Photothermal Iron-Based Riboflavin Microneedles for the Treatment of Bacterial Keratitis via Ion Therapy and Immunomodulation.

Bacterial biofilm formation protects bacteria from antibiotics and the immune system, excessive inflammation further complicates treatment. Here, iron-based metal-organic framework (MIL-101)-loaded riboflavin nanoparticles are designed for the therapeutic challenge of biofilm infection and hyperinflammation in bacterial keratitis. Specifically, MIL-101 produces a thermal effect under exogenous near-infrared light irradiation, which synergizes with ferroptosis-like bacterial death induced by iron ions to exert an effective biofilm infection eradication effect. On the other hand, the disintegration of MIL-101 sustains the release of riboflavin, which inhibits the pro-inflammatory response of macrophage over-activation by modulating their phenotypic switch. In addition, to solve the problems of short residence time, poor permeability, and low bioavailability of corneal medication, the MR@MN microneedle patch is further prepared by loading nanoparticles into SilMA hydrogel, which ultimately achieves painless, transepithelial, and highly efficient drug delivery. In vivo and ex vivo experiments demonstrate the effectiveness of this approach in eliminating bacterial infection and promoting corneal healing. Therefore, the MRMN patch, acting as an ocular drug delivery system with the ability of rapid corneal healing, promises a cost-effective solution for the treatment of bacterial keratitis, which may also lead to a new approach for treating bacterial keratitis in clinics.

Application of sutureless corneal incision for patients with congenital ectopia lentis - Is it feasible, effective and safe?

Purpose: To compare the clinical outcomes, feasibility, and safety between groups with sutured and sutureless wound closure in congenital ectopia lentis (CEL) patients. Methods: Patients with CEL who received phacoemulsification combined with intrascleral fixation of capsular hook (CH) and implantation of capsular tension ring (CTR) and in-the-bag intraocular lens (IOL) were included in this study. Results: A total of 68 eyes of 34 patients aged 18 years or younger were enrolled in this study. Incisions of 21 patients (34 eyes) did not require sutures while sutures were applied in 21 patients (34 eyes). Postoperative uncorrected distance visual acuity, best corrected distance visual acuity and intraocular pressure measurements were comparable on follow-up visits (P > 0.05). The magnitude of surgically induced astigmatism was significantly greater (P = 0.001) in the suture group (Median: 0.47; IQ: 1.63, 2.97) than in the sutureless group (Median: 0.88; IQ: 0.63, 1.35). No cases of endophthalmitis and retinal detachment were found postoperatively in either group, while suture-related complications were observed in the sutured group, including loose suture with discomfort in 5 (14.71%) eyes, loose suture with mucus infiltration in 3 (8.82%) eyes. In total, 22 sutures (64.71%) of 34 eyes required removal. Conclusions: Sutureless clear corneal incision in CEL patients can achieve satisfactory clinical results comparable to sutured wound closure in terms of the efficacy and safety. Advantages of this approach are the reduced risk of suture-related complications, no need for additional surgery under general anesthesia for suture removal, and less cost.

Lens subluxation combined with parry-romberg syndrome: case report.

BACKGROUND: Parry-Romberg syndrome (PRS) is a rare progressive degenerative disorder of unknown etiology. Here we report a rare case of PRS combined with lens subluxation in Eye and ENT hospital of Fudan University, Shanghai. To our knowledge, it is the first reported case of PRS combined with lens subluxation that has been managed surgically with phacoemulsification and CTR placement and IOL implantation in Shanghai. CASE PRESENTATION: A 60-year-old woman was referred for "right visual blur for 2 years" and had persistent right facial paralysis of unknown etiology since the age 12. She had right facial muscle atrophy and paralysis. Eye examination also showed the right eyelid pseudoptosis, enophthalmos, age-related cataract combined with lens subluxation existed in the right eye. The patient was diagnosed as age-related cataract and lens subluxation in the right eye and progressive hemifacial atrophy (Parry-Romberg syndrome). We conducted a combined phacoemulsification, IOL and CTR implantation and pupilloplasty surgery for the patient under general anesthesia and the postoperative UCVA was 20/30 and remained for 1 year's follow up. CONCLUSIONS: Here we reported a rare case of PHA combined with lens subluxation in China. After appropriate eye surgery, the patient achieved satisfying vision result in the right eye.

Safety and efficacy of capsular tension ring and capsular hook implantation for managing ectopia lentis in Marfan syndrome: real-world study.

PURPOSE: To evaluate the safety and efficacy of capsular tension ring and capsular hook (CTR-CH) implantation in Marfan syndrome (MFS) patients with ectopia lentis (EL). SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Retrospective propensity score-matched cohort study. METHODS: This study included patients with MFS who had in-the-bag intraocular lens (IOL) implantation assisted by CTR-CH or modified CTR (MCTR). The safety analysis focused on the resurgery rate. The efficacy analysis compared the corrected distance visual acuity (CDVA) and the incidence of laser capsulotomy after propensity score matching (PSM). RESULTS: This study encompassed 148 eyes that had the CTR-CH procedure and 162 eyes that received MCTR implantation. In the CTR-CH group, the median age at the time of surgery was 5 years, with a mean follow-up duration of 1.81 ± 0.4 years. 5 eyes (3.38%) required a second surgery because of retinal detachment (2, 1.35%), IOL decentration (2, 1.35%), and CH dislocation (1, 0.68%). The resurgery rate was comparable with that of the MCTR group ( P = .486). After PSM, a total of 108 eyes were recruited in each group. Postoperative CDVA was significantly improved in both groups (both P < .001), but comparable between the groups ( P = .057). The posterior capsular opacification took place earlier ( P = .046) while the anterior capsular opacification required laser capsulotomy at a later stage ( P = .037) compared with the MCTR group. CONCLUSIONS: The CTR-CH procedure was a feasible, safe, and efficient approach for managing EL in patients with MFS.

Genotype Impacts Axial Length Growth in Pseudophakic Eyes of Marfan Syndrome.

Purpose: The purpose of this study was to investigate the relationship between axial length (AL) growth and FBN1 genotype in patients with Marfan syndrome (MFS) after lens surgery and customize the selection of intraocular lens (IOL) power. Methods: Patients with MFS who had lens surgery and primary IOL implantation received panel-based next-generation sequencing (NGS). The rate of axial length growth (RALG) was calculated using pre- and postoperative AL measurements and corrected log10-transformed age. A multivariable regression model of RALG was developed after analyzing the effect of FBN1 genotypes and confounding factors. Results: A total of 139 probands of MFS with a median age at lens surgery of 6.25 years (interquartile range [IQR] = 4.67, 12.50 years) were followed up for a median duration of 2.08 years (IQR = 1.16, 3.00 years). The AL growth curve between the age of 3 and 15 years old was logarithmic. Dominant-negative (DN) variants affecting the disulfide-bridge forming cysteines and the conserved residues for calcium-binding had significantly higher RALG than DN variants affecting other structures (P = 0.001) but comparable to that of haplo-insufficiency variants (P = 1.000). Pre-operative AL (b = 0.563, P = 0.011) and genotype constant (b = 2.603, P = 0.011) were significantly associated with RALG in the final model. A Python-based calculator, Marfan IOL Calculator version 2.0, was programmed using the RALG to predict postoperative AL and customize IOL selection based on the ocular biometric parameters and FBN1 genotype. Conclusions: FBN1 genotype impacted the growth of AL in patients with MFS after IOL implantation. Knowing the FBN1 genotype could help cataract surgeons to customize IOL selection.

Predicting axial length in patients with Marfan syndrome and ectopia lentis after modified capsular tension ring and intraocular lens implantation.

PURPOSE: To predict the growth of axial length (AL) in patients with Marfan syndrome (MFS) and ectopia lentis (EL). SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Consecutive retrospective case series. METHODS: Eyes were evaluated that had modified capsular tension ring and intraocular lens (IOL) implantation. The rate of AL growth (RALG) was calculated using AL divided by log10-transformed age. A multivariate linear regression model of RALG was developed after validation. RESULTS: 128 patients with MFS and EL were enrolled with a median follow-up duration of about 3 years. RALG was independent of age between 3 years and 15 years old ( P = .799) and decreased to 0 thereafter ( P = .878). Preoperative AL was associated with RALG in patients under 15 years old ( P = .003). Beta values for the final model of RALG were as below: intercept (-9.794) and preoperative AL (0.664). The postoperative AL was predicted as: postAL = preAL + RALG × log 10 ([postAge + 0.6]/[preAge + 0.6]). The mean prediction error was -0.003 (95% CI, -0.386 to 0.3791) mm and the mean absolute percentage error was 1.93% (95% CI, 0.73% to 3.14%). A Python-based calculator was developed to use the predicted AL in selecting IOL power and setting undercorrection. CONCLUSIONS: The AL growth of patients with MFS followed a logarithmic pattern and ceased at about age 15. A prediction model of postoperative AL was established for individual MFS patients between 3 and 15 years old, which could potentially optimize the IOL power selection.

Changes in Vector Astigmatism After Superotemporal Versus Temporal Clear Corneal Incision Cataract Surgery.

PURPOSE: To investigate vector and refractive astigmatism changes after superotemporal versus temporal clear corneal incision cataract surgery. METHODS: Patients were diagnosed with age-related cataract with corneal astigmatism < 1.5 diopters (D) and were divided into two groups: superotemporal incision (R group) and temporal incision (L group). Uncorrected visual acuity, manifest refraction, corneal topography, anterior segment optical coherence tomography was performed pre- and six months postoperatively. Total ocular astigmatism, corneal astigmatism, vector of surgically induced corneal astigmatism (SICA), non-corneal ocular residual astigmatism (N-CORA), postoperative intraocular lens decentration and tilt were analyzed.  Results: Thirty-eight subjects were included: 21, R group; 17, L group. After surgery, the N-CORA decreased significantly from 1.17±0.72 D to 0.73±0.47 D in all patients (P=0.001), 1.03±0.52 D to 0.70±0.40 D in the R group (P=0.005) and 1.35±0.90 D to 0.78±0.55 D in the L group (P=0.033). Significant differences between t:he R and L groups were found in the postoperative meridian of anterior corneal astigmatism (75.95±52.50 vs 116.79±47.29; P=0.017), total corneal astigmatism (51.65±42.75 vs 95.20±57.32; P=0.011), J45 change vector of SICA in the anterior cornea (-0.10±0.18 vs 0.00±0.11; P=0.048) and total cornea surface (-0.14±0.17 vs 0.03±0.12; P=0.001).  Conclusion: The N-CORA decreased significantly after cataract surgery. Superotemporal and temporal incisions caused differences in the meridian components of oblique astigmatism in some patients but did not have a significant effect on the magnitude of corneal astigmatism.

Structural and functional analysis of SNP rs76740365 G>A in exon-3 of the alpha A-crystallin gene in lens epithelial cells.

Purpose: To clarify the effect of a previously identified single nucleotide polymorphism (SNP; rs76740365 G>A) in the exon-3 of the alpha A-crystallin (CRYAA) gene on the properties of CRYAA and to investigate its function in human lens epithelial cells (HLECs). Methods: The human recombinant wild-type and mutant CRYAA (E156K) were constructed, and the molecular weight was measured by mass spectrometry. The structural changes induced by E156K mutation were analyzed by UV circular dichroism spectra and intrinsic tryptophan fluorescence and were predicted using Schrödinger software. The chaperone-like ability of wild-type and E156K mutant CRYAA was invested against the heat-induced aggregation of ßL-crystallin and the DTT-induced aggregation of insulin. HLECs expressing wild-type and mutated CRYAA were subjected to quantitative PCR (qPCR) and western blot. Cell apoptosis was determined using flow cytometry analysis, and the expression of apoptosis-related proteins were determined using western blot. Results: The mass spectrometric detection revealed that E156K mutation had no significant effect on the apparent molecular mass of the CRYAA oligomeric complex. Evaluation of the structures of the CRYAA indicated that E156K mutation did not significantly affect the secondary structures, while causing perturbations of the tertiary structure. The mutant CRYAA displayed an increase in chaperone-like activity, which might be related to the increase of the surface hydrophobicity. We also predicted that E156K mutation would induce a change from negatively charged surface to positively charged, which was the possible reason for the disturbance to the surface hydrophobicity. Transfection studies of HLECs revealed that the E156K mutant induced anti-apoptotic function in HLECs, which was possibly associated with the activation of the p-AKT signal pathway and downregulation of Casepase3. Conclusions: Taken together, our results for the first time showed that E156K mutation in CRYAA associated with ARC resulted in enhanced chaperone-like function by inducing its surface hydrophobicity, which was directly related to the activation of its anti-apoptotic function.

Comparison of Accuracy in Keratometries Measured by Different Camera Devices for Predicting the Intraocular Lens Power in Lens-Dislocated Patients.

INTRODUCTION: This is a cross-sectional cohort study focused on assessing the influence of ocular biometric parameters of different camera devices for accurately predicting the intraocular lens (IOL) power in the congenital ectopia lentis (EL) patients. METHODS: This study includes a total of 91 eyes of 60 patients with congenital EL from June 2018 to April 2021. All patients underwent lens subluxation surgery with Cionni modified capsular tension rings (MCTR) implantation. Ocular parameters measured by partial coherence interferometry (IOLMaster 700, Carl Zeiss Meditec AG, Jena, Germany) and rotating Scheimpflug camera (Pentacam HR system, Oculus Optikgeräte GmbH, Wetzlar, Germany) were acquired from the database. The authenticity of the different keratometries (K) were analyzed by comparing the prediction error in spherical equivalent under controlled formula SRK/T, Haigis, and after Wang-Koch (WK) adjustment. RESULTS: We observed significant greater K values were obtained in IOLMaster than Pentacam, resulting in more significant hyperopia error while calculating SRK/T. The IOL power calculated with the total corneal refractive power (TCRP) from Pentacam revealed the highest prediction accuracy, indicating that TCRP is the closest to the actual refractive power of the cornea. However, in an exceptional case for long eye patients, total keratometry from IOLMaster was better recommended when using formula Haigis with WK adjustment. CONCLUSIONS: For most instances, TCRP is the best-recommended source of K value while calculating IOL power for EL patients. However, the total keratometry from IOLMaster preferably fits for long eye patients, who require WK adjustment for Haigis formula.

Protective effect of Glutaredoxin 1 against oxidative stress in lens epithelial cells of age-related nuclear cataracts.

Purpose: Glutaredoxin 1 (Grx1) is a key antioxidant protein that catalyzes disulfide redox reactions. In this study, we investigated the expression and protective effect of Grx1 against oxidative stress in nuclear cataracts. Methods: Human anterior capsule membrane samples were obtained from the eyes of cataract patients (experimental group) and non-cataractous (control group) donors. The levels of Grx1 protein and mRNA expression were investigated. The human lens epithelial (HLE) cell line SRA 01/04 was transfected with Grx1-containing plasmid or Grx1 small interfering RNA, and cultured under H2O2 treatment, mimicking oxidative stress conditions. Cell counts, clone formation, cell apoptosis, cell cycle, and levels of oxidized glutathione disulfide and cellular reactive oxygen species (ROS) were evaluated and quantified. Results: Protein and mRNA transcript levels of Grx1 were significantly lower in the human anterior capsule membrane of the age-related nuclear (ARN) cataract group than in the control group. Grx1 overexpression protected HLE cells from H2O2-induced oxidative damage, including alleviating G1 phase arrest, promoting cell proliferation, reducing cell apoptosis, and decreasing intracellular ROS generation. Furthermore, extracellular-signal-regulated kinase (ERK) phosphorylation in the human anterior capsule membrane of ARN patients was higher in the experimental group than in the control group. Grx1 overexpression reduced the levels of oxidized glutathione disulfide and the phosphorylation of ERK. The administration of an ERK phosphorylation inhibitor, PD98059, induced antioxidant effects in Grx1-silenced cells. Conclusions: Grx1 expression is downregulated in the human anterior capsule membrane of ARN patients, accompanied by an increase in ERK phosphorylation. Thus, Grx1 can protect HLE cells against oxidative stress.

Factors Related to Visual Outcomes after Lens Surgery in Isolated Microspherophakia.

Purpose: To evaluate the main factors influencing visual performance after lens subluxation surgery in subjects with isolated MSP. Design: Retrospective study. Methods: In this study, 38 eyes of subjects with isolated MSP (microspherophakia) were included and divided into two groups based on preoperative IOP (intraocular pressure), IOP <21 mmHg, or IOP ≧21 mmHg. Phacoemulsification and scleral-fixated modified capsular tension ring implantation were performed with or without goniosynechialysis according to the IOP. Some ocular biometric parameters, such as corneal curvature, corneal pachymetry, endothelial cell count (ECC), anterior chamber depth (ACD), and axial length, were evaluated. The best-corrected visual acuity (BCVA) and IOP of these subjects were measured before the surgery and during <1 month and 3- to 6-month postoperative follow-ups. Results: Compared with the high IOP group, the normal IOP group was significantly younger and had better preoperative BCVA, a higher ECC, deeper ACD, a lower postoperative IOP, and flatter total corneal refractive power K1. The multivariable analysis revealed that preoperative ACD (b = -0.113, t = -2.070, P=0.047) and preoperative BCVA (b = 0.153, t = 2.562, P=0.015) were significantly associated with postoperative BCVA at 3-6 months. A preoperative ACD of 1.86 mm was found to be the optimal cut-off point for 3- to 6-month postoperative BCVA of ≧20/63 (≤0.52 logMAR). Conclusions: In addition to the effect of normal IOP, better preoperative BCVA and deeper ACD also correlated with better visual outcomes after lens surgery. Preoperative ACD served as a warning for isolated MSP subjects, especially for the risk of irreversible loss of postoperative vision. This trial is registered with "ChiCTR2000039132".

Phacoemulsification Combined With Supra-Capsular and Scleral-Fixated Intraocular Lens Implantation in Microspherophakia: A Retrospective Comparative Study.

Background: Microspherophakia (MSP) is a rare ocular condition, the lens surgery of which is complicated by both insufficient zonules and undersized capsule. Methods: This study included MSP eyes managed with phacoemulsification combined with supra-capsular and scleral-fixated intraocular lens implantation (SCSF-IOL) and made the comparison with those treated by transscleral-fixated modified capsular tension ring and in-the-bag intraocular lens implantation (MCTR-IOL). Results: A total of 20 MSP patients underwent SCSF-IOL, and 17 patients received MCTR-IOL. The postoperative best corrected visual acuity was significantly improved in both groups (P < 0.001), but no difference was found between the groups (P = 0.326). The IOL tilt was also comparable (P = 0.216). Prophylactic Nd:YAG laser posterior capsulotomy was performed 1 week to 1 month after the SCSF-IOL procedure. In the SCSF-IOL group, two eyes (10.00%) needed repeated laser treatment and one eye (5.00%) had a decentered capsule opening. Posterior capsule opacification was the most common complication (6, 35.29%) in the MCTR group. No IOL dislocation, secondary glaucoma, or retinal detachment was observed during follow-up. Conclusions: SCSF-IOL is a viable option for managing MSP and is comparable with the MCTR-IOL. Nd:YAG laser posterior capsulotomy was necessary to prevent residual capsule complications after the SCSF-IOL procedure.

Effect of Different Preoperative Intraocular Pressures on the Prognosis of Traumatic Cyclodialysis Cleft Associated with Lens Subluxation.

PURPOSE: To evaluate the prognosis of patients with blunt trauma-mediated cyclodialysis cleft and lens subluxation treated by dual capsular tension rings (CTRs) with different preoperative intraocular pressures (IOPs). METHODS: Twenty-five patients with cyclodialysis cleft and lens subluxation after blunt trauma were recruited in this study. They were categorized into the low IOP group (IOP < 10 mmHg) and normal IOP group (between 10 mm and 21 mmHg). A modified CTR (MCTR) or CTR was sutured into the ciliary sulcus, and another MCTR or CTR was implanted in the capsular bag. The prognosis outcome measurements, including BCVA (LogMAR), IOP, UBM, and macular character on OCT, were collected during the regular outpatient follow-up after surgery until the recovery of cyclodialysis cleft was found. Measures of cyclodialysis cleft recovery included successful IOP control (defined as an IOP within 10-21 mmHg), confirmation of cleft closure on UBM, and confirmation of IOL centration under slit-lamp examination. Follow-up period or recovery time was regarded as the duration between discharge from the hospital after the operation and the recovery of cyclodialysis cleft. The longest follow-up period was 1 year. RESULTS: Cyclodialysis cleft was successfully treated in 21/25 eyes, whereas lens subluxation was treated effectively in all 25 eyes. CTRs and IOLs were stable in all eyes. BCVA (P < 0.001) and IOP (P < 0.001) were significantly improved after surgery. The primary surgical complication included reversible IOP spike in seven eyes. Postoperative BCVA was found to have correlation with the preoperative IOP (P = 0.016), maculopathy (P = 0.002,), and trauma duration (P = 0.046). Worse BCVA (P = 0.037) and more severe grade of maculopathy (P = 0.030) were observed in the low IOP group (14 eyes). In contrast, a better prognosis and greater probability of IOP spike (P = 0.021) were observed in the normal IOP group (11 eyes). CONCLUSION: Timely and proper intervention for traumatic cyclodialysis cleft associated with lens subluxation is essential. Lower preoperative IOP is associated with worse prognosis and prolonged recovery time, while caution should be taken in IOP spike monitoring in patients with normal preoperative IOP.

Preoperative Characteristics of Ocular Biometry in Children with Unilateral Congenital Cataracts.

The ocular biometry characteristics are clinically significant for children with unilateral congenital cataracts, but there is a lack of data analysis concerning the preoperative measurements. The axial length (AL), mean keratometry (Km), corneal astigmatism (CA), and the anterior chamber depth (ACD) from both eyes before cataract surgery were obtained from 205 patients (410 eyes, 3-15 years of age) with unilateral congenital cataracts. In the congenital cataract eyes, shorter AL (22.44 ± 1.52 mm vs. 22.57 ± 1.04 mm, p = 0.036) and higher CA (- 1.89 ± 0.91 D vs. - 1.24 ± 0.67 D, p < 0.001) were found, and no significant difference was found in the Km and the ACD measurements compared to the contralateral normal eyes. Females had shorter AL and shallower ACD compared to males. However, the Km and CA in the females were significantly larger than that in males. Shorter AL, larger Km, higher CA, and shallower ACD were also found in females who had a binocular axial difference (the value obtained by subtraction of the contralateral normal eye from the congenital cataract eye) that less than zero. The preoperative ocular biometry of shorter AL, larger Km, higher CA, and shallower ACD should be considered in females with unilateral congenital cataracts. The age and the binocular axial differences had a statistically significant correlation (r = -0.192, p = 0.006). Therefore, changes in the binocular axial differences associated with aging may enhance the guidelines for intraocular lens selection and the management of congenital cataracts.

Fibrillin-1 gene mutations in a Chinese cohort with congenital ectopia lentis: spectrum and genotype-phenotype analysis.

AIMS: To identify the mutation spectrum and genotype-phenotype correlations of fibrillin-1 (FBN1) mutations in a Chinese cohort with congenital ectopia lentis (EL). METHODS: Patients clinically suspected of congenital zonulopathy were screened using panel-based next-generation sequencing followed by multiplex ligation-dependent probe amplification. All the probands were subjected to thorough ocular examinations. Molecular and clinical data were integrated in pursuit of genotype-phenotype correlation. RESULTS: A total of 131 probands of FBN1 mutations from unrelated families were recruited. Around 65% of the probands were children younger than 9 years old. Overall, 110 distinct FBN1 mutations were identified, including 39 novel ones. The most at-risk regions were exons 13, 2, 6, 15, 24 and 33 in descending order of mutation frequency. The most prevalent mutation was c.184C>T (seven, 5.34%) in the coding sequence and c.5788+5G>A (three, 2.29%) in introns. Missense mutations were the most frequent type (103, 78.63%); half of which were distributed in the N-terminal regions (53, 51.46%). The majority of missense mutations were detected in one of the calcium-binding epidermal growth factor-like domains (62, 60.19%), and 39 (62.90%) of them were substitutions of conserved cysteine residues. Microspherophakia (MSP) was found in 15 patients (11.45%). Mutations in the middle region (exons 22-42), especially exon 26, had higher risks of combined MSP (OR, 5.51 (95% CI 1.364 to 22.274), p=0.017). CONCLUSIONS: This study extended the knowledge of the FBN1 mutation spectrum and provided novel insights into its clinical correlation regarding EL and MSP in the Chinese population.

Analysis of Corneal Spherical Aberrations in Chinese Bilateral Ectopia Lentis Patients.

Purpose: To analyze the anterior, posterior, and total corneal spherical aberrations (ASA, PSA, and TSA) in patients with Chinese bilateral ectopia lentis (EL). Methods: A cross-sectional study was conducted to evaluate corneal spherical aberration (CSA) using a Pentacam system at the 6-mm optical zone. Axial length, keratometry, astigmatism, and corneal asphericity were also determined. Results: This study included 247 patients (420 eyes) with a mean age of 18.1 years. The values of ASA, PSA, and TSA were 0.136 ± 0.100 µm, -0.118 ± 0.030 µm, and 0.095 ± 0.095 µm, respectively. In the EL patients with Marfan syndrome (MFS), ASA and TSA were significantly lower than in the non-MFS patients (0.126 ± 0.094 µm vs. 0.155 ± 0.107 µm, P = 0.004 for ASA; 0.085 ± 0.091 µm vs. 0.114 ± 0.099 µm, P = 0.003 for TSA), whereas PSA was not significantly different (P = 0.061). The values of ASA and TSA were significantly higher in the patients with EL aged ≥ 40 years old than in younger patients, whereas ASA and PSA were lower in patients aged <10 years old than in older patients (all P < 0.05). In the multiple linear regression analysis, age, keratometry, astigmatism, anterior asphericity, higher-order aberration (HOA), and lower-order aberration (LOA) were positively or negatively correlated with TSA in the patients with EL (r = 0.681, P < 0.001). Conclusions: Corneal spherical aberration was low in the patients with EL especially for MFS and tended to increase with aging. Preoperatively, individual measurement of CSA was necessary for bilateral EL patients with MFS.

Primary scleral-fixated posterior chamber intraocular lenses in patients with congenital lens subluxation.

OBJECTIVE: We investigated the long-term visual outcomes and ocular complications of primary scleral-fixated posterior chamber intraocular lenses (SF-PC-IOLs) in patients with congenital lens subluxation. METHODS: We enrolled 53 patients (77 eyes) with congenital lens subluxation caused by ectopia lentis, Marfan syndrome, and Weill-Marchesani syndrome who underwent primary implantation of a SF-PC-IOL. All patients underwent a complete ophthalmic examination include visual acuity (VA), intraocular pressure (IOP), intraocular lenses (IOL) position, intraoperative complications and postoperative complications. Cox regression analysis and survival analysis were used to evaluate the risk factors for postoperative complications. RESULTS: Seventy seven eyes from 53 patients were included. Mean age at surgery was 23 ± 20 years (5 to 67 years), with a mean follow-up of 39 ± 27 months (12 to 130 months). The best-corrected VA improved from 0.84 ± 0.55 to 0.26 ± 0.43 logarithms of the minimum angle of resolution (p < 0.001). Best-corrected VA improved postoperatively in 73 eyes (94%). The main causes of reduced vision after surgery were retinal pathologies and amblyopia. Complications included transient intraocular haemorrhage (2 eyes, 2.6%), early vitreous incarceration (2 eyes, 2.6%), retinal detachment (6 eyes, 7.8%) and IOL dislocation (3 eyes, 3.9%). Cox regression showed that postoperative eye trauma was a risk factor for long-term postoperative complications. CONCLUSION: SF-PC-IOLs provide good visual outcomes in patients with congenital lens subluxation. The SF-PC-IOLs showed good stability, except in patients suffering from postsurgical eye trauma.

Accuracy of IOL power calculation formulas in Marfan lens subluxation patients with in-the-bag IOLs and implantation of scleral-sutured single-eyelet modified capsular tension rings.

PURPOSE: To investigate the accuracy of intraocular lens (IOL) formulas for the prediction of postoperative refraction in lens subluxation in Marfan syndrome. SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Consecutive retrospective clinical observational case series. METHODS: 60 eligible eyes with lens subluxation from 39 young patients with Marfan syndrome (8.53 ± 4.38 years) underwent phacoemulsification combined with single-eyelet modified capsular tension ring (MCTR) and IOL implantation. The prediction error values with mean zero out (relative prediction error) and their absolute values (AE) were calculated. RESULTS: Generally, the SRK/T formula with Wang-Koch (WK) adjustment had the lowest median AE at 0.418 diopters (D), and the Holladay 1 with WK adjustment had the lowest mean AE at 0.499 D. The median AE of the other 10 formulas, in order from lowest to highest, were Haigis with WK (0.494 D), Holladay 1 with WK (0.495 D), Hoffer Q with WK (0.508 D), Haigis (0.525 D), T2 (0.542 D), Hoffer Q (0.624 D), SRK/T and Holladay 1 (0.660 D), Super (0.680 D), and Barrett Universal II (0.714 D) formulas. Haigis formula was found to be statistically significantly different from SRK/T, Holladay 1, and Barrett Universal II (all 3 P < .001) but not Hoffer Q (P = .236) formula. CONCLUSIONS: The Haigis formula was recommended for young Marfan lens subluxation patients with in-the-bag IOLs and scleral-sutured single-eyelet MCTR implantation. WK adjustments were successful in those cases where the axial length was longer than 25.0 mm.

Analysis of Axial Length in Young Patients with Marfan Syndrome and Bilateral Ectopia Lentis by Z-Scores.

INTRODUCTION: Marfan syndrome (MFS) is characterized by ectopia lentis (EL) and elongated axial length (AL). The characteristics of AL in young patients with MFS and bilateral EL before the lens surgery are not fully understood. METHODS: This study reviewed MFS patients under 20 years old with bilateral EL from January 2015 to October 2020. The Z-scores were introduced in terms of the number of standard deviations from the mean of age-matched normative data. Using Z-scores, the distribution of AL and influence factors were evaluated. The correlations between AL and other biometrics were analyzed. RESULTS: We reviewed 183 patients and enrolled both eyes. The mean age was 8.44 ± 4.69 years. About 36% of the patients were children under 6 years old. The median AL increased from 23.16 mm under 5 years old to 26.20 mm in the 16-20 age group, and when plotted, the trend presented a logarithmic curvature (R2 = 0.145, p < 0.001). The median Z-AL score was 1.24. One-third of eyes had Z-score <0. About 20% of the patients had AL difference over 1 mm between the right and left eyes, and the right one had longer Z-AL scores (p = 0.013). The eye complicated with megalocornea (10, 7.04%) had larger Z-AL scores (4.72 ± 3.51 vs. 1.10 ± 2.25, p = 0.002). A positive correlation was found between Z-AL and Z-corneal curvature radius (r = 0.265, p < 0.001). CONCLUSION: Young patients with bilateral EL but small AL should not be excluded from MFS without systematic examination. The age-adjusted Z-score will facilitate further study of the individual variations in AL across different ages.

Surgical outcomes of modified capsular tension ring and intraocular lens implantation in Marfan syndrome with ectopia lentis.

OBJECTIVE: To evaluate surgical outcomes of modified capsular tension ring (MCTR) and intraocular lens (IOL) implantation in eyes of Marfan syndrome (MFS). METHODS: This retrospective case series included MFS patients receiving MCTR transscleral fixation and IOL in-the-bag implantation. The MCTR was sutured to the sclera through a sulcus by 9-0 polypropylene with the modified knotless Z-suture technique. Main outcomes were visual acuity and postoperative complications. RESULTS: A total of 109 MFS patients (187 eyes) had the surgery. Patients were 15.47 ± 14.36 years old and followed up for 10.07 ± 8.99 months. MCTR and IOL implantations were performed in 174 eyes (93.05%). The postoperative BCVA (LogMAR) was 0.24 ± 0.24 (1 month), 0.26 ± 0.20 (3 months), 0.22 ± 0.20 (6 months), 0.20 ± 0.19 (12 months) and 0.25 ± 0.32 (over 12 months), which was significantly better than preoperatively (0.63 ± 0.35) (p < 0.001). Major complications included posterior capsular opacification (PCO) in 41 eyes (23.56%) and decentered anterior capsular opacification (ACO) in 15 eyes (8.62%). The mean occurrence time was 9.44 ± 7.08 and 8.87 ± 15.08 months respectively. Other complications included transient intraocular pressure elevation in seven eyes (4.02%), retinal detachment in two eyes (1.15%), cystoid macula edema in one eye (0.57%), and endophthalmitis in one eye (0.57%). No eye had suture breakage or IOL dislocation. CONCLUSIONS: The visual improvement is significant in eyes of MFS undergoing MCTR transscleral fixation and IOL in-the-bag implantation. The long-term monitoring of complications is warranted, especially in the first-two years.