶术,内外侧联合开眶术,2种不同入路手术摘除肌锥内视神经内侧眼眶海绵状血管瘤的临床效果.方法 回顾性分析云南省第二人民医院2009年09月至2019年09月收治的68例眼眶海绵状血管瘤,根据术式分为A、B2组.A组为结膜入路前路开眶术共33例,B组为内外侧联合开眶术共35例,评估2组患者在平均住院时间、手术时间、手术操作空间、术中出血量、肿瘤完整摘、术后并发症等指标,差异无统计学意义(P＞0.05).结果 A组平均住院天数为(5.03±1.63)d,B组为(8.34±2.13)d,两者相比差异有统计学意义(t=-4.926,P=0.000).A组平均手术时间为(55.62±5.43)min,B组为(109.21±13.72)min,两者相比差异有统计学意义(t＝-16.428,P=0.000).手术操作空间用注水法测量,用所注水的体积表示,A组平均为(5.22±0.21)mL,B组平均为(16.501±1.22)mL,两者相比差异有统计学意义(t＝-48.362,P=0.000).术中出血量:A组平均为(17.22±1.65)mL,B组平均为(59.29±6.42)mL,两者相比差异有统计学意义(t=-28.098,P=0.000).肿瘤完整摘除率:A组为90.9％(30/33),B组为97.14％(34/35);术后视力:A组患者术后视力提高和不变者占93.9％(31/33),术后视力下降占6.06％(2/33);B组术后视力提高和不变者占85.71％(30/35),术后视力下降或丧失者占14.3％(5/35).结论 结膜入路前路开眶术和内外侧联合开眶术在神经内侧眼眶海绵状血管瘤摘除术中各有优缺点,正确选择手术方式是完整摘除眼眶海绵状血管瘤的关键,术前应根据影像学资料准确判断肿瘤位置和大小.

Objective:To 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.Methods:A 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.Results:Among 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.Conclusions:SC 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.

Objective:To investigate the protective effect of C-X3-C motif chemokine receptor 1 (cx3cr1) antibody, a microglia activation inhibitor, on microcirculation during retinal ischemia reperfusion (RIR) and its possible mechanism.Methods:One hundred and fifty healthy adult C57BL/6 mice were randomized into blank control group, model group and cx3cr1 injection group by random number table method, with 50 mice in each group.The RIR model was established by anterior chamber infusion to elevate intraocular pressure in this study.Mice in the blank control group were intravitreally injected with 2 μl of sterile water.In the cx3cr1 injection group, the RIR model was established at 4 hours after the intravitreal injection (2 μl) of 0.2 μg/μl cx3cr1 antibody.Immunofluorescence staining of frozen eyeball sections was performed to assess the microglia activation by observing the Iba-1 positive expression in different retinal layers three days following the model establishment.Retinal preparation vascular staining was carried out to observe the changes in the density of deep and shallow retinal blood vessels and the number of activated microglia to evaluate the changes in retinal microcirculation.FITC-dextran contrast method was used to determine the retinal vascular leakage area.Real-time fluorescent quantitative polymerase chain reaction (qPCR) method was employed to detect the mRNA expression changes of hypoxia-related factors and inflammatory factors in the mice retina.The study protocol was approved by an Ethics Committee of Kunming Medical University (No.20180106). The use and care of the animals complied with the Regulations of the Administration of Affair Concerning Experimental Animals.Results:The immunofluorescence staining result of eyeball frozen section showed that in the blank control group, Iba-1 positive microglial cells were sparsely distributed in the retinal ganglion cell layer and inner plexiform layer, presenting branched state.In the model group, Iba-1 positive microglial cells were increased and moved outward to the outer retinal plexiform layer and outer nuclear layer obviously, showing globular or amoeba-like.The number of globular or amoeba-like Iba-1 positive cells was significantly reduced in the cx3cr1 injection group in comparison with the model group ( P<0.05). The number of activated microglial cells in different retinal layers of the model group was significantly larger than that of the cx3cr1 injection group and the blank control group (both at P<0.05). Compared with the model group, the number of activated microglial cells around the retinal blood vessels was reduced significantly in the cx3cr1 injection group.The double fluorescence result of retinal vascular staining and activated microglial cells showed that the density of deep blood vessels in the blank control group and cx3cr1 injection group was significantly higher than that of the model group, and the number of microglial cells around superficial and deep retinal vessels was significantly larger in the model group than that of the cx3cr1 injection group (all at P<0.05). The relative vascular leakage rate of the blank control group, model group and cx3cr1 injection group were (100.0±4.7)%, (162.1±10.6)% and (130.5±9.5)%, respectively, and the overall difference was statistically significant ( F=128.66, P<0.01). The relative vascular leakage rate in the cx3cr1 injection group was significantly lower than that in the model group ( P<0.05). The qPCR result showed that the relative expression levels of vascular endothelial growth factor-A (VEGF-A), hypoxia inducible factor-1a (HIF-1α), tumor necrosis factor-α (TNF-α) and interleukin-1β (IL-1β) mRNA were significantly reduced in the retina of the cx3cr1 injection group in comparison with the model group (all at P<0.05). Conclusions:Intravitreal injection of cx3cr1 can protect the vascular integrity of the retinal microcirculation system in RIR mice.

目的 观察光学相干断层扫描血管成像(OCTA)在中心性浆液性脉络膜视网膜病变(CSC)患者脉络膜新生血管(CNV)检测中的应用价值.方法 回顾性病例观察研究.选取2018年3月至2019年3月在云南省第二人民医院诊断为慢性中心性浆液性脉络膜视网膜病变伴脉络膜新生血管患者30例(34眼)纳入研究,总结分析其OCTA与眼底彩色照相、眼底自发荧光、荧光素眼底血管造影(FFA)和OCT等传统影像学对比运用的临床体会.结果 12眼FFA存在荧光素渗漏,但形态模糊,不能确定是否伴有CNV,行OCTA则可以清晰显示新生血管影像.8眼FFA未检出CNV,但OCTA清晰显示出新生血管形态.3眼眼底彩色照相及OCT检查高度怀疑CNV的存在,但因荧光素钠皮试阳性,均无法实施FFA,而OCTA则显示出病变区域血流信号,明确了CNV的存在.6眼FFA无明显荧光素渗漏,OCT示无神经上皮层脱离、无色素上皮层脱离,但OCTA提示CNV的存在.5眼通过随访过程中的病情跟踪观察,发现给予患者单一抗VEGF或PDT治疗之后视网膜下积液无明显吸收,而联合治疗后积液明显吸收.结论 OCTA在CNV的检查敏感性方面优于传统的检测手段.OCTA可以作为一项安全有效的眼底影像学检查手段对慢性CSC患者的CNV进行观察,从而指导该类患者的诊断、治疗及预后分析.

Objective:To analyze the OCT angiography (OCTA) features of eyes affected with angioid streaks.Methods:Retrospective analysis of 26 patients (52 eyes) diagnosed as angioid streaks by multimodal imaging in Ophthalmology Department of Yunnan Second People’s Hospital from May 2017 to February 2019 were included in this study. There were 18 males and 8 females, with the mean age of 50.8±6.9 years. All the patients were binocular. There were 34 eyes in 20 patients with CNV. Among them, 13 eyes had a course of disease within 1 month, 16 eyes had a course of disease over 1 month, and 5 eyes were treated with anti-VEGF drugs. All patients were examined by ultra-wide-angle fundus photography, infrared fundus imaging (IR), spectral-domain OCT, FAF and FFA. At the same time, the Heidelberg OCTA instrument was used for OCTA examination. The central wavelength was 840 nm, the acquisition speed was 85,000 times/s, and the width was 45 nm. A 3 mm × 3 mm scan was obtained. Each cube consisted of two 304 B scans of repeated volumes, and motion correction was performed using two orthogonally captured image volume registration. The results of fundus color photography, IR, FAF, FFA, OCT and OCTA were compared and analyzed to summarize the image features of AS and its secondary CNV in OCTA.dus color photography, IR, FAF, FFA, OCT and OCTA were compared and analyzed to summarize the image features of AS and its secondary CNV in OCTA.Results:Among 52 eyes, 40 eyes showed choroidal capillary shadows in OCTA, and no obvious abnormal OCTA images in 12 eyes. Ten eyes OCTA showed a vascular network beseide the optic disc, FFA showed fluorescent staining instead of fluorescein leakage. Among the 52 eyes, 34 eyes had secondary macular CNV, and 34 eyes had different forms of CNV. Different forms had a certain correlation with the length of medical history and treatment history. Among them, 13 eyes with short course of disease (less than 1 month) and untreated patients had smaller flower-ring morphology, 16 eyes with longer course (more than 1 month) but no treatment had larger fan-shaped segments, and 5 eyes with anti-VEGF therapy were trimmed after the dendritic shape.Conclusions:AS streaks appeared no blood flow signal area in OCTA, and repairing vascular network beside the optic disc can be observed in some AS patients. The CNV performance with different disease course and treatment experience is different.

目的 观察不同脉络膜肿瘤,包括脉络膜黑色素瘤、脉络膜骨瘤、脉络膜转移癌及脉络膜血管瘤,在OCT中的影像学特征.方法 收集2016年1月至2018年9月在云南省第二人民医院眼科就诊的脉络膜肿瘤患者36例(42眼)的临床资料,其中脉络膜黑色素瘤4例(4眼)、脉络膜血管瘤12例(12眼)、脉络膜转移癌10例(14眼)、脉络膜骨瘤10例(12眼),分析不同肿瘤的OCT影像学特征.结果 脉络膜黑色素瘤4眼,OCT均表现为视网膜下多个团状高反射信号,视网膜各层组织被破坏.脉络膜转移癌14眼,OCT表现为脉络膜崎岖不平、波浪状改变,其下隐约可见脉络膜小血管形态,视网膜神经上皮层与色素上皮层脱离,视网膜外层组织内可见大量细颗粒状、团状高反射物堆积.脉络膜骨瘤12眼,10眼OCT示脉络膜层次内一个网状反射影像信号,此处结构与周围脉络膜血管结构完全不同,其后组织反光被遮挡.脉络膜血管瘤12眼,OCT均表现为视网膜下脉络膜隆起,表面光滑,视网膜色素上皮层下反射信号均匀.结论 OCT可作为一项安全有效的眼底影像学检查手段对不同脉络膜肿瘤形态进行观察,结合其他眼底检查手段可为脉络膜肿瘤的诊断和鉴别诊断提供依据.

Objective:To observe the clinical and imaging characteristics of acute idiopathic macular degeneration (AIM).Methods:A retrospective clinical study. From March 2016 to January 2018, 5 eyes (5 AIM patients) in The Second People's Hospital of Yunnan Province were included in the study. Among them, there were 4 males (4 eyes) and 1 female (1 eye); all patients were monocular with the average age of 34.2 years. The course of illness from onset of symptoms to treatment was 4-22 days. All affected eyes were examined by BCVA, fundus color photography, OCT, FAF, and FFA. Among 5 eyes, 1 eye with optic disc vasculitis was given oral glucocorticoid treatment; 4 eyes were not interfered after the diagnosis.Results:The follow-up time was 6 months. During follow-up, BCVA, fundus color photography, and OCT examination were performed. The results were all a sudden decrease in monocular vision, accompanied by visual distortion or central dark spots. At the first visit, the BCVA was 0.1, 0.2, 0.2, 0.05, and 0.5; at the last follow-up, the BCVA of the affected eye was 0.8, 0.6, 0.5, 0.5, and 1.0, respectively. Fundus color photography showed that at the first diagnosis, all the affected eyes showed irregular round yellow-white lesions in the macular area, including 1 eye with small patches of hemorrhage and 1 eye with pseudopyous changes in the macular area. Two to three weeks after the initial diagnosis, the yellowish-white lesions and bleeding in the macular area were basically absorbed. The center of the lesion showed weak pseudopod-like fluorescence, and the surrounding area was surrounded by strong fluorescence in FAF examination. The irregular and strong fluorescence in the early macular area and accumulation of late fluorescein in FFA examination. One eye was receivied glucocorticoid therapy. The upper layer of the retinal nerve in the macular area was detached, and the inferior space showed focal strong reflective material in 3 eyes in OCT examination. At the first diagnosis, the retinal neuroepithelial layer was detached, the top of the RPE layer was irregular with strong reflective material, and the structure of the ellipsoid zone and the chimera zone was unclear; as the course of the disease prolonged, the outer retinal structure recovered.Conclusions:AIM is characterized by inflammatory exudative changes in the outer layer of the retina in the macular area; FFA is characterized by strong subretinal disc-like fluorescence or multifocal weak fluorescence in the macular area; OCT mainly manifests as neuroepithelial detachment and changes in the outer retina and RPE, The structure can be restored by itself.

目的 对新生儿眼底病变进行筛查统计,并分析视网膜出血的影响因素.方法 回顾性分析2013年1月至2016年12月在我院新生儿病房行眼底筛查的1558例新生儿资料.统计新生儿眼底病变发病率以及视网膜出血发病情况,对影响视网膜出血的相关因素进行分析.结果 本组1558例新生儿中筛查出眼底病变患儿196例,占12.6％;类型包括视网膜出血(8.9％)、早产儿视网膜病变(1.3％)、眼底渗出(1.2％)、视网膜有髓神经纤维(0.5％)、视网膜色素沉着(0.4％)、先天性结晶样变性(0.3％)、视网膜母细胞瘤(0.1％).单因素分析结果显示,分娩方式、多胎、宫内窘迫、机械通气是影响视网膜出血的相关因素(均为P＜0.05);而性别、是否足月、出生体质量、是否子痫、是否胎膜早破、是否窒息、是否输血、是否吸氧等因素与视网膜出血的发生并无明显相关性(均为P＞0.05).多因素Logistic回归分析结果显示,阴道分娩、机械通气是视网膜出血的独立危险因素.结论 新生儿眼底病变的发生率较高,其中绝大多数为视网膜出血,阴道分娩和机械通气是导致新生儿视网膜出血的独立危险因素,应针对此类新生儿群体加强观察和干预.

目的 对早产儿视网膜出血进行统计,分析探讨早产儿视网膜出血的危险因素及临床特点.方法 选取2013年1月—2016年12月在我院进行眼底病筛查的早产儿1142例进行回顾性分析,患儿均在散瞳后实施眼底检查,观察视网膜出血情况,评估影响视网膜出血的相关因素.结果 1142例早产儿中,视网膜出血有112例,占9.8％,视网膜出血程度分级Ⅰ、Ⅱ、Ⅲ、IV级分别有42例、47例、23例、0例,分娩方式是引起视网膜出血的主要危险因素,经阴道分娩的早产儿发生视网膜出血的几率较剖宫产高,差异具有统计学意义(P<0.05),是否使用机械通气及助产与视网膜出血也密切相关.结论 分娩方式及机械通气是导致早产儿视网膜出血的重要原因,经阴道分娩的难产患儿是高危人群.

目的 探讨视网膜缺血-再灌注损伤(retinal isehemia-reperfusion injury,RIRI)中小胶质细胞活化与视网膜微循环损伤的关系及作用机制.方法 160只雄性C57BL/6小鼠右眼均采用前房灌注建立RIRI模型为RIRI组,左眼不作处理为正常对照组.在损伤后24 h、48 h、72 h分别进行视网膜冰冻切片、视网膜铺片免疫荧光染色检测小胶质细胞的活化情况,检测相关缺氧因子及炎症因子的表达,与正常对照组比较,研究小胶质细胞的激活状态与微循环损伤的关系,并初步分析其作用机制.结果 视网膜微循环结构损伤观察结果显示,与正常对照组相比,RIRI后24 h组大部分血管仍呈正常形态;RIRI后48 h组,闭塞的血管数量增多;RIRI后72 h组,血管损伤明显加重.浅层毛细血管密度正常对照组,RIRI后24 h、48 h、72 h组间两两比较差异均无统计学意义(均为P ＞0.05).而深层血管网毛细血管密度RIRI后72 h组与其余3组相比明显减少,差异均有统计学意义(均为P ＜0.05),其余3组间差异均无统计学意义(均为P＞0.05).视网膜冰冻切片检测显示,RIRI后24 h组与正常对照组各层视网膜中抗离子钙接头蛋白(ionized calcium bindingadaptor molecule-1,Iba-1)阳性细胞数差异无统计学意义(P＞0.05).RIRI后48 h组各层中Iba-1阳性细胞数与正常对照组及24 h组差异均有统计学意义(均为P＜0.05).RIRI后72 h组各层中Iba-1阳性细胞数与正常对照组、24h组、48 h组差异均有统计学意义(均为P＜0.05).视网膜铺片结果显示,RIRI后48 h组和72 h组活化的小胶质细胞数明显增加,与正常对照组和RIRI后24 h组在两个层次毛细血管中差异均有统计学意义(均为P＜0.05),而72 h组小胶质细胞的活化达到高峰值,与其余组差异均有统计学意义(均为P＜0.05).RT-PCR检测结果显示:血管内皮生长因子和缺氧诱导因子-1oα的缺血缺氧因子,肿瘤坏死因子-α和白细胞介素-1β的炎症因子在正常对照组与RIRI后24 h组、48 h组、72 h组,组间比较差异均有统计学意义(均为P＜0.05).结论 激活的小胶质细胞在RIRI中发挥了对微循环的破坏作用,损伤早期抑制小胶质细胞的活化可能成为此类疾病治疗的新思路.

目的 探讨年龄相关性黄斑病变(age-related macular degeneration,AMD)中不同病变类型视网膜色素上皮脱离(pigment epithelial detachment,PED)的特征,以期为AMD不同类型的诊断及预后评估提供参考依据.方法 通过影像学检查将96例(124眼)患者分为视网膜血管瘤样增生(retinal angiomatous proliferation,RAP)、息肉样脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)、脉络膜新生血管(choroidal neovascularization,CNV).通过光学相干断层扫描(optical coherence tomography,OCT)结合眼底彩色照相、眼底荧光素血管造影、眼底自发荧光等检查方法对以上几类病变中PED大小、形态、病变性质、预后等重要特征进行回顾分析并对比总结.结果 96例患者中RAP-PED为10例(10.42％)、PCV-PED为32例(43.75％)、CNV-PED为54例(45.83％).RAP-PED多为浆液性、双眼发病.PCV-PED多为多灶性、出血性,且多为单眼发病.CNV-PED的数量形态表现多样,大小不等相连,形成"波浪样"外观.结论 加深对各类型AMD中PED发生的几率、类型、合并症以及预后等的认识,有助于更为精确指导诊断治疗及预后分析.

目的:探讨不同病变阶段视网膜色素变性患者的脉络膜血管状态.方法:回顾性分析云南省第二人民医院眼科2000年1月至2015年4月诊断为原发性视网膜色素变性的患者226例(452眼)的眼底特征,并复习相关文献,重点分析总结脉络膜血管情况.结果:31例(62眼)病变前期患者,荧光素眼底血管造影显示动脉期脉络膜血管及视网膜血管充盈正常,未出现充盈延迟或缺损现象.25例(50眼)病变早期患者,荧光素眼底血管造影显示动脉前期可见脉络膜背景荧光显示,部分脉络膜毛细血管未同时充盈,动脉期时上述部分完成充盈.106例(112眼)病变中期患者,荧光素眼底血管造影显示动脉期出现部分脉络膜毛细血管萎缩区,仅能看到残存的粗大脉络膜血管,随造影过程的进展,此区域并未出现充盈,即呈现永久的脉络膜毛细血管充盈缺损.64例(128眼)病变晚期患者荧光素眼底血管造影显示,广泛的脉络膜毛细血管萎缩区,其间可见残存的脉络膜粗大血管,至造影晚期均呈现充盈缺损,萎缩区边缘随造影过程呈强荧光表现.结论:荧光素眼底血管造影可显示脉络膜血管萎缩变化情况,这一指标可作为反映不同病变阶段视网膜色素变性患者病情进展变化的重要依据.

Objective To observe the treat effect of thyroid-associated ophthalmopathy (TAO)with dysthyroid optic neuropathy (DON).Methods The clinical data of 42 cases (60 eyes) of TAO with DON diagnosed in 2009-2013 were retrospectively analyzed.Results Average best correct visual acuity (BCVA) was 0.25 before treatment,and 0.6 post treatment.Visual field was abnormal in 51 eyes (86.67％,51/60) before treatment,and 7 eyes (11.67％,7/60) post treatment.Other index,such as intraocular pressure,orbital tense,exophthalmos,eye muscle,fundus had improvement compared with prior treatment.All patients used methylprednisolone pulse therapy,18 patients received 1 course of treatment 42.9％ (18/42),15 patients received 2 courses of treatment 35.7％ (15/42),9 patients received 3 courses of treatment 21.4％ (9/42);26 patients (36 eyes) undergone orbital decompression surgery.The BCVA improved in 33 eyes,and unimproved in 3 eyes one month later after surgery.Compared with methylprednisolone pulse therapy,the BCVA improved obviously after orbital decompression surgery.Conclusions There are abnormal of visual acuity,visual field,VEP,orbital tense,exophthalmos,eye muscle,fundus;Methylprednisolone pulse therapy and orbital decompression surgery are effective treatments of DON.

视网膜大动脉瘤主要发生于高血压、动脉硬化患者,由于动脉血管内平滑肌纤维逐渐被胶原纤维替代,血管壁失去弹性,血压升高时造成血管壁瘤样扩张、渗出、出血[1].由于视网膜大动脉瘤可能造成玻璃体积血以及内界膜下、视网膜浅层、视网膜深层、视网膜下等视网膜多层次出血[2].容易误诊为视网膜分支静脉阻塞、脉络膜新生血管、Coats病、脉络膜占位病变等可能出现类似表现的出血性疾病[3].进一步深入了解视网膜大动脉瘤的临床尤其是影像检查特征有助于减少误诊.我们同顾分析了一组视网膜大动脉瘤患者的临床资料,着重观察不同层次视网膜出血的临床特征.现将结果报道如下.

<正>Kiilingsworth[1]等在角膜近穿孔甚至穿孔等危重角膜病中,运用保存角膜植片进行治疗性穿透性角膜移植取得良好效果.现在,因为角膜材料稀缺,如何挽救危重角膜病患者的眼球,已成为我们面临的一个难题.治疗性穿透性角膜移植是一种为了挽救眼球、去除病灶、恢复角膜的完整性,运用非活性角膜进行穿透性角膜移植的一种手术

Objective To investigate the characteristics of fundus imaging of retinal vascular proliferative tumors.Methods Review of 5 cases(5 eyes) diagnosed as retinal vascular proliferative tumors,analyzed the characteristics of fundus fluorescein angiography(FFA),B ultrasound and retinal optical coherence tomography(OCT) imaging features.Results Tumor is a single,boundary clear mass,its size is different,The tumor was red,the surface was distributed by blood vessels,visible small pieces of hemorrhage and large blood vessels extending from the retinal surface.Subretinal yellow-white exudate around the tumor.FFA showed capillary network on the tumor surface,dilated blood vessels and leakaged fluorescein,The retinal vascular near the tumor is expanded and fluorescein leakage.B scan showed solid echo.OCT showed retinal neuroepithelium detachment and visible hyperechoic.Conclusion Retinal vascular proliferative tumor characterized by typical retinal red tumor-like lesions and large retinal and subretinal exudation,B scan,FFA,the OCT imaging examinations can help diagnose,and help research the pathology and progression of the retina.

猪囊尾蚴病是人误食猪肉绦虫虫卵或孕节后,在小肠内发育为猪囊尾蚴,并寄生在人的皮下组织、肌肉、脑、眼、心等处引起的人畜共患寄生虫病[1,2].眼是其常见寄生部位[3].眼内猪囊尾蚴临床表现及影像特征已有较多报道[4-6],但其临床病理特征报道较少.我们收集了一组手术治疗的眼内猪囊尾蚴病患者资料,观察了猪囊尾蚴的临床和病理特征.现将结果报道如下. 1对象和方法 2000年至2011年在我院行手术治疗并经病理确诊的眼内猪囊尾蚴病患者8例纳入观察.其中,男性6例,女性2例.年龄11.5～56.0岁.8例患者均为单眼发病.以患者出现眼部或脑部症状为发病起始计算,病史1～24个月.手术前视力光感～0.5.7例患者既往曾进食生猪肉.其中,3例患者表现为眼前黑影,2例患者表现为视力下降,1例患者表现为眼部疼痛,2例患者因脑部猪囊尾蚴病眼部检查时发现.