先天性晶状体不全脱位是一种较为罕见的晶状体悬韧带异常的疾病，其手术治疗极具挑战性。以人工晶状体悬吊为代表的传统手术方式易出现囊袋破裂、玻璃体疝、人工晶状体脱位和继发性青光眼等严重并发症。近年来，以重建囊袋悬韧带隔为目标，新型囊袋辅助装置的应用极大程度提高了先天性晶状体不全脱位的手术成功率。然而，以改良式张力环为代表的囊袋辅助装置在我国仍难以得到普及且操作繁琐。因此，如何最大程度利用普通张力环等最常见的装备，设计出一种安全可靠手治疗先天性晶状体不全脱位的手术方式是眼科界亟待解决的问题。本文将介绍一种二期张力环缝合固定治疗先天性晶状体不全脱位手术技术。该技术仅需使用普通张力环，具有操作简单安全、术后效果稳定和易于技术推广的优点。

Congenital ectopia lentis is a relatively rare zonular disorder of the lens, and its surgical treatment is extremely challenging. The traditional surgical procedures represented by intraocular lens suspension are prone to result in serious complications such as capsular bag rupture, vitreous hernia, intraocular lens dislocation and secondary glaucoma. In recent years, with the goal of reconstructing the capsular bag–zonules diaphragm, the application of new capsular bag-assisted devices has greatly improved the surgical success rate of congenital ectopia lentis. However, the capsular-assisted devices, such as modified capsular tension ring, are still difficult to be popularized in China and the surgical procedures are complicated. Therefore, how to maximize the use of common equipment such as normal capsular tension rings and design a safe and reliable surgical method for the treatment of congenital ectopia lentis is an urgent issue for ophthalmologists. This article aims to introduce a two-stage capsular tension ring fixation for the treatment of congenital ectopia lentis, which has many advantages such as simple and safe operation, stable postoperative effect and less requirements for special equipment, and is worth promoting in clinical practice.

目的：通过对改良“Z”形无线结经巩膜缝线固定人工晶状体手术和传统有线结巩膜缝线固定人工晶状体手术治疗先天性晶状体脱位的比较来评价改良术式的临床疗效。方法：回顾性病例研究。纳入2018年1月—2021年3月期间于中山大学中山眼科中心行手术治疗的先天性晶状体脱位患者73例73眼，按手术方式不同将患者分为无线结组36例36眼和有线结组37例37眼。比较两组患者术前和术后1年的球镜度(DS)、柱镜度(DC)、等效球镜(SE)、最佳矫正视力(BCVA)、眼压(IOP)、眼轴长度(AL)、角膜内皮细胞计数和术后并发症的发生率。结果：两组患者术前各项观察指标组间比较差异无统计学意义(均P>0.05)。两组患者术后1年 BCVA 均较术前提高(均P<0.05)，SE均较术前降低(均P<0.05)。两组患者术后1年 BCVA 、DS、DC、SE、IOP、AL、角膜内皮细胞丢失率组间比较差异均无统计学意义(均P>0.05)。术后1年，有线结组有5例(13.5%)出现缝线暴露，无线结组未出现缝线暴露，组间比较差异有统计学意义(P<0.05)。结论：改良无线结 IOL 巩膜缝线固定手术可改善CEL患者的最佳矫正视力和屈光不正，有效减少缝线暴露及相关并发症。

Objective: To evaluate the clinical efficacy of modified “Z”-shaped knotless transscleral suture fixation intraocular lens (IOL) and traditional knotted transscleral suture fixation IOL in congenital ectopia lentis. Methods: A retrospective case study. A total of 73 eyes of 73 patients with congenital ectopia lentis who underwent surgical treatment in our hospital from January 2018 to March 2021 were included. According to different surgical methods, the patients were divided into the knotless group (36 eyes) and knotted group (37 eyes). Preoperative and postoperative of 1-year diopter sphere (DS), diopter cylinder (DC), spherical equivalent (SE), best corrected visual acuity (BCVA), intraocular pressure (IOP), and axial length (AL), corneal endothelial cell counts and the occurrence of postoperative complications rate were analyzed among two groups. Results: There was no significant difference in preoperative outcome measures between the two groups (P>0.05). BCVA at 1-year postoperative was significantly better (P<0.05), and SE at 1-year postoperative was significantly lower (P<0.05). There was no significant difference in BCVA, DS, DC, SE, IOP, AL, and corneal endothelial cell loss rate between the two groups at 1-year after operation (P>0.05). One year after the operation, there were 5 cases of suture exposure (13.5%) in the knotted group and no suture exposure in the knotless group, and the difference was statistically significant (P<0.05). Conclusions: The modified knotless IOL transscleral suture fixation can improve the best corrected visual acuity and alleviate ametropia of CEL patients, and reduce suture exposure and related complications effectively.

该文报道一例30岁的男性患者因“双眼自幼视力不佳，强光下视物模糊加重4年余”就诊，经过眼部检查评估，诊断为双眼瞳孔残膜、双眼屈光不正。患者接受一期双眼瞳孔残膜切除、二期双眼行有晶状体眼后房型环曲面人工晶状体(toric implantable collamer lens,TICL)植入手术，术后视力恢复良好。文章回顾了该例患者的诊治过程，为临床屈光不正同时伴有瞳孔残膜患者的诊治提供参考。

A 30-year-old male patient presented at our institution with a history of poor vision in both eyes since childhood, exacerbated by blurriness under bright light for over four years. Following a comprehensive ophthalmic examination, the patient was diagnosed with bilateral pupillary membrane remnants and refractive errors. The patient underwent a two-stage surgical intervention, starting with the removal of the pupillary membrane remnants, followed by the implantation of toric implantable collamer lenses (TICL) in the posterior chamber of the lensless eyes. Postoperative outcomes were favorable, with significant improvement in visual acuity. This article reviews the therapeutic journey of the patient, offering insights into the diagnosis and management of individuals with concurrent refractive anomalies and pupillary membrane remnants, thereby contributing to the clinical discourse on the subject.

目的：利用儿童Glasgow受益调查问卷(Glasgow Children’s Benefit Inventory，GCBI)评估先天性上睑下垂手术后儿童健康相关生活质量(health-related quality of life，HRQOL)的受益，并探索受益的影响因素。方法：验证GCBI中文版评估先天性上睑下垂矫正术后长期患儿生活质量的信效度，并用其评估术后儿童HRQOL受益与性别、手术年龄、术后随访时间等相关性。结果：共178名先天性上睑下垂接受下垂矫正术后儿童完成问卷，结果显示不同性别、年龄均有正性受益，术后短期(<1年)受益更明显(P<0.05)。结论：GCBI中文版具有良好的信度和效度，适用于评估先天性上睑下垂儿童术后受益结果。先天性上睑下垂儿童接受下垂矫正术后不同性别均有正性受益，术后短期(<1年)受益更明显。

Objective: To explore the benefits of children’s HRQOL after congenital ptosis surgery using the Glasgow Children’s Benefit Inventory (GCBI) and the factors influencing the benefits. Methods: To verify the reliability and validity of the Chinese version of GCBI in evaluating the long-term life quality of children after correction of congenital ptosis, and to evaluate the correlation between the HRQOL benefit of children after surgery and gender, surgical age, postoperative follow-up time, etc. Results: A total of 178 children after congenital ptosis surgery completed the questionnaire. Different genders or ages of children with congenital ptosis both showed positive benefit after ptosis correction, and the short-term (<1 year) benefit was more obvious after surgery (P<0.05). Conclusion: The Chinese version of GCBI has good reliability and validity,and is suitable for evaluating the postoperative benefit results of children with congenital ptosis. All the children with congenital ptosis received positive benefit after ptosis correction, and the short-term benefit (<1 year) was more obvious.

上睑下垂是眼整形科最常见的疾病之一，由于儿童处于生长发育阶段的特殊性，不规范的诊疗不仅会影响外观，同时可能会导致视功能发育迟缓。目前关于儿童先天性上睑下垂的手术时机和手术方式的选择尚无统一的标准和共识，本文通过对儿童先天性上睑下垂分类细化，阐明合并不同原因导致弱视的患者手术时机的选择；对比不同手术方式，分析其原理及优缺点，进一步明确矫正重度儿童先天性上睑下垂的手术方式，以期规范儿童先天性上睑下垂手术时机和手术方式的选择，提高手术的成功率，减少弱视的发生。

Blepharoptosis is one of the most common diseases in oculoplastics. Due to the particularity of children in the stage of growth and development, non-standard diagnosis and treatment not only affect the appearance,but also lead to the retardation of visual function development. Currently, there are no uniform standards and consensus on the choice of surgical timing and methods for pediatric congenital blepharoptosis. Here through refined classification of congenital blepharoptosis, we try to elucidate the choice of surgical timing for congenital blepharoptosis patients combined with amblyopia caused by a variety of reasons. By comparing different surgical methods, we analyze their mechanisms and advantages, and illustrate the surgical indication for severe congenital blepharoptosis, so as to standardize the choice of surgical timing and methods for pediatric congenital ptosis,improve the surgical success rate, and reduce the occurrence of amblyopia.

目的：探索智能语音随访系统在医疗场景中的新型应用服务模式并分析其在新冠肺炎疫情期间的应用效果，以此评估该系统应用于互联网医院开展医疗咨询服务的实际效能。方法：本研究应用智能语音随访系统针对先天性白内障患儿术后的常见问题进行回访。首先，针对随访目的，设计出完善的结构化随访内容与步骤。其次，部署智能外呼系统自动拨打用户电话，并通过语音识别技术对用户的每次应答进行识别，根据用户的应答自动跳转到下一个随访步骤，在完成一系列问答后根据用户的回答给出恰当的建议，实现电话随访的自动化与智能化。收集2020年2月24日至2月28日期间，智能语音随访系统随访的电话内容、呼叫时间、患儿资料等数据，采用描述性统计分析。结果：2020年2月24日至2月28日期间，中山大学中山眼科中心应用智能语音随访系统电话共随访1154例，其中收到有效回访数据561例，平均有效回访率48.6%。有效回访人群中，有204位(36.4%)家属认为疫情期间复诊时间延长，对宝宝眼睛的恢复有影响，309位(55.1%)家属认为对宝宝眼睛的恢复没有影响。360位(64.2%)先天性白内障患儿眼睛恢复情况良好，没有出现不良反应，169位(30.1%)患儿出现不良反应和体征，包括瞳孔区有白点，眼睛发红和有眼屎流眼泪等。统计患儿不同行为显示，有417位(74.3%)患儿佩戴眼镜，135位(24.1%)患儿没有佩戴眼镜，另有9位(1.6%)患儿佩戴眼镜情况不清楚，经常揉眼的患儿更容易出现眼睛发红(20.4%)、眼睛有眼屎或流眼泪(17.0%)和瞳孔区有白点(6.8%)等不良反应。结论：智能语音随访系统在临床随访中显示出巨大的应用潜力，可作为一种新型的智能医疗服务模式。

Objective: This study was designed to explore its potential value for new medical service model based on the intelligent voice follow-up system and analyze its application effect during the outbreak of COVID-19. The actual effectiveness of this intelligent voice follow-up system applied in the Internet hospital to carry out medical consultation service was discussed. Methods: In this study, an intelligent voice follow up system was developed for postoperative follow-up of children with congenital cataract. First, a well-designed and structured questionnaire contents were developed for postoperative follow-up. Secondly, the intelligent voice follow-up system was deployed. The system would automatically jump to the next follow-up step according to the user’s response, and give appropriate suggestions. Finally, the data of telephone recording, call time, children’s attributes were collected and statistically analyzed. Results: From February 24 to March 15, 2020, 561 families of children with congenital cataract from Zhongshan Ophthalmic Center were recruited by using the intelligent voice follow-up system. The system completed a total of 1 154 calls, of which 561 cases received follow-up data, reaching an average effective call rate of 48.6%. Among 561 cases, 204 (36.4%) thought that the extended time of follow-up visit would affect the recovery of children, while 309 (55.1%) thought that it exerted no effect on the recovery. 360 children (64.2%) achieved good ocular recovery without complications, whereas 169 cases (30.1%) developed ocular symptoms. These include white spots in the pupil area, redness and eye secretions. Statistics of different behavior of children showed that there were 417 (74.3%) children wearing glasses, 135 (24.1%) children did not wear glasses, another 9 (1.6%) children wearing glasses were not clear, often rubbing the eyes of children were more likely to appear redness (20.4%), eye secretions (17.0%) and white spots in the pupil area (6.8%) and other adverse reactions. Conclusion: The intelligent voice follow-up system shows great application potential in clinical follow-up, which can be employed as a new service mode of intelligent medical treatment.

特发性先天性眼球震颤(idiopathic congenital nystagmus，ICN)是一种常见的眼科疾病，患者常有明显的特征性的眼部异常，多伴有学习、社交障碍，对其身心健康影响较大。ICN遗传倾向明显，多表现为X染色体连锁(显性或隐性)，目前研究发现以FRMD7基因突变致病较为显著。近10余年来，国内外学者们在遗传学方面针对ICN和FRMD7基因做了大量的研究工作，取得了令人瞩目的结果。本文就2006年以来研究者们在FRMD7基因所致X连锁ICN的突变类型及位点作一总结，归纳并探讨FRMD7突变可能的致病机制，旨在为学者们提供以往研究结果的查证和未来研究方向的参考。

Idiopathic congenital nystagmus (ICN) is a common ophthalmic disease in which patients often have obvious and characteristic eye abnormalities. ICN patients are often accompanied by learning and social disorders, have a great impact on their physical and mental health. ICN which has an obvious genetic tendency and is mostly manifested as X chromosome linkage (dominant or recessive). Current studies have found that the mutation of FRMD7 gene is the most significant pathogenic factor. In the past 10 years, researchers have done a lot of work on the genetics of ICN and FRMD7 gene, and achieved remarkable results. This review summarizes the typ mutations caused by FRMD7 gene since 2006, and also discusses the possible pathogenesis of FRMD7 mutations, aiming to provide references for scholars to verify previous research results and future research directions.

目的：分析临床应用镜下改良眼轮匝肌手术治疗儿童先天性下睑内翻的效果。方法：回顾性分析安康市中医医院2年期间，应用镜下改良眼轮匝肌手术治疗61例(112眼)儿童先天性下睑内翻，根据睑内翻程度决定缝线跨度、松紧度及去除肌皮瓣量，0/6缝线将睑缘下眼轮匝肌与下睑缩肌缝合，0/8缝线连续缝合皮肤伤口，并做好术后护理。结果：所有患儿随访6~24个月，刺激症状缓解，106眼治愈，2眼好转，4眼复发，总有效率达96.43%。结论：镜下改良眼轮匝肌手术治疗儿童先天性下睑内翻美观、易操作、复发率低。

Objective: To analyze the clinical efficacy of modified orbicularis oculi surgery under microscope in the treatment of congenital lower eyelid entropion of children. Methods: Sixty-one cases (112 eyes) of children with congenital lower eyelid entropion treated by modified orbicularis oculi surgery in Ankang Hospital of Traditional Chinese Medicine from Aug 2020 to Sep 2022 were retrospectively analyzed. The suture span, tightness and the amount of myocutaneous flap removed were determined according to the degree of entropion. The orbicularis oculi muscle of lower eyelid margin was suturedwith 0/6 suture, and the skin wound was sutured continuously with 0/8 suture, and postoperative nursing was done well. Results: All children were followed up for 6–24 months, and the irritation symptoms were relieved, 106 eyes were cured, 2 eyes turned well, and 4 eyes relapsed. The total effective ratereached 96.43%. Conclusion: The modified orbicularis oculi surgery for treatment of children with congenital lower eyelid entropion conforms to visual aesthetics. In addition, it is easy to operate and have a low recurrence rate.

目的： 探讨儿童双目视力筛查仪在先天性白内障中的应用。 方法： 本文为回顾性分析，选择 2017 年 7 月 至 2018 年 5 月在厦门市儿童医院眼科的 3 个月至 2 岁婴幼儿 336 例(672眼)，均由2个固定的医生分别采用小瞳孔红光反射及儿童双目视力筛查仪检查，同时详细记录病史资料。所有患儿均给予裂隙灯检查、复方托品卡胺散瞳后行眼底检查，对眼底无法窥入的者必要时给予眼部B超检查或眼部MRI检查，以排除眼底病变。结果：依据2009年出版的第5版《Harley’s小儿眼科学》的先天性白内障临床分类方法，本组病例中先天性白内障的类型依次为皮质(核)白内障2 7例(35眼)，其中胚胎核2例( 4眼)，板层2 3例(29眼)，核性2例( 2眼)；囊膜性白内障1 9例(28眼)，缝合性白内障2例( 4眼)，极性白内障3例( 6眼)，全白内障2例( 3眼)。儿童双目视力筛查仪筛查出先天性白内障的灵敏度为81.58%，特异度为79.87%，诊断符合率为80.06%；红光反射筛查的先天性白内障的灵敏度为51.32%，特异度为69.97%，诊断符合率为67.86%；两组结果比较均有统计学意义。儿童双目视力筛查仪对皮质(核)性白内障筛查率为97.14%，而红光反射的筛查率为37.14%，结果比较具有统计学意义。结论：儿童双目视力筛查仪在先天性白内障筛查中，具有低漏诊率和误诊率，较高的诊断一致性的优势，尤其对皮质(核)性白内障有高的检出率，将有利于在日常儿童眼保健的辅助筛查工作中，及早发现先天性白内障患儿。

Objective: To explore the application of children’s binocular vision screening instrument in screening congenital cataract. Methods: This was a retrospective analysis of 336 infants aged 3 months to 2 years (672 eyes) who were admitted to the Ophthalmology Department of Xiamen Children's Hospital from July 2017 to May 2018. They were examined by two fixed doctors with the pupil red light reflex and binocular vision screening instrument, and the medical history was recorded in details. All the children received slit lamp examination, compound tropicamide mydriasis and fundus examination. If necessary, the children who could not see fundus were given with B-ultrasonic examination or MRI examination. Results: According to the clinical classification of congenital cataracts published in the 5th edition of Harley’s pediatric ophthalmology in 2009, In this group, there were 27 cases (35 eyes) of congenital cataract, including 2 cases (4 eyes) of embryonic nucleus, 23 cases (29 eyes) of lamellar layer, 2 cases (2 eyes) of nuclear cataract, 19 cases (28 eyes) of capsular cataract, 2 cases (4 eyes) of suture cataract, 3 cases (6 eyes) of polar cataract and 2 cases (3 eyes) of total cataract. The sensitivity of children’s binocular vision screening instrument screening congenital cataract was 81.58%, the specificity was 79.87%, and the diagnostic coincidence rate was 80.06%; the sensitivity of red reflection screening congenital cataract was 51.32%, the specificity was 69.97%, and the diagnostic coincidence rate was 67.86%; the results of the two groups were statistically significant. The screening rate of children's binocular vision screening instrument for cortical (nuclear) cataract was 97.14%, while that of red reflex was 37.14%. The results were statistically significant. Conclusion: In the screening of congenital cataract, the binocular vision screening instrument for children has the advantages of low missed diagnosis rate, high misdiagnosis rate and high diagnostic consistency, especially for cortical (nuclear) cataract. It is helpful to find the congenital cataract early in the auxiliary screening of children’cares eye health