色素膜炎并发白内障的手术治疗体会

目的：探讨并总结色素膜炎并发白内障超声乳化手术过程中的各步技巧、小瞳孔的处理方式及手术的临床效果。方法收集2014年3~9月于西安市第四医院治疗的45例色素膜炎并发性白内障患者的临床资料,所有患者均为小瞳孔,并存在不同程度的瞳孔后粘连,患者均行超声乳化手术及人工晶体植入术。术中采取多种方式扩大瞳孔,达到手术需要。术后观察并分析患者视力恢复及眼部并发症等情况。结果所有患者都顺利完成手术,术后视力有不同程度的提高。术后瞳孔形态均为圆形或近圆形。术后发生角膜水肿2例,眼压升高11例,前房炎症渗出3例,经相应治疗均恢复正常。结论色素膜炎并发性白内障手术中,采取有效的措施扩大瞳孔,谨慎操作,可使手术更加安全有效。     

小瞳孔白内障超声乳化吸除及人工晶体植入术的临床研究

目的：探讨小瞳孔时的白内障超声乳化术的手术技巧和临床效果。方法：对23例(77眼)散瞳后小瞳孔的白内障患者采用钝性分离及非切开的牵拉法扩大瞳孔,进行连续环形撕囊和原位超声乳化并人工晶状体囊袋内植入术。结果：术后随访4～12个月,所有病例术后视力较术前有不同程度的提高,术后视力0．1～0．2者5眼．0．3～0．5者14眼;0．8以上者8眼。结论：采用钝性分离及非切开的牵拉扩大瞳孔法,可使小瞳孔的白内障完成手术并恢复良好的视力和生理性圆瞳孔或近圆瞳孔,手术并发症少。     

葡萄膜炎并发白内障45例手术治疗

目的探讨葡萄膜炎并发白内障术中小瞳孔手术处理方法及手术时机的选择。方法45例(45眼)葡萄膜炎并发白内障患者,在小切口下采用注水式截囊针剥离粘连及虹膜根切后,从根切口剥离或瞳孔缘切除或切开,形成瞳孔,完成白内障摘除人工晶体植入术。结果45例患者均顺利安全完成手术,术后反应较轻,术后视力≥0.4以上者占82.2%。结论葡萄膜炎并发白内障的手术治疗安全、疗效可靠。     

Variable phenotype of Pierson syndrome

Pierson syndrome is caused by mutations in the LAMB2 gene, which encodes the laminin β2 chain, and is clinically characterized by congenital nephrotic syndrome (CNS) and bilateral microcoria. Here, we describe two cases of Pierson syndrome involving atypical phenotypes. Patient 1 presented with congenital microcoria and infantile nephrotic syndrome. Despite persistent nephrotic syndrome, her renal function was maintained normally until she was 6 years old. Genetic analysis revealed two frame-shifting deletions (truncating mutations) in the LAMB2 gene. Patient 2 presented with isolated CNS without ocular involvement. Her renal function deteriorated progressively over several months, and retinal detachment in the right eye developed when she was aged 10 months. LAMB2 analysis revealed a missense mutation in one allele and a frame-shifting deletion in the other allele. Electron microscopy of a renal biopsy revealed irregular lamellation of the glomerular basement membrane (GBM) in both patients. The phenotypes of Pierson syndrome vary widely, and the severity of the renal phenotype is not always parallel to that of the ocular phenotype. The phenotypic variability likely reflects genotype–phenotype correlations, but unknown genetic or environmental modifiers may play an additional role. Ultrastructural changes of the GBM are a useful diagnostic indicator.     

A milder variant of Pierson syndrome

Congenital nephrotic syndrome (CNS) comprises a heterogeneous group of conditions having in common the disruption of normal glomerular permselectivity, and it carries a poor prognosis, with most patients progressing to end-stage renal disease. Recently, mutations in the LAMB2 gene encoding laminin β2 were described as the cause of Pierson syndrome, which is characterized by CNS and a complex ocular maldevelopment with microcoria as the most prominent clinical features. Most affected children exhibit early onset of chronic renal failure, neurodevelopmental deficits, and blindness. We report on a patient with CNS, high-grade myopia, and minor structural eye anomalies, including remnants of pupillary membranes, but no microcoria. The patient had not developed renal failure by the age of 16 months, and he showed no neurodevelopmental deficits. He was identified to be homozygous for a novel LAMB2 missense mutation. This observation, together with two recent reports on milder variants of Pierson syndrome, corroborates the concept that the clinical expression of Pierson syndrome is more variable than initially described, and that milder phenotypes may be related to hypomorphic LAMB2 alleles.     

前房内麻醉在小瞳孔白内障超声乳化术中的应用

目的探讨利多卡因前房内麻醉在小瞳孔白内障超声乳化术中的麻醉效果。方法对双眼小瞳孔白内障患者42例行超声乳化手术,1眼采用0.4%盐酸奥布卡因表面麻醉联合1%利多卡因前房内麻醉,另1眼采用单纯表面麻醉进行对照。并观察两组术中感觉和术中术后并发症发生情况。结果两组术中不适感觉发生率比较有明显差异(P<0.05),术中术后并发症的发生比较差异无统计学意义(P>0.05)。结论在小瞳孔白内障超声乳化术中应用前房内麻醉,可增加麻醉效果,利于手术操作,是简单、安全、有效的方法。     

青光眼小瞳孔下超声乳化吸出人工晶状体植入术

目的：观察青光眼合并白内障小瞳孔下行超声乳化吸出术的临床效果。方法：对32例32眼青光眼合并白内障应用非切开瞳孔扩张法，小瞳孔下行超声乳化吸出联合人工晶状体植入术治疗，结果：随访12月，32例32眼无严重并发症，瞳孔形状基本恢复，直径3－3.5mm者22眼，占68．72％，直径4－5mm者8眼，占25．00％；直径≥6mm2眼，占6．25％，视力眼前手动3眼，占9．37％；0．05－0．08 3眼，占9．37％；0．1－0．26眼，占9．37％；0．1－0．26眼，占18．75％，0．3－0．414眼占43．75％，≥0．56眼，占18．75％，结论：青光眼合并白内障无张力性小瞳孔下行白内障超声乳化吸出联合人工晶状体入术是安全可行的。     

并发性白内障超声乳化术

目的探讨超声乳化术中瞳孔不能散大或伴有虹膜后粘连的手术对策。方法术中先用粘弹剂或用器械分离;对伴有瞳孔闭锁/膜闭部位,可做剪切分离;对小瞳孔可做推拉扩张或行瞳孔切开等手法。然后完成超声乳化手术。结果①术后眼内反应、眼压均有明显波动,但经用药物后很快即被控制并趋向稳定;②术后矫正视力明显提高。结论对小瞳孔或瞳孔闭锁/膜闭等并发性白内障,术前需因人而宜精心设计制定个性化的手术方案,术后同样可取得良好的手术效果。     

小切口手法碎核治疗小瞳孔硬核白内障

目的 采用巩膜隧道小切口对小瞳孔硬核白内障患者进行白内障摘除并折叠人工晶状体植入术,观察患者术后的疗效.方法 住院小瞳孔硬核白内障患者57例69只眼,均施行4mm反眉状巩膜隧道切口白内障摘除并折叠人工晶状体植入术,观察术后的视力、散光程度以及并发症等情况.结果 57例(69只眼)中,术后1个月视力0.5以上者46只眼(66.67%),0.8以上者14只眼(20.29%),低于0.25者4只眼(5.8%);术前及术后1d、1周、1月测得角膜平均散光度分别为:(0.34±0.28)D、(0.41±0.32)D、(0.38±0.31)D、(0.36±0.30)D,术后各时间段角膜散光之间差异无统计学意义(P>0.05),与术前散光比较亦无统计学意义(P>0.05);术中后囊破裂5只眼(7.25%),瞳孔缘撕裂6只眼(8.700/0),因损伤小而没有进行处理;术后角膜内皮水肿9只眼(13.04%)、前葡萄膜炎9只眼(13.04%)、虹膜部分粘连4只眼(5.80%)、眼压增高2只眼(2.90%),经对症治疗均7d内消失.结论 在小瞳孔硬核白内障手术中,采用巩膜隧道小切口进行白内障摘除折叠式人工晶状体植入,能有效减少手术并发症,提高手术成功率及术后疗效、降低成本,手术操作步骤简化安全,易于在白内障复明活动中推广.

Abstract：

Objective To discuss the effect of new method of nucleus chopping for hard nucleus that over degree Ⅳ in manual small-incision microcoria cataract surgery. Methods All 57 cases(69 eyes)with the hard nucleus of degree Ⅳ-Ⅴ had undergone manual small-incision microcoria cataract surgery with applying intratunnel nucleus chopping method that designed by the author. The vision acuity, astigmatism and complication of postoperative complication had been discussed. Results The visual acuity was ≥ 0.5 in 46 eyes (66.67%), ≥ 0.8 in 14 eyes(20.29%)and <0.25 in 4 eyes(5.80%)one month after operation. After operation cornea astigmatism had none statistics difference(P>0.05)between that of Preoperative. The complications included cornea edema(13.04%), uveitis(13.04%), iris accretion(5.80%), after-cataract(5.80%)and increased tension(2.90%). The complications diminished after symptomatic treatment in one week. Conclusions Scleral-tunnel small incision for hard nucleus in manual small incision microcoria cataract surgery is safe, simple and the less complication. It is virtue of rapid recovery, good outcome and medical expenses.     

小瞳孔白内障的超声乳化及折叠式人工晶状体植入术

目的探讨小瞳孔白内障的超声乳化及折叠式人工晶状体的手术技巧和临床效果。方法临床确诊的小瞳孔白内障患者46例54眼,经透明角膜隧道切口,采用钝性分离的方法扩大瞳孔,行环行撕囊、双手原位超声乳化及折叠式人工晶体囊袋内植入手术。结果术后随访5～12月,术后视力提高46眼占85.19%,视力≥0.5者34眼占62.96%,所有患者的瞳孔保持生理性圆形或是近圆形。手术并发症主要有角膜水肿、前房纤维素性渗出、后囊破裂。结论掌握合理的手术技巧,小瞳孔白内障的超声乳化术同样可以取得良好的手术效果。