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。

Analysis of clinical features in children with congenital corneal opacity

Objective To investigate the clinical features of children with congenital corneal opacities(CCO),to provide evidence for its diagnosis and treatment.Methods Clinical data of CCO children who received operation in our hospital from January 1,2017 to December 31,2017 were retrospectively analyzed.The data included general features,clinical classification,unilateral or bilateral affected eye(s),associated ocular and systemic abnormalities,pathological changes and received treatment.Results Totally 66 patients(93 eyes)were included in the study,and with mean age of 3-36(13.11±9.63)months.There were 39 patients(59.1%)with unilateral CCO.The common clinical features were Peters abnormality(32 patients,48.5%)and sclerocornea(15 cases,22.7%).Synechia(61 eyes,65.6%)and cataracts(22 eyes,23.7%)were the two most common ocular abnormalities.Systemic abnormalities were present in 5 patients(7.6%),including growth retardation(4 patients)and congenital brain defects(1 patient).Eighty-five eyes(91.4%)in 93 eyes underwent penetrating keratoplasty,and lamellar keratoplasty was performed in 6(6.5%)eyes.Conclusion Peters abnormality and sclerocornea are the common clinical features of CCO children at 3 years of age and under.Besides of corneal opacity,CCO patients may also have ocular abnormalities such as synechia and cataract,and systemic abnormalities such as growth retardation.Penetrating keratoplasty is the main operation of CCO.