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.

先天性晶状体脱位(congenital ectopia lentis, CEL)是一种罕见的遗传相关性疾病，其主要临床特征是晶状体悬韧带先天性发育异常，导致晶状体偏离正常解剖位置。随着病情的进展，CEL可引起高度屈光不正甚至弱视外，还可能导致继发性青光眼和视网膜脱离等严重的并发症。目前，手术仍是改善CEL患儿视觉质量及防治并发症的主要手段。常用的手术方式包括晶状体摘除术、前房型人工晶状体(intraocular lens, IOL)植入术、囊袋支撑装置联合IOL植入术及经巩膜IOL固定术等，这些手术方式各具特点，但目前最佳手术方式仍未有定论。既往大量文献表明，手术能够显著改善CEL患儿视力，但随着眼球的生长发育，CEL患儿术后屈光状态常出现近视漂移。此外，术后并发症如缝线暴露，IOL瞳孔夹持、IOL脱位、视网膜脱离等仍有可能发生，需要长期的严密随访。这些因素都使得CEL的治疗具有挑战性。为此，文章就CEL的手术方式、视力预后、术后屈光变化及术后并发症进行综述，旨在为该疾病的临床诊断及治疗提供更为全面和深入的理解。

Congenital ectopia lentis (CEL) is a rare genetic disorder characterized by the displacement of the lens from its normal anatomical position due to abnormalities in the lens zonular. As the progression of the disease, CEL can lead to high refractive error, even amblyopia, as well as other serious complications such as secondary glaucoma and retinal detachment. Currently, surgical intervention remains the primary method to improve the visual quality and prevent complications in children with CEL.Common surgical options include lens extraction, anterior chamber intraocular lens (IOL) implantation, IOL implantation combined with capsular tension devices, and transcleral fixation of IOL. Each surgical approach has its own characteristics, but there is currently no consensus on the best surgical method. Previous literature has shown that surgery can significantly improve vision in children with CEL; however, due to the growth of the eye, postoperative refractive status often experiences myopic shift. Additionally, complications such as suture exposure, IOL pupil capture, IOL dislocation, and retinal detachment may still occur, necessitating long-term close follow-up. These factors make the treatment of CEL challenging. This article reviews the surgical approaches, visual prognosis, postoperative refractive changes, and postoperative complications associated with CEL, aiming to provide a more comprehensive and in-depth understanding for the clinical diagnosis and treatment of this disease.

先天性小眼畸形是一种罕见的先天性眼球发育异常，表现为眼球体积缩小和结构紊乱，常伴有眼组织缺损，严重影响患者的视觉功能。该病可单独发生，仅影响眼部，也可作为全身综合征的表现之一，同时伴有全身多器官或系统的异常。目前已发现82个先天性小眼畸形致病基因，能解释近2/3患者的发病原因。MAB21L1和MAB21L2是高度同源的两个基因，两者的致病变异均可导致小眼畸形，但又各自存在独特的表型特征。本期封面文章报道了MAB21L2基因变异导致小眼畸形2例，并通过回顾文献绘制变异频谱，从变异热点、变异类型、表型特征和基因型-表型相互关系等方面，对MAB21L1和MAB21L2进行比较，为两者缺陷所致眼遗传病的鉴别和诊断提供依据。

先天性小眼畸形是一种罕见的先天性眼球发育异常，表现为眼球体积缩小和结构紊乱，常伴有眼组织缺损，严重影响患者的视觉功能。该病可单独发生，仅影响眼部，也可作为全身综合征的表现之一，同时伴有全身多器官或系统的异常。目前已发现82个先天性小眼畸形致病基因，能解释近2/3患者的发病原因。MAB21L1和MAB21L2是高度同源的两个基因，两者的致病变异均可导致小眼畸形，但又各自存在独特的表型特征。本期封面文章报道了MAB21L2基因变异导致小眼畸形2例，并通过回顾文献绘制变异频谱，从变异热点、变异类型、表型特征和基因型-表型相互关系等方面，对MAB21L1和MAB21L2进行比较，为两者缺陷所致眼遗传病的鉴别和诊断提供依据。

先天性白内障是严重影响婴幼儿视功能的疾病。随着白内障手术和人工晶状体植入手术技术的发展，先天性白内障患者术后多可获得高质量的视觉康复。然而，如何更好防治手术相关的不良事件和并发症、先天性白内障伴随的其他眼部发育不良疾病的治疗以及形觉剥夺性弱视的治疗，仍然是先天性白内障手术后需要重视的临床问题。

      封面展示的是双眼先天性白内障术后继发青光眼(左眼)与正常眼(右眼)的对比示意图。该并发症起病隐匿、难以预测，是先天性白内障术后二次致盲的首要原因。针对这一术后并发症，美国婴儿无晶状体眼治疗研究组 (infant aphakia treatment study, IATS)将儿童白内障术后青光眼相关不良事件(glaucoma-related adverse events,GRAEs，包括了青光眼和可疑青光眼)定义为：1)青光眼：眼压>21 mmHg(1 mmHg=0.133 kPa)，且有以下一种或以上的解剖学改变：(a)角膜直径增加；(b)双眼不对称进行性近视漂移伴角膜直径和(或)眼轴的增加；(c)视杯直径进行性增大，杯盘比增加≥0.2；(d)必须进行手术才能控制眼压。2)可疑青光眼：停用局部糖皮质激素(激素)后连续2次眼压>21 mmHg，或可通过抗青光眼药物控制眼压，但无上述任何青光眼的解剖改变。所以，如何更精准地预防该术后并发症，防止对患儿视功能造成进一步的损害，是目前关键的临床问题。

       因此，文章对先天性白内障摘除及人工晶状体植入术后继发性青光眼和可疑青光眼的发生、相关危险因素、治疗和预防的手段进行总结，以期进一步提高对先天性白内障术后高眼压和青光眼防治的认识，减少术后并发症对患儿视功能造成的进一步损害。

先天性白内障是严重影响婴幼儿视功能的疾病。随着白内障手术和人工晶状体植入手术技术的发展，先天性白内障患者术后多可获得高质量的视觉康复。然而，如何更好防治手术相关的不良事件和并发症、先天性白内障伴随的其他眼部发育不良疾病的治疗以及形觉剥夺性弱视的治疗，仍然是先天性白内障手术后需要重视的临床问题。

      封面展示的是双眼先天性白内障术后继发青光眼(左眼)与正常眼(右眼)的对比示意图。该并发症起病隐匿、难以预测，是先天性白内障术后二次致盲的首要原因。针对这一术后并发症，美国婴儿无晶状体眼治疗研究组 (infant aphakia treatment study, IATS)将儿童白内障术后青光眼相关不良事件(glaucoma-related adverse events,GRAEs，包括了青光眼和可疑青光眼)定义为：1)青光眼：眼压>21 mmHg(1 mmHg=0.133 kPa)，且有以下一种或以上的解剖学改变：(a)角膜直径增加；(b)双眼不对称进行性近视漂移伴角膜直径和(或)眼轴的增加；(c)视杯直径进行性增大，杯盘比增加≥0.2；(d)必须进行手术才能控制眼压。2)可疑青光眼：停用局部糖皮质激素(激素)后连续2次眼压>21 mmHg，或可通过抗青光眼药物控制眼压，但无上述任何青光眼的解剖改变。所以，如何更精准地预防该术后并发症，防止对患儿视功能造成进一步的损害，是目前关键的临床问题。

       因此，文章对先天性白内障摘除及人工晶状体植入术后继发性青光眼和可疑青光眼的发生、相关危险因素、治疗和预防的手段进行总结，以期进一步提高对先天性白内障术后高眼压和青光眼防治的认识，减少术后并发症对患儿视功能造成的进一步损害。

先天性白内障是严重影响婴幼儿视功能的疾病。随着白内障手术和人工晶体植入手术技术的发展，先天性白内障患者术后多可获得高质量的视觉康复。然而，如何更好防治手术相关的不良事件和并发症、先天性白内障伴随的其他眼部发育不良疾病的治疗以及形觉剥夺性弱视的治疗，仍然是先天性白内障手术后需要重视的临床问题。文章对先天性白内障摘除及人工晶体植入术后高眼压和继发性青光眼的发生、相关危险因素、治疗和预防的手段进行总结，以期进一步提高对先天性白内障术后高眼压和青光眼防治的认识，减少术后并发症对视功能造成的进一步损害。

Congenital cataract is a significant condition that profoundly impacts the visual function of infants and young children. Advancements in cataract surgery and intraocular lens implantation have enabled the achievement of high-quality visual rehabilitation after congenital cataract surgery. Nevertheless, effective prevention and treatment of surgery-related adverse events and complications, as well as managing other ocular dysplasia and form deprivation amblyopia that may arise in conjunction with the surgery, continue to pose important clinical challenges following congenital cataract surgery. This article provides a comprehensive overview of the occurrence, risk factors, treatment and prevention of high intraocular pressure and secondary glaucoma after congenital cataract and intraocular lens implantation. Its aim is to enhance the comprehension of preventive and therapeutic measures for high intraocular pressure and glaucoma after congenital cataract surgery, thereby minimizing potential postoperative complications and preserving visual function.

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：探讨先天性瞳孔残膜围手术期的护理方法。方法：对13例(15眼)先天性瞳孔残膜患儿围手术期给予心理护理、安全护理、优先安排术前检查、术后严密观察病情变化、延续性护理并观察其效果。结果：13例(15眼)先天性瞳孔残膜的患儿手术均顺利完成，术后均无出现并发症，其中6例患者术后视力较术前视力有所提高，住院满意度为92.3%(12/13)。结论：对先天性瞳孔残膜患儿围手术期给予针对性的心理护理、安全护理、延续性护理是瞳孔残膜围手术期的有效护理方法。

Objective: To explore the perioperative nursing methods of persistent pupillary membrane. Methods: In the perioperative period, 13 cases (15 eyes) with persistent pupillary membrane were given preoperative nursing care, psychological nursing, careful observation of the change of condition, safety nursing, continuous nursing and observation of its effect. Results: A total of 13 cases (15 eyes) with persistent pupillary membrane were successfully operated, among which 6 cases had improved postoperative visual acuity compared with preoperative visual acuity, and no postoperative complications occurred. The satisfaction rate of hospitalization was 92.3% (12/13). Conclusion: It is an effective nursing method to give psychological nursing, safety nursing and continuous nursing to children with persistent pupillary membrane in perioperative period.

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.