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.
马方综合征 (Marfan syndrome, MFS) 是一种由原纤维蛋白-1(fibrillin-1,FBN-1)突变引起的全身性遗传性疾病，FBN-1基因突变与MFS相关表型的联系相关，目前已报道的MFS常见的眼部表现包括角膜扁平、长眼轴、晶状体异位以及视网膜病变等异常，这些眼部异常将对MFS患者的视力产生影响，如角膜异常可影响角膜高阶像差的异常，可能导致近视或散光等屈光状态异常，从而影响视觉质量，损害视力清晰度。此外，MFS的眼底血管病变，也可能导致MFS患者的视力丧失，研究发现，MFS视网膜血管及脉络膜血管的密度较正常人减少，并与最佳矫正视力相关，由于光感受器的代谢与营养供应与视网膜及脉络膜血管息息相关，血管异常可能与视力损失相关。由于MFS患者存在视力损害的风险，其早期诊断和治疗尤为重要，因此，了解MFS眼部病变的特点及其对视力的影响，对制定针对MFS眼病的治疗方案具有重要的意义。另外，由于MFS眼部异常与FBN1基因突变相关，其基因突变类型多样，致病机制复杂，总结MFS眼部特点对其发病机制的继续探索有一定的指导作用，因此，文章拟就MFS患者眼部生物学参数特点及其对视力的影响这一领域国内外的相关研究进展进行综述。
Marfan syndrome (MFS) is a systemic hereditary disease caused by fibrillin-1 (FBN-1) mutations. FBN-1 gene mutations are associated with MFS-related phenotypes. Common ocular manifestations of MFS reported so far include corneal flattening, long axial length, ectopia lentis, and retinal abnormalities. These ocular abnormalities will affect the vision of MFS patients. For example, corneal abnormalities can affect abnormalities in corneal higher-order aberrations, which may lead to abnormal refractive states such as myopia or astigmatism, thereby affecting visual quality and compromising visual acuity. In addition, retinal vascular abnormalities may also lead to vision loss in MFS patients. Studies have found that the density of retinal and choroidal blood vessels in MFS patients is lower than that in normal individuals and is associated with best corrected visual acuity. Given the close relationship between the metabolism and nutrient supply of photoreceptors and retinal and choroidal vasculature, vascular abnormalities may be linked to visual impairment. Since MFS patients are at risk of visual impairment, early diagnosis and treatment are particularly important. Therefore, understanding the characteristics of ocular manifestations in MFS and their impact on vision is crucial for devising effective treatment strategies for MFS-related ocular conditions. Additionally, as ocular abnormalities in MFS are linked to mutations in the FBN1 gene, which exhibit diverse mutation types and complex pathogenic mechanisms, summarizing the ocular features of MFS can provide valuable insights for further exploration into its pathogenesis. Therefore, this article aims to review the progress of domestic and international research on the ocular biological parameters of MFS patients and their impact on vision.
近年来，晶状体脱位逐渐引起眼科医生的重视，针对晶状体脱位手术的探索与改进一直在持续进行中。随着超声乳化技术的发明和眼内植入材料的改进，晶状体脱位患者人工晶状体(intraocular lens,IOL)的植入和固定成为眼科医生关注的焦点。IOL悬吊术作为治疗严重晶状体脱位以及无晶状体眼且囊袋支撑不足或无囊袋患者的IOL植入的经典术式，主要经历了传统的经巩膜后房型IOL缝线固定术、以Z字形缝合为代表的无线结后房型IOL缝线固定术和无缝线的IOL巩膜层间固定术三个阶段，理解并掌握各种IOL悬吊术的优势和局限性对提高眼科医生的诊疗水平、改善患者的预后具有重要意义。
In recent years, ectopia lentis has gradually attracted the attention of ophthalmologists, and the exploration and improvement of surgery for ectopia lentis has been in progress. With the invention of phacoemulsification technology and the improvement of intraocular implant materials, the implantation and fixation of intraocular lens (IOL) in patients with ectopia lentis has become the focus of ophthalmologists. Suspensory IOL implantation is a classic operation for IOL implantation in patients with severe lens dislocation or aphakia with insufficient capsular bag support. It has mainly gone through three stages: traditional trans-sclera suture-fixed posterior chamber IOL, kontless posterior chamber IOL fixation represented by Z suture, and sutureless intrascleral IOL fixation. Understanding and mastering the advantages and limitations of various IOL fixation methods is of great importance for improving the diagnosis and treatment level of ophthalmologists and as well as the prognosis of patients.
