Alport's syndrome and the eye

Alport's syndrome comprises hereditary deafness, nephritis and ocular abnormalities. The features of Alport's syndrome are illustrated by a family with Alport's syndrome and hereditary oesophageal leiomyomatosis. The evidence that Alport's syndrome is due to a widespread basement membrane disorder is noted. Treatment of anterior lenticonus, the principal ocular abnormality, by lensectomy and intraocular lens insertion is recommended.     

Weill-Marchesani综合征继发青光眼手术治疗的新观察

目的探讨Weill-Marchesani综合征继发青光眼的手术治疗方法。设计回顾性小样本病例系列。研究对象2009年6月至2010年1月在北京同仁医院住院手术治疗的年龄13~32岁的Weill-Marchesani综合征继发青光眼晚期患者3例。方法 2例为晶状体半脱位继发青光眼,采取晶状体及前部玻璃体切除术联合Ahmed阀植入术或直视下眼内睫状体光凝术。1例为人工晶状体(IOL)眼继发闭角型青光眼,采取Ahmed阀植入术联合前部玻璃体切除术。主要指标眼压。结果术后随访4~6个月。采取Ahmed阀植入术联合手术的2例患者不用药物眼压控制在21 mm Hg以下;采取眼内睫状体光凝术的1例患者局部用2种降眼压药物眼压稳定控制在21 mm Hg以下。1例术后早期发生严重脉络膜脱离,1例术后早期出现恶性青光眼,经保守治疗恢复。结论本文有限的病例结果提示,Weill-Marchesani综合征继发青光眼采用晶状体及前部玻璃体切除术联合青光眼引流阀植入术或眼内睫状体光凝术可有效地控制眼压。     

眼前节毒性综合征

眼前节毒性综合征(toxic anterior segment syndrome,TASS)是内眼术后眼前节的无菌性炎性反应,已成为一个重要的白内障手术并发症。手术中进入前房的各种非感染性毒剂均可能导致TASS的发生,主要表现为角膜弥漫性水肿、睫状充血、前房纤维素性渗出等,大多数患者糖皮质激素治疗有效,少数可遗留永久性眼内组织损伤,以至于影响视力,故早期诊断和治疗显得至关重要。我们现就TASS作一综述,希望能引起眼科医师的关注。     

术后早期晶状体囊袋阻滞综合征分析

目的探讨白内障超声乳化联合人工晶状体植入术后早期晶状体囊袋阻滞综合征（capsular block syndrome，CBS）的易发因素、处理方法及效果。方法分析我院2006年1月至2007年1月所发生的9例白内障术后早期CBS病例的临床特点，并随访了不同处理方法的效果。结果9例患者均发生在术后早期（术后1天-1周）。其中8例裸眼视力低于0.4，且具有明显的近视漂移[平均等效球镜（-2.50＋0.71）D]，另1例裸眼视力0.9（1.0＊＋0.25／-0.75×95）。6例患者使用了四襻式亲水性丙烯酸酯人工晶状体，2例患者使用了一片式亲水性丙烯酸酯人工晶状体，1例患者植入了三片式丙烯酸酯人工晶状体。前囊撕囊直径5-5.5mm，人工晶状体与后囊之间留有明显的间隙。对其中6例患者施行了前房灌洗术，术中吸除囊袋内部分黏弹剂样物质，术后第1天裸眼视力均恢复至0.7以上，平均等效球镜减少-1.60 D；1例施行了Nd：YAG激光前囊周边部打孔术，裸眼视力恢复至0.8；1例患者因瞳孔不能充分散大，后囊已有轻微混浊，我们施行了Nd：YAG激光后囊切开，裸眼视力恢复至0.6；另1例因裸眼视力0.9，没有明显近视，未作处理，2周后囊袋内液体吸收，后囊贴附；随访5个月8例患者后囊均未发生混浊（8例后囊未切开，其中1例已行后囊切开）。结论前囊撕囊直径过小，术中黏弹剂残留是术后早期CBS的两个易发因素，术后早期前房灌洗以及择期Nd：YAG激光前囊或后囊切开均可以有效治疗术后早期CBS。     

Fuchs综合征并发白内障的人工晶状体植入

目的　评价 Fuchs综合征并发白内障的手术方法。方法　对 11例 Fuchs综合征并发白内障患者行白内障囊外摘出 (ECCE)联合人工晶状体 (IOL)植入术。结果　 11例患者白内障囊外摘出联合人工晶状体植入术后无严重手术并发症 ,不同程度葡萄膜炎易被控制 ,无眼压增高 ,术后平均随访 18m o,矫正视力 0 .4～ 1.2 ,0 .5以上占 81.8% .结论　Fuchs综合征并发白内障的人工晶状体植入手术安全、疗效好     

两种软性材质可折叠IOL在剥脱综合征性白内障患者中临床应用

目的:研讨剥脱综合征性白内障植入Acrysof Natural和硅胶人工晶状体的效果。方法:选择无青光眼发作史的剥脱综合征性白内障65例70眼行超声乳化吸出白内障,无术中并发症60例63眼。其中30例33眼用植入器植入Acrysof Natural人工晶状体;另30例30眼用植入器植入硅胶(silicone)人工晶状体。统计植入术中并发症;术后1d,1wk,3mo,1a的视力、眼压、前房情况、人工晶状体稳定性。结果:Acrysof Natural在植入过程中展开慢,可控性好,在剥脱综合征患者瞳孔不易散大情况下仍能顺利植入,无并发症。对照组硅胶人工晶状体展开时弹射快,有7眼植入过程中发生翻转,在部分悬韧带不健全的病例同时因瞳孔缩小不易观察和控制,造成5眼植入时袢划破后囊膜(16.6%)。1枚弹入玻璃体,取出后改睫状沟植入。术后视力对后囊破裂者不列入正常统计。结论:选择Acrysof Natural人工晶状体单体、袢宽软、植入过程中展开慢、好控制、可避免植入过程并发症。其光学部分相对薄、稳定性好、前房相对较深。组织相容性好,术后反应轻。     

Alport综合征临床分析

目的总结Alport综合征的临床表现，尤其是眼部特征。方法回顾性分析32例被确诊为Alport综合征患者的内科、耳鼻喉科和眼科检查结果。结果所有患者均有不同程度的。肾脏病变：18例有。肾衰，4例肾功能不全，10例血尿。20例患者有感音神经性耳聋。13例患者有眼部异常表现，其中5例为典型性改变：前圆锥晶体3例，黄斑周围斑点2例。结论眼部异常不是Alport综合征诊断的必需条件，但因其典型的眼科表现应当引起眼科医师的注意。     

Alport综合征眼部临床表现分析

目的:总结Alport综合征的临床表现,尤其是眼部特征。方法:回顾性分析32例被确诊为Alport综合征患者的内科、耳鼻喉科和眼科检查结果。结果:患者30例(93.7%)有疾病家族史。所有患者均有不同程度的肾脏病变:18例(56.3%)有肾功能衰竭,4例(12.5%)肾功能不全,10例(31.3%)血尿。患者20例(62.5%)有感音神经性耳聋。患者13例(40.6%)有眼部异常表现,其中5例(15.6%)为典型性改变:前圆锥晶体3例,黄斑周围斑点2例。结论:眼部异常不是Alport综合征诊断的必需条件,但因其典型的眼科表现应当引起眼科医师的注意,以便早期诊断治疗。     

Ocular manifestations of Alport syndrome

AIM： To analyze the clinical manifestation of Alport syndrome, especially the ocular features. METHODS: The physical, ophthalmologic and audiologic examination results of thirty-two patients with Alport syndrome were analyzed retrospectively. RESULTS: Thirty (93.7%) patients had some family history. All patients had renal disease: eighteen (56.3%) patients with chronic renal failure, four (12.5%) patients with renal insufficiency, and the other ten (31.3%) patients with hematuria. Twenty (62.5%) patients had sensorineural deafness. Thirteen (40.6%) patients had ocular deformity, five (15.7%) patients had typical ocular changes: three patients with anterior lenticonus, and two patients with macular flecks. CONCLUSION: Ocular anomalies are not requisite for the diagnosis of Alport syndrome. But its typical ocular features should be recognized by the ophthalmologists which supports the diagnosis.     

Vitrectomy with air tamponade for surgical repair of rhegmatogenous retinal detachment by eye position guided fluid-air exchange

AIM: To observe the efficacy and safety of pars plana vitrectomy (PPV) with eye position guided fluid-air exchange (FAX) and air tamponade in the treatment of rhegmatogenous retinal detachment (RRD). METHODS: RRD patients without severe proliferative vitreoretinopathy (PVR) C1 or more were enrolled. All patients underwent PPV combining with air tamponade. During operation, the primary retinal break(s) were placed at lower site and subretinal fluid was aspirated through the break(s) at the same time when eye position guided FAX was proceeding. Sufficient laser spots were made to seal the retinal break(s) after FAX, and filtered air was left in vitreous cavity as tamponade agent finally. The main outcomes were primary and final success rates, best corrected visual acuity (BCVA), and the secondary outcomes were rate of postoperative cataract surgery and high intraocular pressure. RESULTS: A total of 37 eyes (20 males and 17 females) with a follow-up time of ≥6mo were included. The range of RRD was 5.6±1.8h, and the number of retinal breaks was 1.9±1.2. The breaks located at inferior quadrants (between 3:00 and 9:00) in 5 cases (13.5%), and both superior and inferior breaks were found in 3 cases (8.1%). A total of 25 cases (67.6%) with macular detached involvement, 9 cases (24.3%) with intraocular lens, and 8 patients (21.6%) were treated with phacoemulsification and intraocular lens implantation together. The success rate of primary retinal reattachment was 100% (37/37). At 6mo postoperatively, BCVA (logMAR) was increased from 1.13±1.07 to 0.23±0.15 (P<0.001). Phacoemulsification combined with intraocular lens implantation was performed in 2 patients (5.4%), and one of them underwent macular epiretinal membrane peeling in addition (2.7%). Furthermore, high intraocular pressure was found in 4 cases (10.8%). CONCLUSION: PPV with air tamponade by eye position guided FAX can achieve a high reattachment success rate in the management of patients with RRD, and it has the advantages of short postoperative prone time and fewer operative complications.     

Alport综合征的临床表现

目的:分析Alport综合征患者的临床表现和眼部病变的特征。方法:对近21a来我院确诊的31例Alport综合征患者的资料进行回顾性分析,记录其一般情况、家族史、眼部、肾功能及耳科检查结果等。结果:患者中男21例(68%),女10例(32%)。确诊年龄19.8±9.7岁。患者中有17例满足3项以上诊断标准(55%),另外14例均进行了肾穿刺活检电镜检查支持诊断。12例有眼部异常(39%),4例同时有前锥形晶状体和黄斑周围视网膜斑点2项;1例仅有晶状体异常;7例仅有视网膜斑点。28例进行肾穿(90%),电镜检查符合诊断。20例有家族史(64%)。21例有听力障碍(68%)。结论:Alport综合征患者中眼部异常的表现有独特性;了解眼部病变特征并结合全身病史有助于疾病的诊断和随诊。     

非正常晶状体眼行人工晶状体悬吊术的疗效观察

观察由各种原因导致的非正常晶状体眼因无足够后囊膜支撑而施行人工晶状体悬吊术的疗效。 方法:使用美国ALCON公司CZ70BD悬吊式人工晶状体对8例9眼非正常晶状体白内障患者（过熟期白内障3眼,外伤性白内障伴晶状体半脱位4眼,马凡氏综合征2眼）因术中无后囊膜支撑施行人工晶状体悬吊术;对8例8眼Ⅰ期白内障术后非正常晶状体眼患者（伴有瞳孔异常的无晶状体眼7眼,人工晶状体严重偏位1眼）施行Ⅱ期人工晶状体悬吊术,观察其术中、术后并发症及术后视力情况。随访1~6mo。 结果:术中或术后5眼（29%）出现前房或玻璃体少量出血,10d左右基本吸收;术后1眼（6%）发生脉络膜脱离,1mo恢复;切口微漏致低眼压2眼（12%）,加压包扎5d恢复。术后1d,裸眼视力＞0.1者13眼（76%）;术后10d,裸眼视力＞0.1者17眼（100%）,术后10,30d,裸眼视力＞0.3者分别为11眼（65%）和12眼（71%）。 结论:人工晶状体悬吊术是治疗易发生无后囊膜支撑的非正常晶状体眼的一种较好的选择。     

Alport综合征6例临床分析

目的总结Alport综合征的临床特征,尤其是[部表现。方法对6例Alport综合征患者的[部、电听力及肾功能检查结果进行回顾性分析。结果有[部改变者4例,高频感音性神经性耳聋者2例,肾功能衰竭者3例,肾组织活检示Alport综合征改变者2例,阳性家族史5例。结论对于有前锥形晶体和/或视网膜黄斑周围微粒改变者应行肾活检以确诊Alport综合症。     

Comparison of clinical outcomes between iris-fixated anterior chamber intraocular lenses and scleral-fixated posterior chamber intraocular lenses in Marfan syndrome with lens subluxation

Background: To compare the clinical outcomes in Marfan's with subluxated lens having phaco‐emulsification with simultaneous scleral‐fixated posterior chamber intraocular lens or iris‐fixated anterior chamber intraocular lens implantation. Design: Randomized case series in the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat‐sen University, Guangzhou, China. Participants: Seventy‐one eyes of 49 patients with Marfan syndrome with subluxated lens. Methods: This is a randomized case series of patients with Marfan syndrome and subluxated lenses who underwent phaco‐emulsification combined with scleral‐fixated posterior chamber intraocular lens or iris‐fixated anterior chamber intraocular lens implantation. Main Outcome Measures: The evaluation indexes included the surgery time, best corrected visual acuity, intraocular pressure, aqueous flare and cells counts, corneal endothelium counts and complications. Results: Increase in best corrected visual acuity in both groups was not significant. The aqueous flare and cells rose in both groups postoperatively. Significant difference between the two groups at 1 week postoperatively was found, whereas no statistically significant difference was found later. The loss rate of corneal endothelium cells in the scleral‐fixated posterior chamber intraocular lens group was 13.2% and 19.5% at 3 months and 1 year postoperatively, which in the iris‐fixated anterior chamber intraocular lens group was 13.3% and 19.3% (P > 0.05). Prolapse of vitreous was found in 21 cases intraoperatively. The posterior capsule opacification rate was 32% and 15%, respectively. The decentration of the intraocular lens was found in 19 eyes (48.7%) in the scleral‐fixated posterior chamber intraocular lens group 1 year postoperatively, whereas none was found in the iris‐fixated anterior chamber intraocular lens group. Conclusions: Iris‐fixated anterior chamber intraocular lens after phaco‐emulsification presented a safe, simple and efficient approach for managing subluxated lens in Marfan syndrome.     

虹膜夹型与巩膜固定型人工晶状体在无晶状体眼患者中的疗效比较

目的：分析无晶状体患者虹膜夹型人工晶状体(ICIOL)与巩膜固定型人工晶状体(SFIOL)在视力、手术时间及术后并发症方面的疗效。

方法：前瞻性研究。共纳入2018-10/2020-02在我院门诊就诊的60例60眼无晶状体眼患者。将所有患者分为两组,每组各30例。组I患者行ICIOL植入术,组II患者行SFIOL植入术。筛除既往有眼部病变和视网膜手术史患者。术前、术后随访9mo。

结果：随访9mo后,ICIOL组中26例(87%)患者,SFIOL组中24例(80%)患者达到最佳矫正视力(BCVA,LogMAR)为0.50～0.00。两组BCVA 均值具有可比性。 ICIOL组手术时间较SFIOL组更短(P<0.01)。SFIOL组并发症明显增多,而ICIOL组的并发症较少。

结论：两组的视觉结果具有可比性。在无晶状体矫正中,ICIOL并发症少,手术时间短,是较SFIOL更好的手术方式。     

晶状体不全脱位的手术治疗

目的：评价不同手术方式治疗晶状体不全脱位的疗效。方法：对12例12眼晶状体不全脱位作晶状体摘除,其中超声乳化吸除6眼,囊外摘除2眼,囊内摘除2眼,植入人工晶状体12眼,结果：术后视力提高11眼,1眼视力不能提高的原因为视网膜脱离。术中和术后无严重并发症。结论：晶状体不全脱位的治疗有多种不同的方法可以选择,治疗目的是减少并发症和提高视力。     

Cataract surgery in the small adult eye

Microphthalmos is a rare condition that is often associated with several other ocular abnormalities. Given the considerable differences between microphthalmic and anatomically normal eyes, cataract surgery is technically demanding in these patients, and special attention must be given to adequate preoperative planning of these procedures. Furthermore, the unique nature of these surgeries creates a particular subset of intraoperative and postoperative complications. However, with the advent of piggyback intraocular lens placement, the visual outcomes of cataract surgery in small adult eyes have improved considerably over the past 20 years. This review discusses the nature of the microphthalmic eye, and addresses proper pre-, intra-, and postoperative care of the microphthalmic patient.     

Pigmentary dispersion syndrome subsequent IOL implantation in P.C.

Excessive iris trauma during cataract surgery with or without IOL implantation and intermittent contact between the lens implant and the iris in the post-operative period may give rise to a pigment dispersion syndrome that can, in some cases, lead to a pigmentary glaucoma. This communication examines possible causes of the syndrome and the precautions to be undertaken in order to reduce its incidence.     

超声乳化联合IOL植入治疗前圆锥晶状体伴有Alport综合征的视觉效果

目的:观察超声乳化联合IOL植入治疗7眼前圆锥晶状体伴有Alport综合征患者的手术效果。方法:Alport综合征患者4例,7眼因前圆锥晶状体行超声乳化联合IOL植入手术。结果:所有患者的术后视力均有明显提高。结论:我们认为对于前圆锥晶状体伴有Alport综合征患者采用超声乳化联合IOL植入手术治疗,是一种安全的疗法。