综述

先天性晶状体脱位的治疗及预后研究进展

Recent advances in research onthe treatment and prognosis of congenital ectopia lentis

:424-430
 
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.
其他期刊
  • 眼科学报

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
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  • Eye Science

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
    浏览
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