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.