Cogan—Reese综合征伴面部血管瘤

本文报告一例合并有颜面部血管瘤的Ｃｏｇａｎ－Ｒｅｅｓｅ综合征，讨论了该病的临床生理学特点及发病机制。角膜内皮细胞异常增生形成的细胞性膜为原发病变，虹膜改变及虹膜前粘连属继发病变，支持角膜内皮细胞异常是由于神经嵴细胞组织发生异常增殖的学说。治疗主要针对眼压升高及角膜水肿，降眼压是基本措施，滤过性手术可早期施行。     

单眼虹膜角膜内皮综合征患者对侧眼的临床表现

目的　了解单眼虹膜角膜内皮 (iridocornealendothelial,ICE)综合征患者对侧眼的临床表现。方法　观察有病历记录的 42例临床确诊为单眼ICE综合征患者对侧眼的临床表现。对其中 1 9例ICE综合征患者对侧眼的屈光状态、眼压、裂隙灯检查、房角镜检查、超声生物显微镜检查结果进行分析。并将 42例中的 35例ICE综合征患者对侧眼的角膜内皮细胞计数结果与 35例年龄匹配的正常人角膜内皮计数进行比较 ,t检验分析两组间角膜内皮细胞密度、六角形细胞比例及细胞大小变异度的差异。结果　在 1 9例ICE综合征患者的对侧眼中 ,88 9%为正视眼或远视眼 ,1例高眼压 ,50 0 %(6/ 1 2 )前房深度 <2 2mm ,3例虹膜萎缩或发育欠佳 ,3例前房角窄Ⅱ～窄Ⅲ和 (或 )前房角粘连关闭 ,2例前房角锥状粘连 ,1例视野检查发现相对旁中心暗点 ,31 6 %病例合并全身性疾病。 35例ICE综合征患者对侧眼的平均角膜内皮细胞密度为 (2 593 4± 432 7)个 /mm2 ,低于正常对照组 (2 784 9± 2 80 6)个 /mm2 ,两者比较差异有显著意义 (t=- 2 43 ,P =0 0 1 9) ;六角形细胞的百分率为 (56 5± 6 4) % ,低于正常对照组的 (58 8± 5 7) % ,两组比较差异无显著意义 (t=- 1 0 0 5 ,P =0 332 ) ;细胞大小变异度为 0 332± 0 0 3 ,低于正常对照组     

Cogan—Reese综合征伴面部血管瘤

Ｃｏｇａｎ－Ｒｅｅｓｅ综合征伴面部血管瘤梁伟亮胡小玲广东省开平市第一人民医院（５２９３００）Ｃｏｇａｎ－Ｒｅｅｓｅ综合征临床上少见，伴面部血管瘤更罕见，现报告１例及其治疗效果。患者张××女３０岁左眼视朦，间歇性眼球胀痛３个月，间有恶心、雾视，于１９９...     

微孢子虫性角膜基质炎

微孢子目是形成孢子的，专性的真核原和寄生物，属于微孢子门。眼部的微孢子虫病，根据感染微孢子虫种属的不同，具有特征性的眼部临床表现。脑炎微孢子虫属引起的感染局限在角膜和结膜的上皮细胞，产生弥漫性、点状的上皮角结膜炎。小孢子虫属和微孢子虫属引起的感染通常侵及包括角膜细胞在内的角膜基质。     

维吾尔族假性剥脱综合征患者角膜内皮形态学分析

目的：:对比分析维吾尔族假性剥脱综合征（PEX）、剥脱综合征性青光眼（PEXG）、原发性开角型青光眼（POAG）及年龄相关性白内障（ARC）角膜内皮形态学差异。方法：:回顾性病例对照研究。选取2019年7月至2020年8月在中国人民解放军新疆军区总医院全军眼科中心就诊治疗的维吾尔族PEX患者76例（76眼）,PEXG患...     

转录因子T-bet在单纯疱疹病毒感染小鼠外周血中的表达

目的研究单纯疱疹病毒Ⅰ型(herpes si mplex virus type1,HSV-1)感染小鼠眼球以后转录因子T-bet在小鼠外周血中的表达,探讨T-bet的表达与单纯疱疹性角膜基质炎之间的关系。方法将106空斑单位(plague forming unit,PFU)·L-1的HSV-1Mckrae毒株接种于BALB/c鼠的角膜上建立单纯疱疹性角膜基质炎(herpetic stromal keratitis,HSK)动物模型,分别于角膜接种病毒后的第1d、3d、7d、10d、14d、21d及28d,用毛细管取小鼠的左眼眼眶静脉窦血1mL,提取淋巴细胞,用半定量逆转录聚合酶链反应(RT-PCR)检测T-bet mRNA的表达水平;在裂隙灯显微镜下观察小鼠角膜的临床变化,组织学检查角膜的病理改变;用ELISA法检测T-bet蛋白在角膜组织中的表达水平。结果BALB/c鼠的角膜接种HSV-1后的1~5d,角膜擦拭液中均检测出HSV-1复制,表明小鼠感染了单纯疱疹病毒;裂隙灯显微镜观察:HSV-1感染鼠的角膜后,小鼠均患了急性角膜上皮炎,并于感染后1周内痊愈。其中81.7%(49/60)的小鼠自感染病毒后第10d起开始出现角膜基质炎改变,表现为灶状角膜基质混浊,局部角膜新生血管化,角膜基质内大量的炎性细胞浸润。角膜基质混浊逐渐进展,在病毒感染后的第14~21d达到高峰。RT-PCR检测的数据显示:未感染HSV-1的对照组小鼠的外周血中不表达T-bet mRNA;HSV-1感染小鼠的早期即可诱导T-bet mRNA在小鼠外周血中的表达,并且表达持续存在;T-bet mRNA表达的高峰时间(10~28d),位于角膜基质炎发生和发展的过程中。ELISA法检测角膜提取物中的T-bet蛋白显示了相似的结果。结论HSV-1上调T-bet mRNA在小鼠外周血中的表达;T-bet的表达与角膜基质炎的发生和发展密切相关。     

Early corneal findings in Cogan's syndrome

We evaluated the ocular manifestations of Cogan's syndrome in 13 consecutive patients. The most frequent and earliest ocular finding was bilateral peripheral subepithelial keratitis consisting of faint, nummular lesions. The subepithelial keratitis was responsive to topical administration of corticosteroids. Peripheral subepithelial keratitis was seen in four patients and strongly suspected on review of history in three additional patients. Deep stromal keratitis was seen in two patients, while only one patient developed classic findings of Cogan's syndrome, ie. interstitial keratitis with vascularization. Additionally, five patients presented with noncorneal ocular inflammatory disease and deafness (atypical Cogan's syndrome). Early subepithelial keratitis in Cogan's syndrome may be mistakenly diagnosed as trival ocular inflammatory disease, and suppressed by topical corticosteroids, resulting in delayed diagnosis of Cogan's syndrome in the absence of classic corneal findings. Early diagnosis of Cogan's syndrome is important inasmuch as prompt treatment of cochlear symptoms with systemic corticosteroids may prevent or ameliorate deafness.     

Scrolls of Descemet's Membrane in Healed Syphilitic Interstitial Keratitis

A 45-year-old woman with healed syphilitic interstitial keratitis presented with decreased vision, foreign-body sensation of the right eye, and bilateral conjunctival injection. Examination disclosed a network of tissue on the posterior corneal surface, with extension into the anterior chamber. Bullous keratopathy developed after intracapsular cataract extraction, and a keratoplasty was performed. Pathologic examination of the corneal button disclosed corneal edema, posterior stromal vascularization, much thickening of Descemet's membrane, and concentrically laminated scrolls of Descemet's membrane-like tissue that extended from the posterior corneal surface into the anterior chamber. Endothelium was attenuated considerably throughout and in one area was replaced by a single layer of iris melanocytes.     

Atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis

We report a case of atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis in a woman with ovarian cancer. A 62‐year‐old woman with ovarian cancer developed bilateral interstitial keratitis and panuveitis accompanied by bilateral sensorineural hearing loss and chondritis. Auricular cartilage biopsy ruled out relapsing polychondritis and the diagnosis of atypical Cogan's syndrome was set clinically.     

Corneal dystrophies. I. Dystrophies of the epithelium,Bowman's layer and stroma

Most corneal dystrophies are autosomal dominant, bilateral disorders that primarily affect one layer of an otherwise normal cornea, progress slowly after their appearance in the first or second decade, and are not associated with a systemic disease. Epithelial basement membrane dystrophy and Fuchs' endothelial dystrophy are seen commonly by the general ophthalmologist; fleck, posterior polymorphous, granular or lattice dystrophies are seen more rarely, and others may never be seen in general office practice. While the distinctive clinical appearance of most corneal dystrophies allows accurate diagnosis, the integration of slitlamp findings with histopathologic and biochemical findings aids in the understanding of the clinical observations and provides a more rational basis for therapy. Transmission electron microscopy is the most accurate method of histopathologic diagnosis. Epithelial dystrophies usually manifest intraepithelial cysts and abnormal basement membrane. In stromal dystrophies, an abnormal substance accumulates within the keratocytes or among the collagen fibrils; it may be an excess normal metabolite (like glycosaminoglycans in macular dystrophy), a material not usually present (like amyloid in lattice dystrophy), or a substance of unknown composition (like hyaline in granular dystrophy). Each dystrophy is illustrated with a composite drawing. Endothelial dystrophies will be reviewed separately in a second article.     

Behçet's disease

Behçet's disease is characterized by three primary components: iridocyclitis (historically with hypopyon), aphthous lesions in the mouth, and ulceration of the genitalia. Erythema nodosum, arthropathy and thrombophlebitis often accompany these manifestations, but the ocular symptoms may be the most important and serious manifestations of the disease. Central nervous system involvement, most often due to necrotizing vasculitis, may be the most protean manifestation of the disease, leading to death. The frequency of ocular manifestations is 70–85% in patients with the disease; the underlying disease mechanism in all organ systems is an occlusive vasculitis. Although the most common ocular symptom is that of anterior uveitis, often with hypopyon as a very late sign, the presence of necrotizing retinal vascular lesions is well known and often obscured by the severity of the anterior reaction. Definitions, incidence, clinical characteristics, differential diagnosis, and management of Behçet's ocular disease are discussed, as are the interrelationships of the different organ manifestations. The ophthalmologist should be familiar with the full spectrum of disease presentation since he or she may be the first physician to encounter the Behçet patient.     

The Differential Diagnosis of Descemet's Tears (Haab's Striae) and Posterior Polymorphous Dystrophy Bands: A Clinicopathologic Study

Band-like structures, clinically similar to Haab's striae, occur in posterior polymorphous dystrophy (PPMD) and are often confused with them. The difference in clinical appearance based on difference in histopathology allows a clear differentiation. The edge of Haab's striae are thickened, curled, and secondarily proliferate Descemet's membrane, while the area between the edge is thin and smooth. Posterior polymorphous dystrophy bands are the exact opposite. The band is a thickening of Descemet's membrane between the edges with thinner, more normal Descemet's membrane outside of them. PPMD bands may be associated with typical vesicles or appear as the only sign of dystrophy, even in the eyes of patients with a known family history.     

Progressive essential iris atrophy,Chandler's syndrome,and the iris nevus (Cogan-Reese) syndrome: A spectrum of disease

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome are considered to be variations of a single disease process, which is characterized by abnormalities of the cornea, anterior chamber angle, and iris. In each variation, the typical patient is a white woman with unilateral disease, negative family history, and an onset of symptoms in early to middle adulthood. Since the membrane theory of Campbell suggests that the disease is a fundamental abnormality of the corneal endothelium, rather than the iris, the term “iridocorneal endothelial syndrome”, as proposed by Yanoff, may be an appropriate inclusive term for the spectrum of disease, although further study of the pathogenesis is needed. For each variation of the disease, corneal edema and secondary glaucoma are both treated primarily by medical or surgical reduction of the intraocular pressure, although penetrating keratoplasty is occasionally required for cases with advanced corneal edema.     

Horner's syndrome with abducens nerve palsy

A 68-year-old male patient presented with a week of sudden diplopia. He had been diagnosed with nasopharyngeal cancer 8 months prior and had undergone chemotherapy with radiotherapy. Eight-prism diopter right esotropia in the primary position and a remarkable limitation in abduction in his right eye were observed. Other pupillary disorders and lid drooping were not found. After three weeks, the marginal reflex distance 1 was 3 mm in the right eye and 5 mm in the left eye. The pupil diameter was 2.5 mm in the right eye, and 3 mm in the left eye under room illumination. Under darkened conditions, the pupil diameter was 3.5 mm in the right eye, and 5 mm in the left eye. After topical application of 0.5% apraclonidine, improvement in the right ptosis and reversal pupillary dilatation were observed. On brain magnetic resonance imaging, enhanced lesions on the right cavernous sinus, both sphenoidal sinuses, and skull base suggested the invasion of nasopharyngeal cancer. Lesions on the cavernous sinus need to be considered in cases of abducens nerve palsy and ipsilateral Horner's syndrome.