Effect of lens surgery on health-related quality of life in preschool children with congenital ectopia lentis

AIM:To evaluate the effect of lens surgery on health-related quality of life(HRQoL)of preschool children with congenital ectopia lentis(CEL).METHODS:A prospective self-controlled study was conducted in Zhongshan Ophthalmic Center.Children aged from 5 to 7y whom were diagnosed with CEL and underwent phacoemulsification with scleral-fixated posterior chamber intraocular lens implantation and their parents were enrolled in this study.All of them completed the child and proxy(parental)PedsQL™4.0 before and after the surgery.Their preoperative scores were compared to their postoperative ones.Subgroup analyses were performed based on gender and preoperative bilateral presenting visual acuity of the children.RESULTS:Thirty-two children with CEL successfully underwent surgery without any complications,among whom 8 had monocular surgery and 24 had binocular surgery.Preoperative and postoperative questionnaires were completed by 32 child-parent pairs.Surgical intervention could significantly improve the vision of affected children(P<0.001).The medians of physical,psychosocial and total health scores self-reported by the children were 68.75(62.50,81.25),65.00(60.00,80.00)and 67.39(60.87,78.26)preoperatively and were 93.75(87.50,100.00),90.00(83.33,96.67)and 89.13(85.32,95.65)postoperatively.The preoperative scores of the affected children were significantly lower in all scales than age-matched healthy children(P<0.001).All the postoperative scores were significantly higher than the preoperative scores in affected children and their parents(P<0.001).In the physical functioning evaluation,the preoperative score reported by parents of girls was higher than parents of boys(P=0.041),and the postoperative score of girls was higher than that of boys(P=0.036).CONCLUSION:CEL is associated with significantly worse quality of life in preschool children.Surgical intervention can significantly improve the HRQoL in affected children from both personal and family perspective.

LENTIS Comfort Toric人工晶状体植入术后视觉质量分析

目的:探讨白内障手术中植入LENTIS Comfort Toric人工晶状体后的视觉质量。方法:回顾性对照临床研究,收集数字化导航辅助下白内障超声乳化摘除联合人工晶状体植入术患者40例40眼,植入LENTIS Comfort Toric(MF15T)人工晶状体为A组(24眼),植入单焦点Toric人工晶状体为B组(16眼);观察术后3mo不同距离视力、术后散光质心值、脱镜率、离焦曲线变化以及人工晶状体稳定性等。结果:两组患者术后均无严重并发症出现,两组术后3mo远视力无差异(P>0.05);A组中视力、近视力明显优于B组(P<0.01);A组术后脱镜率为99%,B组为45%(P<0.05);两组术后3mo散光质心值均较术前改善,但组间无差异(P>0.05);术后3mo A组离焦曲线于+0.50、-1.75D分别出现峰值,形成平台并平缓下降,B组于+0.25、-0.25D出现峰值后呈陡峭趋势下降。术后1d A组出现1眼(4%)人工晶状体轴位旋转大于10°;B组出现6眼(38%)人工晶状体轴位旋转大于10°,2眼(33%)顺时针旋转,4眼(67%)逆时针旋转;两组均无人工晶状体移位发生。结论:两种人工晶状体均可矫正散光,其中LENTIS Comfort Toric人工晶状体能为白内障合并散光人群提供个性化的选择,可同时解决散光和获得全程视力,术后光学干扰发生率低,稳定性更好,视觉质量及满意度高。

Agreement of corneal curvature and central corneal thickness obtained from a swept-source OCT and Pentacam in ectopia lentis patients

AIM:To assess the inter-device consistency of corneal curvature and central corneal thickness between Pentacam and a swept-source Fourier-domain anterior segment optical coherence tomography(AS-OCT)in ectopia lentis patients.METHODS:Totally 72 eyes of ectopia lentis patients were recruited.Central corneal thickness(CCT),corneal curvature values and corneal astigmatism were obtained from both the Pentacam and AS-OCT(CASIA2).Repeatability was evaluated for both devices.The coef ficient of repeatability(COR)and the relative COR was calculated.Bland-Altman plots were conducted to evaluate the interdevice agreement of measurement.Orthogonal linear regression was used to examine any proportional bias.RESULTS:The mean difference of CCT,steep anterior corneal cur vature(anterior K_S),flat anterior corneal curvature(anterior K_f),anterior corneal astigmatism(ACA),steep posterior corneal cur vature(posterior K_S),flat posterior corneal cur vature(posterior K_f),posterior corneal astigmatism(PCA),steep true net power(TNP K_S),flat true net power(TNP K_f)and total corneal astigmatism(TCA)between Pentacam and CASIA2 were 7.03±9.70μm,-0.19±0.41 D,-0.27±0.35 D,0.04±0.47 D,-0.17±0.23 D,-0.11±0.11 D,-0.02±1.02 D-0.41±0.43 D,-0.52±0.46 D,and-0.15±0.96 D,respectively.For measurement of TNP K_f with the Pentacam and CASIA2,a mean difference of 0.52 D and COR of 0.90 with P=0.02 was detected.There was no significant difference in CCT(P=0.393),anterior K_f(P=0.107),anteriorKs(P=0.414),ACA(P=0.131),posterior K_f(P=0.286),posterior Ks(P=0.418),PCA(P=0.105),TNP Ks(P=0.054),and TCA(P=0.977)between Pentacam and CASIA2.CONCLUSION:Our study reveals good agreement of CCT,corneal curvature and corneal astigmatism measured by CASIA2 and Pentacam in ectopia lentis patients.However,there was significant difference for CCT and corneal curvature values obtained by the two devices.

Trends and characteristics of congenital ectopia lentis in China

AIM： To elucidate the trends and characteristics of congenital ectopia lentis（CEL） in southern China.METHODS： CEL patients from China admitted to Zhongshan Ophthalmic Center（ZOC） from January 2006 to December 2015 were recruited in our study. Residence, gender, hospitalization time, age at surgery, and the presence of other ocular abnormalities and system disease were statistically analyzed in different subgroups.RESULTS： Four hundred and thirty-seven hospitalizations（306 in-patients） diagnosed with CEL from a total of 283 308 hospitalizations were identified, which accounted for 0.15% of the total in-patients. Of the identified CEL in-patients, the total ratio of boys to girls was 2.22：1. Based on a subgroup analysis according to age, patients aged 12-18 years old constituted the highest proportion（31.70%） of all hospitalized CEL patients, and those 0-3 year old constituted the lowest proportion（8.82%） of the total number. The number of CEL increased from 18 to 72 and the hospital based prevalence increased from 8.60% to 18.10% from 2006 to 2015, and the average age at surgery decreased from 9 years old in 2006 to 7.6 years old in 2015. CONCLUSION： The results reveal upward trends in both the number of CEL hospitalizations and hospital based prevalence of CEL in this 10-year study period, but a reduction in the age at surgery, which may reflect the increase of public awareness of children＇s eye care in China.

Evaluation of functional outcome and stability of sutureless scleral tunnel fixated IOLs in children with ectopia lentis

AIM:To evaluate functional outcome of sutureless scleral tunnel intraocular lens(SSTIOL)in children with crystalline lens subluxation of more than 7 clock hours.METHODS:A prospective interventional study was conducted consisting of 45 eyes of 44 children in age group 6-18 y having>7 clock hours of lens subluxation who underwent lensectomy-vitrectomy followed by SSTIOL implantation.Primary outcome was improvement in best corrected visual acuity(BCVA)and secondary outcomes were assessment of intraocular lens(IOL)tilt using ultrasound biomicroscopy(UBM),mean change in astigmatism at last follow-up of 1 y and associated complications.RESULTS:The mean preoperative and postoperative BCVA was 1.05±0.28 and 0.64±0.45(log MAR)respectively(P=0.001)at last follow-up.The mean astigmatism preoperatively and postoperatively was-4.17±2.69 D and-1.86±1.25 D respectively(P=0.011).Significant IOL tilt(>5 degrees)was present in 5 cases.The mean percentage endothelial loss was 3.65%±1.92%.The most serious complication encountered was retinal detachment seen in 2 cases.CONCLUSION:SSTIOL implantation provides efficient visual rehabilitation in children provided there is stringent case selection.We recommend caution in children having white-to-white distance>12 mm and presence of peripheral retinal degenerations.

A novel FBN1 missense mutation (p.C102Y) associated with ectopia lentis syndrome in a Chinese family

AIM: To characterize the disease-causing mutations in a Chinese family with ectopia lentis syndrome (ELS). METHODS: Patients and their family members were given complete physical, ophthalmic, and cardiovascular examinations. Genomic DNA samples were extracted from the peripheral blood of the pedigree members and 100 healthy controls. Mutation screening was performed in the fibrillin -1 (FBN1) gene by bi -directional sequencing of the amplified products. The mutation was analyzed using two bioinformatics methods. RESULTS: A novel heterozygous c.305G>A mutation in exon 3 of FBN1 was detected. As a result of this change, a highly conserved cysteine residue was replaced by a tyrosine residue (p.C102Y). Another mutation was found in the same exon (c.303T>C), which did not change the amino acid sequence. Both mutations were discovered in each affected individual, but not in the unaffected family members, or in 100 ethnically matched controls. A bioinformatics analysis predicted that mutation p.C102Y would affect protein function. CONCLUSION: In the first epidermal growth factor-like module, we identified a novel FBN1 mutation (p.C102Y), which caused ELS in the family. Our study presented a unique phenotype, including some distinct ophthalmic findings, such as hypoplasia of the iris and anisometropia. Our results expanded the mutation spectrum of FBN1 and enriched the overall knowledge of genotype-phenotype correlations due to FBN1 mutations.

Surgical management of non-syndromic ectopia lentis

AIM:To compare whether aphakic contact lenses or secondary iris-claw intraocular lenses are superior in the refractive management post-pars plana vitreolensectomy in a pedigree with an FBN1 mutation causing non-syndromic ectopia lentis(NSEL)with retinal detachment(RD).METHODS:Eight affected individuals had pars plana vitreolensectomy for bilateral ectopia lentis(EL).Twelve eyes of 6 patients had secondary iris-claw intraocular lenses inserted and 4 eyes of 2 patients were managed with contact lenses.Rhegmatogenous retinal detachment(RRD)was treated when necessary.Pre-and post-operative assessment included visual acuity,endothelial cell count and dilated fundal examination.RESULTS:Macula-on RRD was present in all individuals>18 y,64%(7/11 eyes)presenting post-vitreolensectomy with 57%having bilateral non-synchronous RRD.Surgical aphakia was managed with iris-fixated intraocular lenses(IOL group,n=6),or contact lenses(CL group,n=2).Visual acuity≥0.3 log MAR(driving standard)was achieved in 75%of IOL group eyes and 25%of the CL group eyes.Mean loss of corneal endothelial cell count in the IOL group was 4%at 2 y post-operative.CONCLUSION:In this cohort,refractive management with iris-claw IOLs provided superior outcomes to contact lenses and the authors recommend this as the optimal refractive correction in EL patients.

The refractive outcome of Toric Lentis Mplus implant in cataract surgery

AIM： To evaluate the refractive outcome of Toric Lentis Mplus intraocular lens（IOL） implant.· METHODS： This is a retrospective case series.Consecutive patients with corneal astigmatism of at least1.5 D had Toric Lentis Mplus IOL implant during cataract surgery. The exclusion criteria included irregular astigmatism on corneal topography, large scotopic pupil diameter（〉6 mm）, poor visual potential and significant ocular comorbidity. Postoperative manifest refraction,uncorrected distance visual acuity（UDVA）, best-corrected distance visual acuity（BCVA）, uncorrected intermediate visual acuity at（UIVA） 3/4 m and uncorrected near visual acuity（UNVA） were obtained.·RESULTS： There were 70 eyes from 49 patients in this study. Patients were refracted at a median of 8.9wk（range 4.0 to 15.5） from the operation date. Sixty-five percent of eyes had 6/7.5（0.10 log MAR） or better, and99% 6/12（0.30 log MAR） or better postoperative UDVA.Eighty-nine percent could read Jaeger（J） 3（0.28 log MAR）and 95% J5（0.37 log MAR） at 40 cm. The median magnitude of astigmatism decreased from 1.91 D to 0.49 D（Wilcoxon, P〈0.001） after the operation. The range of the cylindrical error was reduced from 1.5-3.95 D（keratometric） preoperatively to 0.00-1.46 D（subjective refraction transposed to corneal plane） postoperatively.· CONCLUSION： Toric Lentis Mplus IOL has good predictability in reducing preexisting corneal astigmatism.

Mutation analysis of FBN1 gene in two Chinese families with congenital ectopia lentis in northern China

AIM: To summarize the phenotypes and identify the underlying genetic cause of the fibrillin-1(FBN1) gene responsible for congenital ectopia lentis(EL) in two Chinese families in northern China.METHODS: A detailed family history and clinical data from all participants were collected by clinical examination. The candidate genes were captured and sequenced by targeted next-generation sequencing, and the results were confirmed by Sanger sequencing. Haplotyping was used to confirm the mutation sequence. Real-time PCR was used to determine the FBN1 messenger ribonucleic acid(m RNA) levels in patients with EL and in unaffected family members.RESULTS: The probands and other patients in the two families were affected with congenital isolated EL. A heterozygous FBN1 mutation in exon 21(c.2420_IVS20-8 del TCTGAAACAins CGAAAG) was identified in FAMILY-1. A heterozygous FBN1 mutation in exon 14(c.1633 C>T, p.R545 C) was identified in FAMILY-2. Each mutation cosegregated with the affected individuals in the family and did not exist in unaffected family members and 200 unrelated normal controls.CONCLUSION: The insertion-deletion mutation(c.2420 IVS20-8 del TCTGAAACA ins CGAAAG) in the FBN1 gene is first identified in isolated EL. The mutation(c.1633 C>T) in the FBN1 gene was a known mutation in EL patient. The variable phenotypes among the patients expand the phenotypic spectrum of EL in a different ethnic background.

Acute lens-induced glaucomas: A review

Lens-induced factors are important and common mechanisms causing acute elevation of intraocular pressure. While in most cases, the diagnosis and management are straight-forward, in others it is difficult and can lead to improper procedures, complications and poor visual outcomes. This review was done with the aim of studying the various types of lens-induced glaucomas, classifying them in an easy way to understand manner, their clinical features, current management and future possibilities.

Modern Surgical Treatment of Congenital Subluxated Lenses

Traditional surgical management of subluxated lenses is usuallyassociated with a high incidence of operative and postoperative complicationand a poor visual outcome.We treated a series of 14 patients(26 eyes)whose visual acuity could not be improved with optical correction(phakic oraphakic)with modern microsurgery and automated suction-cutting devices.Preoperative visual acuity ranged from 0.05 to 0.3.Improvement in post-opera-tive visual acuity was documented in all operated eyes,ranging from 0.4 to 1....