Coats病的诊断与治疗进展

Coats病又称为外层渗出性视网膜病变,它是一种以视网膜毛细血管和微血管异常扩张为特征,并常伴有视网膜内或视网膜下脂质渗出及渗出性视网膜脱离的疾病,晚期可并发新生血管性青光眼甚至眼球萎缩.Coats病在临床表现与形态学上具有显著的多样性,近一个世纪以来,随着对该病认识地不断深入,国内外玻璃体视网膜专家对Coats病的诊断与治疗也产生了新的看法,本文将着重对Coats病的诊断与治疗进展作一综述.     

Vitrektomie bei fortgeschrittenem Morbus Coats

BACKGROUND: Coats disease is a retinal vasculopathy of unknown cause. Untreated cases usually lead to an exudative retinal detachment and rubeosis iridis with secondary glaucoma. Photocoagulation and/or cryotherapy are generally the first interventions in treating the disease. Pars plana vitrectomy may be indicated in cases of vitreous hemorrhage or retinal detachment. METHODS: We performed pars plana vitrectomy in 9 eyes with Coats disease between 1992 and 1999. A retinal detachment was present in 3 cases, and three showed a vitreous hemorrhage. In two cases surgery was indicated because of paramacular localization of the pathological vessels with associated exudations. RESULTS: The two cases with paramacular involvement showed improvement in visual acuity of eight lines. In the remaining cases visual acuity remained within two lines compared to the initial visual acuity. All eyes except one could be saved. CONCLUSION: Pars plana vitrectomy is a useful option in treating advanced Coats disease, especially in cases associated with vitreous hemorrhage or retinal traction.     

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 disease of adult onset

CASE REPORT: We report a 66-year-old male who exhibited an exudative retinal vasculopathy, diagnosed as Coats disease of adult onset, treated with laser photocoagulation. DISCUSSION: Most cases of Coats disease are diagnosed in childhood. When this entity appears in adults it usually exhibits a more benign clinical course. Diagnosis of this condition must be considered in those patients showing a retinal vasculopathy with a marked exudative component. Treatment is indicated if the fovea is threatened by lipid deposition.     

Coats病的手术治疗

目的总结手术治疗Coats病的效果。方法对Coats病伴不同程度渗出性视网膜脱离的患者16例17只眼行巩膜外冷凝术和玻璃体手术治疗，手术后随访时间4.25～62.25个月, 平均随访时间13.10个月。结果手术治疗后8只眼视网膜完全复位(无硅油充填)，视网膜复位率47%；1只眼在硅油充填的情况下视网膜在位；8只眼手术后视网膜未能完全复位。5只眼手术后视力提高，2只眼视力稳定，7只眼视力下降。手术并发症有一过性渗出性视网膜脱离加重，视网膜前局限性增生，白内障形成，继发青光眼和玻璃体积血。结论Coats 病伴视网膜脱离经手术治疗后大多数病例视网膜可复位，部分患者视力提高。(中华眼底病杂志,2005,21:145-147)     

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。     

Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture

PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines. METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease. RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass. CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.     

Unilateral Coats’-like disease and an intragenic deletion in the TERC gene: A case report

We report a case of a 25-year-old woman with unilateral Coats’-like disease. Her brother was previously diagnosed with an autosomal dominant form of dyskeratosis congenita. Genetic testing was performed by screening the TERC gene for mutations and identified heterozygosity for the n.68_124del mutation. Our case demonstrates that the exudative retinopathy seen in Coats’-like disease can be caused by mutations in a telomere-capping gene TERC as a part of the dyskeratosis congenita spectrum without other systemic involvement. This is an interesting case that illustrates that retinal Coats’-like involvement can be the first manifestation of dyskeratosis congenita.     

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.     

Serous macular detachment secondary to distant retinal vascular disorders

PURPOSE: To report tomographic features of macular edema associated with distant retinal vascular disorders. METHODS: Six eyes with macular edema (three with extramacular branch retinal vein occlusion and three with Coats disease) underwent fluorescein angiography to detect dye leakage and optical coherence tomography to evaluate the macular features and measure the foveal height (distance between inner retinal surface and retinal pigment epithelium) and the retinal thickness (thickness of neurosensory retina) at the central fovea. RESULTS: Fluorescein angiography showed widespread nonperfusion and retinal vein staining in the eyes with a branch retinal vein occlusion not involving the macula. In eyes with Coats disease, nonperfused areas, telangiectatic retinal vessels, and aneurysms were seen in the peripheral fundus on fluorescein angiography. In one eye with Coats disease, telangiectatic vessels and aneurysms were seen in the nasal mid periphery of the optic disk. There was no dye leakage in the macular area in any eyes. Optical coherence tomography showed subretinal fluid and retinal swelling at the fovea in all eyes. The foveal height ranged from 300 to 697 microm (mean, 478 microm) and retinal thicknesses ranged from 170 to 280 microm (mean, 233 microm). CONCLUSION: Distant retinal vascular leakage causes serous retinal detachment in the macula. The macula is predisposed to collect subretinal fluid although retinal vascular leakage is far from the macula.     

Classification and management of Coats disease: the 2000 Proctor Lecture

PURPOSE: To review the methods and results of management in a large series of patients with Coats disease, to determine risk factors for poor visual outcome and enucleation, and to propose a practical classification of Coats disease. METHODS: In a retrospective consecutive series in 150 patients, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with management, including observation, laser photocoagulation, cryotherapy, and various techniques of retinal detachment surgery and enucleation. The anatomic outcome, complications of treatment, visual results, and reasons for enucleation were tabulated. Factors predictive of poor visual outcome (20/200 or worse) and enucleation were determined using Cox proportional hazards regression models. Based on these observations, a staging classification of Coats disease, applicable to treatment selection and ocular prognosis, is proposed. RESULTS: In 117 patients (124 eyes) with a mean follow up of 55 months (range, 6 months to 25 years) primary management was observation in 22 eyes (18%), cryotherapy in 52 (42%), laser photocoagulation in 16 (13%), various methods of retinal detachment surgery in 20 (17%), and enucleation in 14 (11%). Anatomic improvement or stability was achieved in 76% of eyes, and final visual acuity was 20/50 or better in 17 eyes (14%), 20/60 to 20/100 in eight (6%), 20/200 to finger counting in 30 (24%), and hand motion to no light perception in 49 (40%) Enucleation was ultimately necessary in 20 eyes (16%). Risk factors predictive of poor visual outcome (20/200 or worse) included postequatorial (P =.01), diffuse (P =.01), or superior (P =.04) location of the telangiectasias and exudation, failed resolution of subretinal fluid after treatment (P =.02), and presence of retinal macrocysts (P =.02). The main risk factors for enucleation were elevated intraocular pressure (greater than 22 mm Hg; P less than or equal to.001) and iris neovascularization (P less than or equal to.001). Coats disease was classified into stage 1, telangiectasia only; stage 2, telangiectasia and exudation (2A, extrafoveal exudation; 2B, foveal exudation) stage 3, exudative retinal detachment (3A, subtotal; 3B, total); stage 4, total detachment and secondary glaucoma; and stage 5, advanced end-stage disease. Poor visual outcome (20/200 or worse) was found in 0% of eyes with stage 1, 53% with stage 2, 74% with stage 3, and 100% of stages 4 and 5 Coats disease. Enucleation was ultimately necessary in 0% of stages 1 and 2, 7% of stage 3, 78% of stage 4, and 0% of stage 5 disease. CONCLUSIONS: Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of eyes. However, poor visual outcome of 20/200 or worse commonly results. Patients who present with stages 1 to 3 Coats disease have the best visual prognosis, and patients with stages 4 and 5 have a poor visual prognosis.     

Elevated vascular endothelial growth factor levels in Coats disease: rapid response to pegaptanib sodium

Purpose The purpose was to report a case of elevated intraocular vascular endothelial growth factor (VEGF) levels in a 2-year-old boy with stage 4 Coats disease. Design Interventional case report. Methods A 2-year-old boy with severe Coats disease and complex retinal detachment refractory to surgical intervention underwent two injections of intravitreal pegaptanib sodium in his right eye 8 weeks apart. Results After intravitreal injection there was a marked reduction in VEGF (908 to 167 pg/ml) levels and improvement of exudation, hemorrhage, and nearly complete reattachment of his retina within 3 weeks of treatment. The patient remains stable 6 months post-treatment. Conclusions Coats disease may have a component of dysregulation of VEGF-mediated angiogenesis as evidenced by elevated levels in this patient.     

Novel transscleral sutureless subretinal fluid drainage using a 25-gauge trocar-cannula with a self-closing valve in patients with advanced Coats disease

AIM: To assess surgical outcomes of a novel method of transscleral drainage of subretinal fluid using a 25-gauge trocar-cannula with a self-closing valve (DTV) in patients with severe exudative retinal detachment (ERD) in Coats disease. METHODS: Retrospective consecutive cases of 20 patients (20 eyes) of severe ERD due to Coats disease (stage 3B) in total 156 Coats patients between June 2015 and April 2019 were included in this study. The participants were aged 1 to 10y with a mean age of 3.50±1.79y. The mean follow-up time were 11.9mo. Subretinal fluid was drained transsclerally using a novel method of DTV. The height of the retinal detachment and the regression of abnormal vessels including telangiectasias and aneurysms were observed. Complications including vitreoretinal fibrosis, tractional retinal detachments (TRD), endophthalmitis, retinal holes, and hemorrhages were evaluated. RESULTS: Following surgeries, the patients showed the replacement of ERD and regression of telangiectatic retinal vessels observed with binocular indirect ophthalmoscopy. Six patients received retinal cryotherapies and 12 patients received laser photocoagulations following first external subretinal fluid drainage using DTV. All patients underwent intravitreal anti-vascular endothelial growth factor therapies to induce residual subretinal fluid absorption. During follow-ups, 8 patients underwent a second drainage operation, 17 patients received retinal laser photocoagulations and 7 patients received cryotherapies. Vitreoretinal fibrosis was found in 7 patients and 6 patients underwent micro-invasive vitrectomies during the follow-up period. Severe TRD, iatrogenic retinal holes, and hemorrhages were not found. CONCLUSION: The authors present a new therapeutic approach that successfully drains subretinal fluid in advanced stage 3B Coats disease with severe ERD. This is a simple, safe and less invasive approach when compared with traditional managements. However, it should be strictly selected for patients with high bullous ERD close to the central axis of the eye in order to avoid the complication of retinal holes.     

Coats病的超声影像特征及血流动力学分析

目的 探讨Coats病的超声影像学及血流动力学特征.方法 前瞻性、自身对照研究.对18例(18只眼)临床确诊的Coats病患者,采用A超测量眼轴长度;彩色多普勒超声(CDFI)观察眼部病灶的形态改变,同时与彩色眼底图像进行比较.CDFI检测患者眼动脉(OA)、视网膜中央动脉(CRA)及睫状后动脉(PCA)的收缩期峰值血流速度(PSV)和舒张末期血流速度(EDV)、单位时间内平均最大血流速度(TAMX)、搏动指数(PI)及阻力指数(RI).以患者的对侧健眼作为对照组.Coats病组与对照组的眼轴长度和血流参数比较,采用两样本均数比较t检验.结果 Coats病组眼轴长度(21.36±1.19)mm,较对照组(22.92±1.09)mm缩短,差异有统计学意义(t=3.617,P<0.05).Coats病组超声影像改变包括视网膜脱离、视网膜囊肿、视网膜下机化形成等,少数患眼有视网膜前增殖膜,玻璃体一般无混浊,偶见病灶内"钙化斑"及眼球壁骨化现象.Coats病组血流频谱未见明显异常,其CRA的PSV(6.96±1.21)cm/s和EDV(1.99±0.36)cm/s,较对照组PSV(8.30±1.46)cm/s和EDV(2.48±0.47)cm/s下降,差异有统计学意义(t=2.995,3.476,P<0.05),其他血流参数无明显异常.结论 Coats病为先天性疾病,可能影响患者眼球发育.超声检查有助于Coats病的诊断与鉴别诊断.Coats病患者可能存在视网膜中央动脉灌注不良,其血流动力学改变尚需进一步观察研究.     

Coats病的病理机制及治疗研究进展

Coats病是以视网膜毛细血管异常扩张和视网膜内层及外层渗出为特征,大多见于男性青少年,目前并没有完全明确的病因及病理机制。Coats病常见的治疗方法有激光光凝,冷冻疗法,药物治疗,联合治疗(抗VEGF药物联合激光),手术治疗(单纯玻璃体切除和或巩膜外放液)以及中医药等新兴治疗方法。本文主要从中西医对Coats病病因病理机制的认识及中西医治疗方法进行系统综述。     

Coats disease

Coats disease is an idiopathic disorder characterized by an abnormal development of retinal vessels (telangiectasia), with a progressive deposition of intraretinal or subretinal exudates, leading to exudative retinal detachment. Classically is isolated, unilateral and affects mainly boys between 4 to 8 years. The diagnostic methods include direct and indirect opthalmoscopy, fluorescein angiography, ultrasonography, CT scan, MR imaging, especially ?n the advanced stages of disease, when is difficult to differentiate from retinoblastoma. The treatment includes laser therapy of abnormal leaking retinal vessels and cryotherapy ?n early stages, more advanced stages require surgical techniques of retinal reattachment. Stabilization of the disease course or clinical improvement can be achieved using a carefully selected therapy.     

Coats disease

In spite of an unclear definition of Coats disease, this angiomatosis can be defined by the presence of retinal telangiectasis of nondetectable etiology, complicated by severe exudative phenomena. The diagnosis of this serious affection remains difficult because of its numerous clinical forms, which vary according to the age at which the disease appears and the progressive nature of the exudative phenomena. The consequences on the young child are all the more dangerous because it is too often discovered late. It seems necessary to attempt to stop the progression of Coats disease by destroying the telangiectasis responsible for intra- and subretinal exudation. No coagulation technique has been shown to be more effective than others and the predominance of heterogeneous series in the literature makes it difficult to evaluate the therapeutic results. In cases of severe retinal detachment, these coagulations can only be done after the often difficult drainage of the subretinal fluids.     

Adult Coats' disease in branch retinal vein occlusion

We report a case of adult Coats disease secondary to a branch retinal vein occlusion. Angiograms show the relationship of the peripheral telangiectasis, aneurysm formation and vascular incompetence to the peripheral retinal ischaemia. The possible role of vascular remodelling in the pathogenesis of this uncommon complication of a common condition is discussed.