Congenital corneal anesthesia: A case series

Congenital corneal anesthesia (CCA) is an extremely rare condition where the cornea is affected in isolation or as a part of congenital syndrome, or can be associated with systemic anomalies. This case series of 12 eyes provides an overview of various clinical presentations and their final treatment outcomes. The average age of presentation was 3.2 years with a female preponderance (75%). Fifty percent of the patients had bilateral involvement and 50% had corneal ulcers at presentation. Two eyes required therapeutic keratoplasty for corneal perforation. All patients had isolated CCA except for one who had an associated hereditary and sensory autonomic neuropathy.     

Congenital corneal anesthesia

Congenital corneal anesthesia is a rare clinical entity that poses a diagnostic dilemma, particularly in the pediatric age group. The sensory deficit may be confined to the cornea, or extend to other divisions of the trigeminal nerve. The sensory deficit may occur as an isolated abnormality, as part of a complex neurological syndrome, or it may occur in association with multiple somatic abnormalities and congenital insensitivity to pain. This condition usually presents between the ages of 8 to 12 months. Poor vision, photophobia, conjunctival injection, and corneal ulceration in the absence of pain and distress in a child should alert the clinician to the possibility of anesthetic cornea. In the early stages of presentation, punctuate keratopathy is the main feature, which may progress to non-healing persistent corneal epithelial defects. This stage may progress to acute corneal lysis and perforation. In most patients, conservative approaches such as copious lubrication, prevention of self-harm and cautious use of bandage contact lenses are effective in preventing progressive corneal damage. Tarsorrhapy is effective in promoting epithelial healing and permanent lateral tarsorraphy may prevent further development of epithelial defects. Amniotic membrane graft may be considered in order to improve epithelial healing. Corneal grafts carry a poor prognosis. Accurate initial diagnosis, evaluation, and proper management are paramount to prevent visual loss due to long-term complications of corneal anesthesia. This review of the literature outlines the problems and approaches in diagnosis, evaluation, and management of this rare condition.     

先天性角膜混浊患儿的临床特征分析

目的分析先天性角膜混浊(congenital corneal opacities,CCO)患儿的临床特征,为其诊断及治疗提供依据。方法回顾性分析2017年1月1日至12月31日在我院接受手术治疗的CCO患儿的临床资料,包括CCO患儿一般特征及临床分类、患病单双侧、相关的眼部和全身异常、病理改变和接受的治疗等资料。结果本研究纳入66例(93眼)CCO患儿,其中年龄3～36(13.11±9.63)个月,39例(59.1%)为单眼患病,最常见的临床类型是Peters异常(32例,48.5%)和角膜巩膜化(15例,22.7%);虹膜粘连(61眼,65.6%)和白内障(22眼,23.7%)是两种最常见的眼部异常;5例(7.6%)患儿出现全身异常,包括生长迟缓(4例)和先天性脑缺陷(1例)。93眼中85眼(91.4%)进行了穿透性角膜移植术,6眼(6.5%)进行了板层角膜移植术。结论年龄≤3岁的CCO患儿中最常见的临床类型为Peters异常及角膜巩膜化;除角膜混浊外,CCO患儿还可能存在虹膜粘连、白内障等眼部异常或生长迟缓等全身异常;穿透性角膜移植术为治疗CCO的主要手术方法。     

先天性白内障术后继发青光眼与中央角膜厚度的研究

目的:探讨儿童先天性白内障手术后无晶状体眼或人工晶状体眼的平均中央角膜厚度(CCT)的改变,并与同年龄组健康人群的中央角膜厚度进行对照研究。方法:该实验包括随机抽样的先天性白内障术后的45个无晶状体眼及人工晶状体眼患者共45眼,同时有46个健康的进行性别和年龄配比的志愿者作为对照研究,对他们进行包括中央角膜厚度及眼压(Goldmann眼压计及Tonopen眼压计)的全面的眼部检查。将测得的眼压与角膜中央厚度结果进行分组比较。结果:研究对象的45眼中包括35只无晶状体眼与10只人工晶状体眼,其角膜厚度中位数为626μm(范围为523~870μm),而正常对照组的中央角膜厚度为556.0μm(范围为490~640μm)其差异有统计学意义(P<0.05);先天性白内障患儿术后无晶状体眼与手术时一期植入人工晶状体的眼之间的中央角膜厚度的差异有统计学意义(P=0.011)。但先天性白内障术后的患儿中二期植入人工晶状体眼与无晶状体眼之间中央角膜厚度的差异则不具备统计学意义(P=0.847)。患者的年龄中位数为36mo(年龄从1wk到10岁不等),且其年龄与CCT之间呈负相关(r=-0.485,P=0.001)。结论:儿童先天性白内障术后无晶状体眼与人工晶状体眼的患者显著比正常对照组的角膜变厚。这种差异对解释术后眼内压升高有重要的作用,同时对儿童先天性白内障无晶状体眼患者术后最常见的继发青光眼的并发症的解释有重要的作用。     

接触镜相关性角膜敏感性变化的机制研究

目的:针对配戴不同类型接触镜后角膜敏感性变化的机制进行研究,以期对接触镜的临床验配和并发症的早期发现与处理提供帮助。方法:采用问卷的形式收集基本信息,应用Cochet and Bonnet aesthesiometer测量49例98眼角膜敏感性,按照不同分组进行比较分析。结果:分别配戴RGP和软镜,戴镜1～12mo组,1～5a组中央部角膜敏感性均较未戴镜组明显下降(P=0.000),而二组之间差异无显著意义(RGP镜片P=0.921,软镜P=0.685);配戴Dk为36和82的RGP镜片1～12mo,二者差异无显著意义(P=0.263);分别配戴RGP镜片和软镜1～12mo,1～5a,两组中央角膜敏感性差异无显著意义(1～12mo组P=0.263,1～5a组P=0.366)。结论:配戴接触镜导致的角膜敏感性下降是多种机制共同作用的结果,不同类型镜片的机制略有不同。     

组织工程角膜治疗兔无菌性角膜溃疡

目的:探讨利用组织工程技术制备角膜基质进行板层角膜移植治疗无菌性角膜溃疡的可行性和疗效。方法:用去垢剂联合胰酶、DNA-RNA酶去除猪角膜基质细胞后制备成组织工程角膜基质;将16只兔的角膜基质内植入壳聚糖膜使之形成角膜溃疡,随机从16只兔中选8只行组织工程角膜基质板层移植;另外8只作为对照组,行新鲜的同种异体板层角膜移植。术后对角膜进行裂隙灯、HE染色光学显微镜检查。结果:组织工程角膜基质移植后无免疫排斥发生,术后8～10wk角膜溃疡完全修复,角膜恢复透明性,与对照组疗效相同。结论:组织工程角膜基质具有良好的生物相容性和治疗作用。     

Corneal sensitivity and morphology of the corneal subbasal nerve plexus in primary congenital glaucoma

Background

To quantify the corneal subbasal nerve density and the total number of nerve fibers in primary congenital glaucoma (PCG) and to evaluate their impact on corneal sensitivity.

Methods

Forty eyes of 26 PCG patients were compared with 40 eyes randomly selected from 40 non-glaucoma patients who populated the control group. Central corneal sensitivity (CCS) was assessed by means of Cochet–Bonnet esthesiometry. The mean subbasal nerve density and the total number of nerve fibers were quantified by laser-scanning confocal microscopy. Normality of data was assessed by Kolmogorov–Smirnov testing. Differences in parameters were assessed with Student''s t-test, while correlations with CSS were assessed with Pearson''s correlation.

Results

Significant differences were identified in the mean subbasal nerve density (2108±692 μm in PCG, 2642±484 μm in controls, P=0.003) and in the total number of nerve fibers (12.3±4.2 in PCG, 15.4±3.1 in controls, P=0.02). Both groups presented comparable mean CCS and tortuosity. Both groups presented strong correlations between CCS and mean nerve density (r=0.57 in PCG, r=0.67 in controls, all P<0.05), and between CCS and total number of nerve fibers (r=0.55 in PCG, r=0.56 in controls, all P<0.05).

Conclusion

PCG exerts significant changes in both the mean subbasal nerve density and the total number of nerve fibers. However, these changes do not appear to affect central corneal sensitivity.     

Improvement in corneal scarring following bacterial keratitis

Aim

Bacterial keratitis results in corneal scarring and subsequent visual impairment. The long-term evolution of corneal scars has not been well described. In this case series, we identified patients who had improvement in corneal scarring and visual acuity from a clinical trial for bacterial keratitis.

Methods

We searched the records of the Steroids for Corneal Ulcers Trial (SCUT) for patients who had improvement in vision between the 3-month and 12-month visits and reviewed their clinical photographs.

Results

Of the 500 patients enrolled in SCUT, five patients with large central corneal scars due to bacterial keratitis are presented. All experienced improvement in rigid contact lens–corrected visual acuity from months 3 to 12. All patients also had marked improvement in corneal opacity during the same time period. None of the patients opted to have penetrating keratoplasty.

Conclusions

Corneal scars may continue to improve even many months after a bacterial corneal ulcer has healed. The corneal remodeling can be accompanied by considerable improvement in visual acuity, such that corneal transplantation may not be necessary.     

真菌性角膜溃疡45例临床分析

目的:探讨真菌性角膜溃疡的临床表现及综合治疗。方法:确诊真菌性角膜溃疡45例,进行抗真菌综合治疗,观察治疗转归,探索有利于缩短真菌性角膜溃疡的临床治疗措施。如刮除溃疡组织、碘酊烧灼、生理盐水冲洗、积极抗真菌治疗、手术等。结果:真菌性角膜溃疡病程较长,常伴有前房炎症反应,用药治疗有效时自觉症状好转,病情开始转向稳定,溃疡面趋平,有少量新生血管长入,愈合后留有薄翳、斑翳或白斑,对视力有不同程度影响。发现晚、治疗不及时或中断的患者则预后较差。结论:真菌性角膜溃疡的治疗有赖于早期诊断明确,及时治疗,规范用药。且疗程要长,临床上在未明确角膜炎症性质前应注意慎用激素和抗生素。     

52例真菌性角膜溃疡临床观察

真菌性角膜溃疡是眼科临床较为常见而治疗较为棘手,致盲率较高的眼部疾患之一。通过对５２例真菌性角膜溃疡的发病原因、诊断和治疗方法进行探讨,说明它的早期诊断、正确治疗是痊愈的关键。另外,近年来基层所对抗生素,激素的滥用是本病增多及治疗困难的一个主要原因,应引起临床医生的重视,做好农民植物性眼外伤的预防及处理。     

角膜移植术治疗真菌性角膜溃疡临床报告

目的：探讨角膜移植术治疗真菌性角膜溃疡的手术适应证及其临床效果。方法：对临床上经刮片或共焦显微镜检查，确诊为真菌性角膜溃疡和疑为真菌性角膜溃疡患者，给予局部及全身抗真菌治疗，对病情不能控制，角膜溃疡直径≥7mm的患者55例，用甘油或干燥保存的角膜组织行角膜移植术，对术中取下的角膜做组织病理学检测。结果：随访3～6个月，55例(55只眼)均经一次手术有效控制角膜炎症，成功恢复角膜外形。视力有进步者48例，其中视力可达0．1～0．3者16例。55例真菌培养均阳性。经鉴定，镰刀菌属35例，曲霉菌属13例，烟曲菌属2例，孢子菌属2例，其它丝状菌属3例。共焦显微镜检查，显示出无损伤，快速，阳性率高的优点。结论：对真菌性角膜溃疡患者，及时应用甘油或干燥保存的角膜组织行手术治疗，不仅控制炎症，缩短病程，且为复明奠定基础，因此具有重要临床应用价值。     

婴儿先天性白内障摘出术后早期中央角膜厚度变化与眼压的相关性研究

目的　测量并观察生后１２个月以内行先天性白内障摘出术患儿术后术眼中央角膜厚度（ｃｅｎｔｒａｌｃｏｒｎｅａｌｔｈｉｃｋｎｅｓｓ,ＣＣＴ）及眼压（ｉｎｔｒａｌｏｃｕｌａｒｐｒｅｓｓｕｒｅ,ＩＯＰ）的变化。方法　选取２６例（４９眼）生后１２个月内行白内障摘出术的先天性白内障患儿为观察组,按行手术时月龄分为三组:１组:月龄≤３个月,２组:３个月＜月龄≤６个月;３组:６个月＜月龄≤１２个月。分别在术前、术后１个月、３个月、６个月时测量术眼ＣＣＴ与ＩＯＰ。另选取与各观察组最后一次随访时的患儿年龄与性别相匹配的相对正常眼婴幼儿作为对照组。结果　观察组术后１个月、３个月、６个月的ＣＣＴ为（５７８．３９±４７．７９）μｍ、（５８１．３４±５１．５４）μｍ、（５８８．２９±４９．９０）μｍ,均显著大于术前（５６３．５６±３７．１４）μｍ及对照组（５３４．９６±４０．３４）μｍ（均为Ｐ＜０．０５）。观察组术前、术后１个月、３个月、６个月ＩＯＰ测量值间差异无统计学意义（Ｐ＞０．０５）,观察组术后６个月ＩＯＰ测量值大于对照组（Ｐ＜０．０５）。观察组术后６个月ＣＣＴ与ＩＯＰ线性相关（ｒ＝０．５３８,Ｐ＝０．００４）。结论　婴儿先天性白内障摘出术后早期的ＣＣＴ显著大于正常同龄婴儿,且与ＩＯＰ线性相关。     

带蒂结膜瓣移植术在角膜溃疡治疗中的应用研究

目的探讨带蒂结膜瓣移植治疗角膜溃疡的临床效果。方法对56例药物治疗无效的难治性角膜溃疡行病灶清除及带蒂结膜瓣移植术,合并穿孔者行带眼球筋膜囊的改良带蒂结膜瓣移植术,术后针对原发病进行药物治疗。结果 53例1次手术治愈角膜溃疡,达到了保存眼球的目的。3例术后结膜瓣脱落,反复行结膜瓣遮盖术2～3次,但最终角膜融解。结论对于药物治疗无效的难治性角膜溃疡,带蒂结膜瓣移植术仍是有效的治疗方法,尤其是对角膜穿孔者,可以控制炎症,保存眼球,并为进一步行角膜移植术创造条件。     

角膜移植治疗真菌性角膜溃疡的临床观察

探讨角膜移植治疗真菌性角膜溃疡的有效性及影响因素。方法：应用角膜移植治疗真菌性角膜溃疡４４例４４只眼,穿透性角膜移植２９例２９只眼,板层角膜移植１５例１５只眼,３３例为８ｍｍ以上的大植片角膜移植。结果：４１例感染控制并保存了眼球,３２全视力较术前有不同程度提高,１８例获得０．０５以上有用视力,１例穿通性角膜移植术后最佳矫正视力达１．０,１例板层角膜移植术后最佳矫正视力达０．６。３例角膜严重感染者,     

先天性白内障儿童眼球发育的观察

目的通过观察眼轴和角膜屈光力,了解先天性白内障儿童的眼球发育状况.方法将64例单眼先天性白内障和107例双眼先天性白内障患儿分为1岁以下、1～3岁、4～5岁、6～9岁、10岁以上5个年龄组比较分析其眼轴和角膜屈光力情况.结果在P＝0.05水平上只有1～3岁组单眼先天性白内障患眼眼轴较健眼有显著性差异;单眼先天性白内障与双眼先天性白内障患眼轴长度无显著性差异;64.4%先天性白内障眼眼轴长于正常同龄儿童均值;单眼先天性白内障患儿的患眼与健眼间角膜屈光力无显著性差异.结论形觉剥夺只是影响先天性白内障眼轴发育的诸多因素之一,为儿童先天性白内障选择人工晶状体屈光度时也应考虑其他因素对眼轴发育的影响.     

那特真(Natacyn)治疗真菌性角膜溃疡的疗效观察

目的 观察那特真(Natacyn)治疗真菌性角膜溃疡的疗效。方法 用那特真滴眼治疗真菌性角膜溃疡23例,第一天每1小时一次;第二日起至2周内每2小时一次,持续2周後根据病情给于每日6～8次滴眼。结果 本组23例中治愈18例,占78.3％,其中视力达0.1以上者10例。有效5例,占21.7％。结论 那特真是治疗浅层真菌性角膜溃疡较为理想的药物。     

酮康唑粉剂联合润尔乐治疗真菌性角膜溃疡

目的 探讨酮康唑粉剂与润尔乐眼药水联合局部应用治疗真菌性角膜溃疡的效果。方法 对30例(30眼)真菌性角膜溃疡患者,用酮康唑粉剂与润尔乐眼药水混合局部涂抹于角膜溃疡处。结果 除1例重型因角膜穿孔和眼内炎行眼内容物剜出术外,其余29例均取得明显治疗效果。结论 酮康唑粉剂联合润尔乐眼药水治疗真菌性角膜溃疡疗效好,作用快,方法简便,无明显毒副作用。     

改良角膜活检法对真菌性角膜溃疡的临床诊断

目的对角膜活检加178．2mmol·L－1（100g·L－1）氢氧化钾法诊断真菌性角膜溃疡的阳性率进行评价。方法对80例临床诊断真菌性角膜溃疡患者,在行穿透性角膜移植前,角膜刮片、角膜活检加178．2mmol·L1氢氧化钾湿片法和角膜移植术后组织病理学染色的结果进行比较。结果角膜活检加178．2mmol·L－1氢氧化钾湿片法诊断真菌性角膜溃疡阳性率为97．5％．结论角膜活检加178．2mmol·L1氢氧化钾湿片法是一种快速、便捷、阳性率高的诊断真菌性角膜溃疡的方法。