背景 Peters异常是临床上罕见的眼病,且容易与一些先天性眼科异常疾病相混淆.了解Peters异常的临床特征和治疗方法有助于对其进行及时的诊治,改善患者的预后. 目的 总结Peters异常患儿的临床体征和治疗方法,为其诊断和个性化治疗提供依据.方法 采用描述性研究方法,对2014年1月1日至2016年3月30日在北京儿童医院眼科诊治的Peters异常患儿4例8眼的临床资料进行回顾性分析.纳入的患儿均行手持裂隙灯显微镜下眼前段检查、角膜曲率和角膜厚度测定及眼轴长度和眼压测量,对患儿全身和眼部的临床特征及针对患儿的不同情况所采取的不同治疗方式进行总结和分析. 结果 根据患儿的眼部和全身表现及文献报道的标准可将Peters异常分为Ⅰ型、Ⅱ型和Peters plus综合征.从纳入患儿的临床特征来看,纳入的4例8眼均有角膜白斑、周边房角与角膜的粘连、前房较浅或消失,2例2眼仅有上述表现,属于Ⅰ型Peters异常;6例6眼除上述症状外,出现晶状体前囊中央混浊,并且前囊与角膜内皮粘连,属于Ⅱ型Peters异常.测量的4眼角膜厚度均较厚,平均为(680±127) μm;测量的4眼角膜曲率均平坦,平均为(37.40±1.79)D;测量的4眼眼轴均较短,长度为(21.06±0.19) mm;8眼角膜直径均较小,平均为(9.4±0.5) mm;4眼有虹膜缺损,4眼无虹膜;1例内斜,3例眼球震颤.3例患儿除Ⅱ型Peters异常眼部表现外,还存在全身神经源性发育异常,诊断为Peters plus综合征.根据患眼的具体情况制定治疗方案:术前眼压高于30 mmHg(1 mrnHg=0.133 kPa)的2眼行小梁切除术和前部玻璃体切割术,术后眼压降至21 mmHg以下;角膜中央明显混浊的1眼行穿透角膜移植术,术后1个月角膜植片发生排斥反应,术后3个月植片完全混浊;4眼合并晶状体混浊者行晶状体摘出术,2眼植入人工晶状体,2眼行光学虹膜切除术,术后视力均有所改善. 结论 Peters异常的主要临床特征是角膜白斑、浅前房和虹膜周边与角膜粘连,伴有晶状体混浊者一般是晶状体前囊膜混浊并与角膜内皮粘连.需要手术的患眼需要根据具体情况个性化设计手术方案.%Background Peters anomaly is a rare eye disease.Understanding the clinical features of Peters anomaly is helpful for us to correctly manage this disease.Objective This study was to investigate the clinical characteristics and treatment methods of Peters anomaly and provide available basis for the diagnosis and individualized treatment of Peters abnormal.Methods A descriptive study was performed.The clinical data from 8 eyes of 4 patients with Peters anomaly who were diagnosed and treated in Beijing Children's Hospital from January 1,2014 to March 30,2016 were retrospectively analyzed.The clinical manifestations of the anterior ocular segment were examined by handheld slit-lamp microscope,and corneal curvature,corneal thickness,axial length and intraocular pressure were measured.The therapy and outcomes of the patients were evaluated.Results Peters anomaly was classified into type Ⅰ,type Ⅱ and Peters plus syndrome based on the findings and literature'criteria.Regarding to the clinical findings,the adhesion of peripheral angle with cornea,shallow or disappeared anterior chamber were found in all the eyes,and these manifestations were type Ⅰ Peters anomaly and occurred in 2 eyes of 2 patients.The opacity and adhesion of the central lens capsula to corneal endothelium was seen in 6 eyes which belonged to type Ⅱ Peters anomaly.The mean corneal thickness was (680± 127) μm in 4 measured eyes,which was thicker than normal eyes;the mean corneal curvature was (37.40±1.79)D in 4 measured eyes;the mean axial length was (21.06±0.19)mm and the mean diameter of cornea was (9.4 ± 0.5) mm in 4 measured eyes.Iridocoloboma was found in 4 eyes and congenital aniridia was in 4 eyes.In addition,esotropia appeared in 1 patient,and nystagmus occurred in 3 patients.The 3 patients of type Ⅱ in this study were found to have the disorders associated with systemic neurogenic developmental abnormalities and diagnosed as Peters plus syndrome.Trabeculectomy with anterior vitrectomy was performed in 2 eyes with the intraocular pressure ＞30 mmHg(1 mmHg =0.133k Pa) and the introcular pressure reduced to below 21 mmHg after operation.Penetrating corneal transplantation was carried out on 1 eye with central opacity.However,rejection of graft occurred at 1 month and became complete cloudy at 3 months after surgery.Cataract extraction surgery was performed on 4 eyes with lens opacity and intraocular lens were implanted in 2 eyes or iridectomy was carried out in another 2 eyes,and the visual quality was evidently improved after operation.Conclusions The clinical characteristics of Peters anomaly include leukoma,shallow anterior chamber,peripheral iris adhesion with cornea and lens opacity due to adhesion of lens anterior capsule to corneal endothelium.Personalized operative regimen should be determined based on the clinical findings of the eyes with Peters anomaly.
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