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Corneal endothelial characteristics and biometric parameters in microcornea
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作者 Xiang-Zheng Zhang Li Pei +8 位作者 Jia-Ning Shi Xi Lu Ran-Yi Ding Xiao-Wei Zhong Xin Wang Du-Lei Zou Wei-Yun Shi Can Zhao Ting Wang 《International Journal of Ophthalmology(English edition)》 2025年第10期1856-1863,共8页
AIM:To assess the corneal biometric parameters and endothelial cell characteristics in microcornea patients,and exploring their correlations.METHODS:This cross-sectional study included 28 patients of microcornea with ... AIM:To assess the corneal biometric parameters and endothelial cell characteristics in microcornea patients,and exploring their correlations.METHODS:This cross-sectional study included 28 patients of microcornea with uveal coloboma(MCUC),13 patients of microcornea without coloboma(MCNC),and 30 age-matched healthy individuals(the control group).Corneal biometric parameters such as axial length(AL),anterior chamber depth(ACD),and white-to-white corneal diameter(WTW)were measured using the IOL Master.The corneal endothelial cell density(ECD),percentage of hexagonal cells(6A),average cell area(AVE),maximum cell area(MAX),minimum cell area(MIN),cell area standard deviation(SD),and coefficient of variation(CV)were collected by specular microscopy.RESULTS:This study included MCUC and MCNC patients with age-and sex-matched controls.All patients exhibited significantly reduced WTW(MCUC:8.51±0.71 mm;MCNC:9.08±0.42 mm)and worse logMAR BCVA(MCUC 0.62±0.43;MCNC 0.46±0.28)compared to controls(both P<0.001).The ECD was 3106.32±336.80 cells/mm²in the MCUC group and 2906.92±323.53 cells/mm²in the MCNC group,both significantly higher than the control group(2647.43±203.06 cells/mm²,P<0.05).In contrast,the CV,AVE,SD,and ACD in the MCUC and MCNC groups were significantly lower compared to controls(P<0.01).In patients with microcornea,the WTW was negatively correlated with the ECD and 6A,but positively with the CV,MAX,AVE,and SD.The ACD was negatively linked to the ECD,but positively to the AVE.CONCLUSION:The corneal ECD and 6A are increased,while the CV is decreased in patients with microcornea,particularly in those accompanied by uveal coloboma.The ECD and morphology demonstrate close correlations with the WTW and ACD. 展开更多
关键词 microcornea corneal endothelial cell corneal biometric parameters uveal coloboma anterior chamber depth
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Comparison of outcomes after early congenital cataract surgery in eyes with/without microcornea
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作者 Fan Zhang Zhang-Liang Li +1 位作者 Jia-Ying Wu Yun-E Zhao 《International Journal of Ophthalmology(English edition)》 2025年第10期1875-1879,共5页
AIM:To find out intraoperative and postoperative outcomes of congenital cataract surgery in eyes with microcornea.METHODS:This retrospective consecutive case series study compared outcomes after congenital cataract su... AIM:To find out intraoperative and postoperative outcomes of congenital cataract surgery in eyes with microcornea.METHODS:This retrospective consecutive case series study compared outcomes after congenital cataract surgery in eyes with/without microcornea.Infants(<1 year old)who underwent lensectomy surgery left aphakic were included.Microphthalmos was defined as an eye that has a horizontal corneal diameter less than or equal to 9.0 mm.RESULTS:There were 40 infants(54 eyes)in the microcornea group and 58(87 eyes)in the control group.The two groups were age-and sex-matched.The microcornea group showed significantly smaller corneal diameter(P<0.001),steeper corneal keratometry(P=0.001),thinner lens thickness(P<0.001),and shorter axial length(AL,P<0.001).And microcornea increased the incidence of poor pupil dilation(P<0.01).The two groups showed no significant differences in postoperative intraocular pressure(IOP),best-corrected visual acuity,central corneal thickness(CCT),AL,and the incidence of strabismus and nystagmus at the last follow-up.CONCLUSION:Although microcornea have different features from normal ones,the one-year follow-up after surgery has shown that early surgical intervention for congenital cataracts in eyes with microcornea can result in favourable outcomes with an acceptable rate of postoperative complications.Regular follow-up and timely management of postoperative complications are crucial for successful outcomes. 展开更多
关键词 congenital cataract microcornea aphakic
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白内障合并小角膜手术治疗1例
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作者 苗傲 徐婕 +2 位作者 陈天慧 赵镇南 蒋永祥 《中国眼耳鼻喉科杂志》 2025年第S1期49-52,共4页
53岁男性,因“双眼自幼视力差,近5年视力进一步下降”就诊。最佳矫正视力为双眼眼前手动;眼压为双眼15 mmHg(1 mmHg=0.133 kPa);查体见双眼眼球震颤伴眼位固定内斜,双眼角膜透明,角膜横径小;双眼下方虹膜缺损,瞳孔形状不规则;双眼晶状... 53岁男性,因“双眼自幼视力差,近5年视力进一步下降”就诊。最佳矫正视力为双眼眼前手动;眼压为双眼15 mmHg(1 mmHg=0.133 kPa);查体见双眼眼球震颤伴眼位固定内斜,双眼角膜透明,角膜横径小;双眼下方虹膜缺损,瞳孔形状不规则;双眼晶状体核性混浊明显,眼后节窥不入。初步诊断:双眼并发性白内障,双眼先天性小角膜,双眼先天性葡萄膜缺损,双眼眼球震颤,双眼内斜视,双眼弱视。患者入院后行左眼白内障囊外摘除术,术后1个月术眼最佳矫正视力提升至0.03。讨论体会:对于角膜直径低于正常值的患者,需要完善眼生物测量以明确诊断;白内障囊外摘除术对硬核白内障合并小角膜的治疗安全、有效。 展开更多
关键词 白内障 先天性小角膜 白内障囊外摘除术
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Insight into small eyes: a practical description from phenotypes presentations to the management 被引量:3
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作者 Sana Niazi Sorcha NíDhubhghaill +2 位作者 Farideh Doroodgar Zisis Gatzioufas Mohammad Hossein Dehghan 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2024年第2期380-391,共12页
This narrative review aimed to have an algorithmic approach to microphthalmos by a systematic search.The definition can be related to a number of special phenotypes.In the more challenging cases of complex microphthal... This narrative review aimed to have an algorithmic approach to microphthalmos by a systematic search.The definition can be related to a number of special phenotypes.In the more challenging cases of complex microphthalmos,relative anterior microphthalmos,and nanophthalmos,the surgeon can approach these cases more safely if they have a deep understanding of the anatomical variations and ideal formulae for intraocular lens computation and knows how to avoid intra-and post-operative complications.In this article,we review the criteria by which we recognize and describe pre-,intra-,and post-operative considerations,as well as discuss the ideal intraocular lenses for microphthalmos,given the intricate varieties of small eye phenotypes. 展开更多
关键词 MICROPHTHALMOS NANOPHTHALMOS lenses INTRAOCULAR anterior chamber axial length eye cataract microcornea syndrome
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单纯性先天小角膜一家系的调查 被引量:3
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作者 毕瑞明 许松玲 马欣 《中国优生与遗传杂志》 2004年第6期105-105,111,共2页
目的 寻找由DNA损伤 (如突变 )引起的人类表型缺陷 ,为人类遗传资源的收集与保藏以及人类基因结构与功能的研究打下基础。方法 通过实地调查得到表型缺陷家系 ,然后进行系谱分析。结果 得到一单纯性先天小角膜家系 ,四代 2 2位成员中... 目的 寻找由DNA损伤 (如突变 )引起的人类表型缺陷 ,为人类遗传资源的收集与保藏以及人类基因结构与功能的研究打下基础。方法 通过实地调查得到表型缺陷家系 ,然后进行系谱分析。结果 得到一单纯性先天小角膜家系 ,四代 2 2位成员中有 7例患者。结论 单纯性先天小角膜是由DNA损伤引起的人类表型缺陷 ,该病症符合常染色体显性遗传 ,具有一定的外显率和表现度 ,且表现度与性别有关。 展开更多
关键词 家系 单纯性先天小角膜 常染色体显性遗传
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Penetrating keratoplasty in children under 3 years old with congenital corneal opacities
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作者 Made Susiyanti Burhana Mawarasti Florence M.Manurung 《International Journal of Ophthalmology(English edition)》 SCIE CAS 2022年第1期45-51,共7页
AIM:To evaluate the graft rejection and visual outcomes after penetrating keratoplasty(PK)in the presence of various congenital corneal opacities in children.METHODS:In this retrospective cohort study,children who und... AIM:To evaluate the graft rejection and visual outcomes after penetrating keratoplasty(PK)in the presence of various congenital corneal opacities in children.METHODS:In this retrospective cohort study,children who underwent PK were then followed for 5 y.The patient’s medical records were collected from June 2014 until June 2019 and analyzed in December 2019.All patients were children under three years old with congenital corneal opacities with or without microcornea who came to a pediatric ophthalmologist and underwent PK in Jakarta Eye Center(JEC).Beforehand,all children have participated in a thorough evaluation for PK.In the case of severe microcornea was not advised to undergo surgery.The visual outcomes and graft survival rate were described in percentages.The graft survival plot was presented with Kaplan-Meier,while the visual acuity was analyzed using the Wilcoxon signed ranks test.RESULTS:Sixteen eyes from eleven patients(seven girls and four boys)underwent PK.The graft survival rate of the first 6,12,and 18 mo later of keratoplasty was 100%,83.3%,and 66.7%,respectively.The overall mean survival time is 22 mo(standard error 2.419),and no significant difference between the patients underwent PK before and after 36 mo of their age(P=0.52).The graft failure was 50%,and postsurgery complications included cataract 43.7%,band keratopathy 12.5%,and scleromalasia 6.25%.Wilcoxon test analysis of visual acuity post keratoplasty was not statistically significant(P=0.34),while overall showed 44%improvements of visual outcome for 5 y of follow-up.With a good survival at one year up to 22 mo(83.3%),the visual acuity could be achieved(63%),and showed improvements(44%)during follow-up.CONCLUSION:The complications are frequent for pediatric PK.Thus,corneal surgery on infants requires careful case selection,adequate pre-operative evaluation,skilled surgery(optical correction),very close cooperation family–physician,intensive post-operation care,and amblyopia management in the future. 展开更多
关键词 pediatric penetrating keratoplasty congenital corneal opacities microcornea CHILDREN
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先天性白内障合并小角膜的眼部表现及治疗 被引量:3
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作者 赵一璇 孙靖 +2 位作者 肖显文 周玉 张红 《国际眼科杂志》 CAS 2014年第4期742-744,共3页
目的:探讨小角膜合并先天性白内障患者的眼部临床表现、手术治疗时机及疗效。 方法:回顾性分析2000-01/2012-12在天津医科大学眼科中心接受治疗的先天性白内障合并小角膜患者11例18眼的临床资料,术前检查包括角膜直径(<9.5 mm ... 目的:探讨小角膜合并先天性白内障患者的眼部临床表现、手术治疗时机及疗效。 方法:回顾性分析2000-01/2012-12在天津医科大学眼科中心接受治疗的先天性白内障合并小角膜患者11例18眼的临床资料,术前检查包括角膜直径(<9.5 mm )、眼部异常和全身异常,术后随访至少1a,评价术后视力和并发症等。 结果:患者11例中有先天性白内障家族史4例,18眼中合并虹膜缺损6眼,眼球震颤18眼,原始玻璃体动脉残留1眼。对11例18眼患者施行三种手术方案:(1)行囊外白内障摘除术( extra capsular extraction of cataract , ECCE )者5眼;(2)Ⅰ期行 ECCE ,Ⅱ期行人工晶状体植入术(posterior chamber intraocular lens ,PC-IOL)者4眼;(3)Ⅰ期行ECCE联合PC-IOL植入者9眼。术后患者视力均有明显改善,18眼术后最佳矫正视力0.145&#177;0.0958(0.01~0.20)。 ECCE术中并发症主要为后囊破裂1眼,术后并发症主要为后发性白内障10眼,继发性青光眼2眼。 结论:先天性白内障合并小角膜常伴发虹膜、脉络膜缺损,眼球震颤等合并症,人工晶状体的植入能明显提高患者视力,但术中应注重防范角膜损伤、后囊破裂等并发症,术后加强管理,特别是眼压的监测,以极早防治青光眼等并发症。 展开更多
关键词 小角膜 小眼球 先天性白内障 临床表现 治疗
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中国白内障-小角膜综合征一家系突变基因及表型分析
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作者 张达人 卢岚 +6 位作者 曾杰 李丹丽 王云 王希振 黄瓅 樊宁 刘旭阳 《中华实验眼科杂志》 CAS CSCD 北大核心 2022年第10期955-959,共5页
目的分析白内障-小角膜综合征(CCMC)一家系的临床特征和分子遗传学特点。方法采用家系调查研究方法,收集2019年7月于厦门大学附属厦门眼科中心确诊的汉族CCMC一家系。详细询问病史,对家系中部分成员进行眼部检查,包括视力、眼压、裂隙... 目的分析白内障-小角膜综合征(CCMC)一家系的临床特征和分子遗传学特点。方法采用家系调查研究方法,收集2019年7月于厦门大学附属厦门眼科中心确诊的汉族CCMC一家系。详细询问病史,对家系中部分成员进行眼部检查,包括视力、眼压、裂隙灯显微镜、彩色眼底照相、眼部B型超声、角膜直径、眼前节光相干断层扫描、超声生物显微镜、角膜内皮镜及角膜地形图检查等。采集部分患者及家系成员外周血,提取DNA。对先证者DNA进行靶向高通量测序,所用芯片包含188个与晶状体异常相关的已知致病基因,在家系其他成员中应用Sanger测序对可疑位点进行检测,验证突变是否与临床表型共分离,明确该家系致病基因。采用蛋白功能预测软件REVEL对发现的变异位点进行生物信息学分析,评估该变异的致病性;采用InterPro分析蛋白保守结构域;采用ProtParam工具分析突变蛋白物理化学性质;采用PolyPhen-2在线软件预测突变蛋白的有害性;通过NCBI网站对致病基因的突变位点进行同源性分析,比较其在不同物种中的保守性。结果该家系共4代39人,其中患者11例,符合常染色体显性遗传。临床表现为先天性白内障合并小角膜,其他眼部检查及全身系统性检查结果未见明显异常。基因检测结果显示先证者存在CRYAA基因杂合变异c.61C>T,导致第21位氨基酸由精氨酸变异为色氨酸(p.Arg21Trp),Sanger测序验证结果证实该杂合变异与疾病共分离。ProtParam分析显示该位点变异后蛋白产物阳离子基团总数减少,且亲水性及稳定性下降;PolyPhen-2在线软件预测为有害变异;同源性分析显示该变异位点对应的氨基酸序列在多物种中高度保守。结论CRYAA基因c.61C>T p.Arg21Trp可能是导致该家系发病的原因,为国内首次报道。 展开更多
关键词 白内障-小角膜综合征 家系 基因检测 突变 CRYAA基因
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手持式全视网膜镜下25G玻璃体手术治疗先天性小角膜合并脉络膜缺损视网膜脱离 被引量:2
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作者 梁军 张伟 杨艳 《中国中医眼科杂志》 2016年第4期254-256,共3页
目的观察手持式全视网膜镜下25G治疗先天性小角膜合并脉络膜缺损视网膜脱离的临床疗效。方法回顾性分析先天性小角膜合并脉络膜缺损视网膜脱离患者13例(13只眼),均行手持式全视网膜镜下睫状体平坦部角膜缘4.0mm三切口闭合式25G玻璃... 目的观察手持式全视网膜镜下25G治疗先天性小角膜合并脉络膜缺损视网膜脱离的临床疗效。方法回顾性分析先天性小角膜合并脉络膜缺损视网膜脱离患者13例(13只眼),均行手持式全视网膜镜下睫状体平坦部角膜缘4.0mm三切口闭合式25G玻璃体切割术。术前术后观察视力变化、视网膜复位及复发情况。结果所有病例随访6—24个月,平均12个月。13只眼中,1次手术视网膜复位成功12只眼,占92-3%。1只眼于术后6个月因颞上周边视网膜增殖牵引再次发生视网膜脱离。行再次玻切术后视网膜复位。患眼术后视力均不同程度提高,脉络膜缺损累及视盘的4例患者术后视力提高至数指。脉络膜缺损位于视盘下方者9例.其中2例患者术后矫正视力为0.1以上,余7例患者术后矫正视力均在0.02~0.1。结论先天性小角膜合并脉络膜缺损视网膜脱离行玻切视网膜复位手术由于受到小角膜小瞳孔的影响,手术视野小,难度大,利用手持式全视网膜镜全视野与25G切口及切割效率的优势可以较好地完成手术,手术安全性好,成功率高,有一定的临床应用价值。 展开更多
关键词 手持式全视网膜镜 25G 小角膜 脉络膜缺损视网膜脱离
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合并小眼球和(或)小角膜先天性白内障的人工晶状体植入术 被引量:2
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作者 云波 钱进 《中国实用眼科杂志》 CSCD 北大核心 2010年第1期22-24,共3页
目的探讨合并小眼球和(或)小角膜的先天性白内障的人工晶状体植入术的手术疗效和操作难点。方法对9例(18只眼)眼轴长度为18.1~20.5mm和(或)角膜直径为7.5~9.Omm的先天性白内障患者施行超声乳化白内障吸除联合人工晶状体,... 目的探讨合并小眼球和(或)小角膜的先天性白内障的人工晶状体植入术的手术疗效和操作难点。方法对9例(18只眼)眼轴长度为18.1~20.5mm和(或)角膜直径为7.5~9.Omm的先天性白内障患者施行超声乳化白内障吸除联合人工晶状体,术后随访时间(12~60)月。结果术后矫正视力均高于术前矫正视力,脱盲率为93.7%,脱残率16.7%。并发症包括角膜水肿13只眼(72.2%),瞳孔区渗出膜7只眼(38.9%),人工晶状体偏位3只眼(16.7%)。结论对于合并小眼球和(或)小角膜的先天性白内障施行人工晶状体植入术能够有效地改善患者的视功能,但必须注重手术操作技巧,防止并发症的发生。 展开更多
关键词 白内障 儿童 小角膜 小眼球
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