Coats病临床诊断中的问题分析

目的 分析Coats病临床诊断中存在的问题以及误诊的主要原因。 方法 由具有丰富临床经验的眼底病专家全面回顾分析一组临床拟诊和漏诊的Coats病患者间接检眼镜检查、眼底彩色像、荧光素眼底血管造影（FFA）、随访治疗记录等临床资料，主要通过重新系统阅读FFA检查图像,根据特发性视网膜毛细血管和微血管的异常扩张这一Coats病的主要特征修订或确认原诊断,探讨Coats病临床诊断中误诊的主要原因。 结果 68例75只眼中，初诊为Coats病，最后确诊为非Coats病45例。其中，最后诊断为视网膜静脉阻塞21例；视网膜血管炎9例；糖尿病视网膜病变3例；陈旧性后葡萄膜炎3例；先天性视网膜劈裂症3例；增生性玻璃体视网膜病变、家族性渗出性玻璃体视网膜病变、视网膜蔓状血管瘤、陈旧性孔源性视网膜脱离、黄斑前膜、特发性黄斑旁毛细血管扩张症各1例。初诊为非Coats病，最后确诊为Coats病23例。其中，最初诊断为陈旧性后葡萄膜炎6例；视网膜血管炎5例；渗出型老年性黄斑变性4例；视网膜大动脉瘤2例；视网膜血管瘤3例；眼内肿瘤2例；中心性浆液性脉络膜视网膜病变1例。 结论 Coats病临床诊断中错误较多。未能全面掌握Coats病的临床特点以及Coats病定义的核心是发生与Coats病相关的临床诊断错误的主要原因。 (中华眼底病杂志, 2005, 21: 377-380)     

Coats病的研究进展

自从六十年代广泛采用荧光眼底血管造影及光凝疗法以来,在Coats病的诊断和治疗上取得了很大的进步,在其他方面亦有不少研究,今就身边能查到的文献综述如下。 Coats(1908)以“大量渗出型视网膜病”为题,从临床和病理方面对本病做了阐述。病例分三种类型:1.没有明显血管改变的病例;2.有显著血管改变的病例;3.有动静脉交通的病例。1912年又以“外层渗出性视网膜炎(外层出血性视网膜炎)”为题发表第二篇论文,其中删去了第三类病例,因当时已确认为视网膜血管瘤病(von Hippel病)。后者虽有些继发改变如渗出、视网膜脱离等类似Coats病,但其特点为较大的不规则圆形血管瘤,分别有进入的动脉和输出的静脉。     

严重Coats病的激光治疗一例报告

Coats病于1908y George Coats首先报道，故命此名。男性多见。该病的特征是视网膜血管结构改变，呈血管瘤样扩张，毛细血管断裂及渗漏，引起视网膜水肿，脂质沉着及渗出性视网膜脱离，造成视力下降。继发改变可有新生血管形成、玻璃体出血、白内障、虹膜红变及新生血管性青光眼，严重病例可致眼球萎缩。本病的治疗主要是在病变早期激光封闭视网膜异常血管，减少视网膜渗漏，阻止病变发展。而当眼底有大量渗出和视网膜下积液出血时，增加了治疗的难度。患者与医生往往放弃治疗。然而只要诊断明确，治疗得当，反复多次激光治疗，部分患者还是可以得到较好的效果。现将1例严重Coats病的激光治疗取得较好的效果病例报告如下。     

Coats病的诊断与治疗

Coats病即外层渗出性视网膜病变,于1908年由George Coats首先描述,它是以视网膜毛细血管扩张,视网膜内黄白色渗出以及渗出性视网膜脱离为特点的眼底病。本病须与视网膜母细胞瘤（retinoblastoma,RB）鉴别。过去,我院病理科几乎每年都会有将Coats病误诊为RB而作眼球摘除的病例,但近20多年来,无一例错摘事件发生,这应归功于我们对Coats病与RB认识的加深和影像诊断学的发展。     

玻璃体切除硅油充填联合眼内光凝治疗晚期Coats病

目的：探讨玻璃体切除硅油充填联合眼内光凝治疗伴有广泛渗出性视网膜脱离的晚期Coats病的临床治疗效果。方法：伴有广泛渗出性视网膜脱离的晚期Coats病患13例(13眼)进行玻璃体切除硅油充填联合眼内光凝治疗,其中并发性白内障同时行晶状体切除3眼。术后随访时间0．5～2a。观察术后视力、眼压、视网膜复位情况、视网膜血管改变及术后并发症。结果：术后3mo,13例患视网膜均复位,经FFA检查见异常扩张的血管逐渐闭塞和退缩;其中9眼最佳矫正视力均有不同程度提高,≥0．31眼,0．1～0．33眼,≤0．15眼;另有4眼术后视力没有明显变化。术后6和14mo时各有1例因血管渗漏致下方局限性渗出性视网膜脱离,经补行激光治疗后视网膜下液吸收,视网膜复位。结论：玻璃体切除硅油充填联合眼内光凝是治疗伴有广泛渗出性视网膜脱离的晚期Coats病的最为安全有效的方法。     

Coats病的分型与年龄

目的：就Coats病的分型与年龄问题进行探讨。 方法：分析华西医科大学附属第一医院眼科1981年1月至1994年12月经眼底荧光血管造影（fundus fluorescein angiography,FFA）确诊的Coats病75例，79眼。 结果：男性59例（78.7%），女性16例（21.3%）；初诊时平均年龄26岁，其中19-60岁患者45例（60.0%）.5例在检眼镜下未见血管异常，在FFA检查中均发现有视网膜毛细血管及小血管扩张、微动脉瘤存在。 结论：作者认为Coats病在本质上不应该有Ⅰ型和Ⅱ型的区别，在临床上不宜继续沿用这种分型方法。Coats病不仅限于婴幼儿及青少年，可发生于任何年龄。 （中华眼底病杂志，1996，12：77-79）     

视网膜冷凝治疗Coats病的临床疗效观察

Coats病治疗的关键是破环异常扩张的视网膜毛细血管及小动脉，临床上常用的治疗方法有激光和冷冻。激光适用于渗出水肿较轻的病例，但当渗出水肿严重引起视网膜脱离时，激光则难以达到治疗效果，部分病例可通过冷凝治疗来达到治疗目的。我们观察伴有视网膜脱离行视网膜冷凝病例的随访情况，以了解冷凝治疗伴有视网膜脱离的Coats病的疗效。     

伴有严重渗出性视网膜病变的Coats病1例

Coats病是散发的非遗传性疾病,不伴有系统性异常,常单眼发病。我们报道1例27岁俄罗斯Coats病女患者。眼底检查显示左眼下方视网膜血管瘤样改变,血管迂曲扩张、呈串珠状,伴有严重的浆液性视网膜脱离。中心凹亦脱离,有成簇的白色沉积物。     

外层渗出性视网膜病变1例

外层渗出性视网膜病变又称为Coats氏病，又称视网膜毛细血管扩张症。其病因不明，无遗传性，与系统性血管异常无关。     

205例Coats病临床分析

目的分析总结Coats病患者的一般临床规律和眼底特征。方法回顾分析205例Coats病患者211只眼的临床资料，统计分析患者性别、年龄、眼别及视力分布情况；根据检眼镜以及荧光素眼底血管造影(FFA)检查所见的眼底及FFA特征，分析病变部位、范围及病变程度，探究病变分布及发展规律。结果 205例患者就诊时平均年龄28.0岁，20岁以上占54.2%。其中，男性占76.1%；单眼发病占97.1%。211眼中，视力0.3及其以下者占67.3%。所有患者均有眼底微血管异常扩张，90.5%伴有黄白色脂质渗出；异常扩张血管90.1%位于颞侧，73.9%位于赤道以前，72.5%波及1个以上象限。结论 Coats病多发于男性，各年龄段均可发病，几乎均为单眼发病。最本质特征是微血管异常扩张，绝大部分位于颞侧赤道前的视网膜，通常伴有黄白色脂质渗出并波及黄斑，对视功能损害严重。 （中华眼底病杂志，2008，24：276-278）     

视网膜色素变性光感受器的组织病理学观察

目的：研究视网膜色素变性光感受器的组织病理学改变。 方法：对9例11眼视网膜色素变性的光感受器进行光学和电子显微镜观察。 结果：发现赤道部光感受器病理损害最明显，其次为周边区、后极部和黄斑。中期病例病理改变包括外节盘膜退变、变小和连接纤毛减少或消失；内节粗短、线粒体肿胀。晚期病例内、外节和纤毛消失，外界膜粘附在色素上皮细胞或Bruch膜上。光感受器细胞核减少、排列紊乱，细胞变性、结构破坏。移位的Müller细胞和增生肥大的细胞突占据了内外节和光感受器细胞核消失所遗留的空间。色素上皮细胞退变，部分消失或移位到视网膜内。结论：视网膜色素变性光感受器有明显损害。 （中华眼底病杂志，1996，12：160-162）     

人胎视网膜血管发生方式的研究

目的：观察人胎视网膜血管的形成方式。方法：收集13～38周胎儿视网膜86例，免疫组织化学染色(ABC)法，光镜观察。结果：在胎儿12—13周时，间充质的梭形细胞从视盘处进入视网膜，并向锯齿缘迁移，同时分化、增殖为内皮细胞索，此索经管道化和改建成为节细胞层内的血管。第26周，节细胞层内已生成血管“出芽”，朝神经纤维层和内核层生长，分别在神经纤维层内和内核层的内、外缘各形成一层毛细血管网。结论：足月胎儿视网膜基本具有四层血管，分别由两种不同方式生成。 （中华眼底病杂志，1996，12：88-90）     

视网膜色素变性视网膜的组织病理和超微结构观察

目的：探讨视网膜色素变性的病理改变及其发病机制。 方法：对1例常染色体显性遗传性视网膜色素变性患者的视网膜进行组织病理和超微结构观察．结果：视网膜各层组织均有变性改变和结构紊乱，并有区域性差异，后极部变性较周边部为重．视网膜色素上皮细胞病变与感光细胞病变程度密切相关，但后者似较前者为重．感光细胞的超微结构有明显变性改变，尤以外节变性，线粒体变性、胞浆内脂褐索沉着为突出。结论：超微结构变化提示感光细胞能量代谢系统和(或)自噬系统功能障碍。 （中华眼底病杂志，1997，13：24-26）     

兔视网膜中蛋白激酶C的定位研究

目的：证实兔视网膜中有α、β和γ三种蛋白激酶C(protein kinase C，PKC)亚型的分布。方法；采用免疫组织化学技术，以抗PKC同功酶I(α)、Ⅱ(β)、Ⅲ(γ)单克隆抗体对兔视网膜进行PKC同功酶定位研究。 结果：兔视网膜中有PKC-α、PKC-β和PKC-γ的阳性免疫反应。PKC-α免疫反应主要呈现在内核层的双极细胞；PKC-β免疫反应主要呈现在神经节细胞层的神经节细胞；PKC-γ则在神经节细胞层、内网状层以及光感受器的外节呈弥漫性弱阳性染色。 结论：作为中枢神经系统的一部分，视网膜可以同时存在PKC-α、PKC-β和PKC-γ三种亚型。 （中华眼底病杂志，1996，12：242-244）     

Pars plana vitrectomy for treatment of advanced Coats’ disease—presentation of a modified surgical technique and long-term follow-up

Background

To present a modified surgical technique in the treatment of retinal detachment secondary to advanced Coats’ disease in children, and report on long-term anatomical and functional outcome.

Methods

We analysed an interventional case series of 13 patients (13 eyes) with advanced Coats’ disease characterised by retinal detachment in addition to massive subretinal exudates and vascular malformation. The presented patients underwent pars plana vitrectomy (PPV), including a modified technique of exocryotherapy applied after fluid–air exchange in order to achieve complete treatment of the vascular changes, to reduce associated side-effects, and to avoid retinectomy and silicone oil tamponade.

Results

Within a median follow-up period of 37 months (range: 18–66 months), no enucleation was necessary. Four eyes (31 %) did not need any further therapy, and in nine eyes (69 %) additional treatments were performed. Six patients (46 %) required revisional surgery with silicone oil tamponade. In ten eyes (77 %), the pathologic vessels and exudates finally regressed and the retina reattached. Visual acuity (VA) could be stabilized in the majority of patients: in three eyes (27 %) VA improved, in four eyes (36 %) VA remained stable, in four eyes (36 %) visual acuity (VA) deteriorated, and in two eyes VA could not be evaluated.

Conclusions

The presented modified technique allows for sufficient cryotherapy of vascular malformations, even in the presence of massive exudation, in a subset of patients with advanced Coats’ disease, and thus may reduce surgery-related complications and improve the rehabilitation process of these young patients.     

Hemorrhagic retinal macrocysts in advanced Coats disease.

The authors report a case of advanced unilateral Coats disease with associated hemorrhagic retinal macrocysts. Fluorescein angiography showed the macrocysts to be intensely hypofluorescent due to absence of perfused retinal blood vessels in the inner wall and presence of intracavitary blood blocking outer wall and choroidal fluorescence. Around the cystic lesions and in other areas of the peripheral retina, characteristic telangiectatic retinal blood vessels were evident. The mechanism responsible for the development of hemorrhagic retinal macrocysts in Coats disease appears to be coalescence of microcystic spaces in edematous and degenerated chronically detached retina.     

Intraretinal oxygen consumption in the rat in vivo

PURPOSE: To make the first quantitative assessment of the rate of oxygen consumption in high oxygen-consuming layers of both the outer and inner retina of the rat in vivo. METHODS: Oxygen-sensitive microelectrodes were used to measure the oxygen tension as a function of depth through the retina in anesthetized rats. Individual PO2 profiles were fitted to a multilayer mathematical model of PO2 distribution that is able to determine the oxygen uptake in those retinal layers in which the oxygen supply is through diffusion from vascular layers of the retina. This includes the entire outer retina and the region of the inner retina containing the inner plexiform layer. Measurements were performed in the light (13 mW/cm2 at the cornea) and in the dark and the amplitude and time constant for light-induced changes in outer retinal oxygenation determined. RESULTS: Under light-adapted conditions, the oxygen consumption of the outer retina was 148 +/- 11 nL O2/min/cm2 (n = 20) [corrected] and that of the included portion of the inner retina was 184 +/- 17 nL O2/min/cm2 [corrected]. In the dark, outer retinal oxygen consumption increased by 47.8% (P < 0.001), and the time constant for the resultant PO2 decrease in the outer retina was 14.9 +/- 1.8 seconds (n = 16). There was no significant change in inner retinal oxygen consumption between light and dark conditions (P = 0.89). CONCLUSIONS: Under light-adapted conditions the oxygen uptake by the selected region of the inner retina (primarily the inner plexiform layer) is greater than that of the outer retina (P < 0.01). Dark adaptation rapidly and significantly increases outer retinal oxygen consumption, but the inner retina remains unaffected.     

Selective immunohistochemical staining in the paraneoplastic retinopathy syndrome.

BACKGROUND: The mechanism leading to visual loss in paraneoplastic retinopathy is not known. An autoimmune process has been imputed based on immunologic investigations of several patients and by analogy to certain other paraneoplastic syndromes. METHODS: Two patients with documented small cell carcinoma of the lung who had clinical evidence of paraneoplastic retinopathy are described. Histopathologic examination of the retina from one patient and immunohistochemical staining of human retina with serum from control subjects and both patients were performed. RESULTS: Electroretinograms demonstrated dysfunction of photoreceptors in both patients, with predominant loss of rod function in one patient. Post mortem examination showed patchy loss of photoreceptors of the extramacular retina and relative sparing of cones, findings consistent with the clinical and electrophysiologic test results. Serum from both patients stained the retina in an identical manner, with restriction of the stain to the outer retina. Stain was present over the outer plexiform layer, the outer nuclear layer, and the inner and outer segments of most photoreceptors. A sharp demarcation was present between those areas that did and did not stain. All rod inner and outer segments appeared to stain, and many cone inner segments were not stained. Immunologic tests obtained elsewhere did not show serum antibody to the 23 kD protein. CONCLUSION: These findings support the concept of an autoimmune pathogenesis by showing selectivity of the immune response and correlation between the apparent target of the immune response and the clinical and pathologic findings. The mechanism by which cell loss occurs in the retina is not answered by this study. The absence of antibody to the 23 kD protein does not exclude the diagnosis of paraneoplastic retinopathy.     

Coats病黄斑病变的临床分析

目的 探讨Coats病黄斑病变的发生、发展规律及激光治疗后黄斑病变的转归.方法 回顾性系列病例研究.回顾性分析2004年10月至2007年6月首诊的25例(26只眼)Coats病患者临床资料.患者均经双眼前节裂隙灯显微镜检查、散瞳间接检眼镜检查、荧光素眼底血管造影和相干光断层扫描检查确诊.所有患者均行氪黄激光光凝治疗.采用SAS 8.0统计学软件,对黄斑区渗漏灶边缘至周边渗漏灶后缘的距离、视网膜小血管渗漏面积占整个视网膜面积的比例分别与黄斑病变程度及不同年龄组间黄斑病变程度进行累积比数Logistic回归分析.结果 25例(26只眼)黄斑区渗漏灶边缘至周边渗漏灶后缘的距离在0～9个视乳头直径之间,其距离越远,黄斑病变程度越低,差异有统计学意义(X~2=7.3212,P=0.0068);周边视网膜渗漏面积占整个视网膜面积的比例为1/8-1/1不等,其病变所占比例越大,病变程度越高,差异也有统计学意义(X~2=4.8853,P=0.0271);不同年龄分组对黄斑病变程度的影响则尤统计学意义(X~2:0.1491,P=0.6994).激光治疗后随访1至4年,10例(11只眼)黄斑区硬必恶性渗出合并黄斑水肿患者,眼底检查显爪渗出范围明显缩小,渗漏程度明显减轻;矫正视力不变或提高.结论 Coats病黄斑病变是一个从周边到后极的病变过程.治疗的关键是针对周边血管异常扩张及渗漏灶进行激光光凝治疗.