Transscleral suture fixation in congenital ectopia lentis
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ABSTRACT:
SUBMITTER: Panos G
PROVIDER: S-EPMC10113099 | biostudies-literature | 2023 Mar
REPOSITORIES: biostudies-literature
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Project description:IntroductionThis study aimed to compare modified knotless transscleral suture fixation of intraocular lens (IOL) with traditional transscleral suture fixation for adolescents and young patients with congenital ectopia lentis (CEL).MethodsThis retrospective cohort study included 49 patients with CEL (60 eyes) who underwent surgery at the Zhongshan Ophthalmic Center. Improvements based on knotless Z-suture fixation technique were made to form a modified knotless method, in which thicker 8-0 polypropylene sutures were used, and double parallel scleral grooves were constructed behind the limbus instead of triangular lamellar scleral flaps to cover suture stitches. Modified knotless transscleral fixation of IOL was conducted on 30 eyes, and the other 30 eyes underwent traditional transscleral fixation surgery. Pre- and postoperative best-corrected visual acuity (BCVA), refractive error, astigmatism, other ocular parameters, and complications were statistically analyzed.ResultsFor patients in the modified knotless group, the mean cylindrical refractive error and astigmatism at 1 month and 3 months postoperative were lower (all P < 0.05), and the mean IOL tilt degree was smaller at 3 months postoperative (3.21° ± 2.13° vs. 5.65° ± 3.66°, P = 0.032). The incidence of suture exposure in the modified knotless group was also lower than in the controls (0 vs. 16.7%, P = 0.026). No group differences were observed in mean BCVA, spherical equivalent, or other ocular biometric parameters between groups.ConclusionModified knotless technique was a valid method to achieve optimal IOL position and reduce postoperative astigmatism for adolescents and young patients with CEL. It effectively reduced the incidence of knot-related complications, greatly improved the postoperative comfort, and achieved aesthetic benefits.
| S-EPMC9834442 | biostudies-literature
Project description:PurposeTo report the long-term outcomes of transscleral fixation of capsular tension rings (CTR) with intraocular lens (IOL) placement in pediatric patients with ectopia lentis.SettingJohn A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA.DesignRetrospective case series.MethodsPediatric patients requiring lens extraction with transscleral capsular bag fixation with a CTR and IOL for ectopia lentis between January 2006 and January 2016 were analyzed.ResultsThirty-seven patients (67 eyes) who had transscleral fixation of the capsular bag using a CTR fixated with 9-0 or 10-0 polypropylene (Prolene), 8-0 polytetrafluoroethylene (Gore-Tex), or 9-0 nylon were identified. The mean age at time of surgery was 7.25 years (2 to 18 years) and the mean follow-up was 35.3 months (0.25 to 120 months). The proportion of eyes showing improvement in corrected distance visual acuity (CDVA) postoperatively was 78.5%, which demonstrated significance with a 95% confidence interval. In the immediate postoperative period, 1 eye developed a hyphema and 1 eye required IOL repositioning. Long-term complications included posterior capsule opacification in 35 eyes (52%) and uveitis-glaucoma-hyphema syndrome in 1 eye (1.5%). Three eyes (4.4 %) required IOL repositioning for spontaneous delayed IOL dislocation, 2 sutured with 8-0 polytetrafluoroethylene at postoperative month 8 and postoperative year 3 and 1 sutured with 9-0 polypropylene at postoperative year 7.ConclusionTransscleral fixation of the capsular bag using a CTR improved CDVA and provided IOL stability in pediatric patients with ectopia lentis.
| S-EPMC6615904 | biostudies-literature
Project description:PurposeThe purpose of this study was to investigate the characteristics of corneal higher-order aberrations (HOAs) in patients with congenital ectopia lentis (CEL).MethodsClinical characteristics and HOAs of 60 patients with CEL and 75 healthy controls at Zhongshan Ophthalmic Center in China were retrospectively analyzed. The Q value and the corneal HOAs in the CEL group and the controls were measured by using Pentacam and compared value between the CEL and control groups. The correlation between HOAs and age was investigated using the Pearson correlation analysis.ResultsThe Q value of anterior corneal surface in the CEL group was larger than that in the controls (-0.41 ± 0.17 vs. -0.32 ± 0.13, P = 0.001); the total corneal horizontal coma in the CEL group were larger than that in the controls (0.24 ± 0.18 vs. -0.05 ± 0.14, P < 0.001); both the primary spherical aberrations of the anterior and total corneal surface were lower in the CEL group than that in the controls (for anterior corneal surface: 0.15 ± 0.08 vs. 0.27 ± 0.08 µm, P < 0.001; for total corneal surface: 0.10 ± 0.09 vs. 0.23 ± 0.09 µm, P < 0.001), the anterior and total corneal horizontal coma were negatively associated with age, whereas the anterior and total corneal spherical aberrations were positively associated with age in patients with CEL.ConclusionsPatients with CEL had higher corneal horizontal coma and lower corneal vertical coma primary spherical aberrations than healthy controls.Translational relevanceThese findings are informative for the clinical managements in patients with CEL.
| S-EPMC8383910 | biostudies-literature
Project description:BackgroundCongenital ectopia lentis (EL) refers to the congenital dysplasia or weakness of the lens suspensory ligament, resulting in an abnormal position of the crystalline lens, which can appear as isolated EL or as an ocular manifestation of a syndrome, such as the Marfan syndrome. The fibrillin-1 protein encoded by the FBN1 gene is an essential component of the lens zonules. Mutations in FBN1 are the leading causes of congenital EL and Marfan syndrome. Owing to the complexity and individual heterogeneity of FBN1 gene mutations, the correlation between FBN1 mutation characteristics and various clinical phenotypes remains unclear.MethodsThis study describes the clinical characteristics and identifies possible causative genes in eight families with Marfan syndrome or isolated EL using Sanger and whole-exome sequencing.ResultsEight FBN1 mutations were identified in these families, of which three (c.5065G > C, c.1600 T > A, and c.2210G > C) are reported for the first time. Based on in silico analyses, we hypothesized that these mutations may be pathogenic by affecting the fibrillin-1 protein structure and function.ConclusionThese findings expand the number of known mutations involved in EL and provide a reference for the research on their genotype and phenotype associations.
| S-EPMC10178797 | biostudies-literature
Project description:ObjectivesThe aim of our study is to give insight into congenital ectopia lentis (CEL) patients' care-seeking behaviour and explore the factors affecting their follow-up visits.DesignCross-sectional study; in-depth and face-to-face semistructured interview.SettingA large-scale ophthalmology hospital in China.Participants35 patients with CEL and their parents from May 2017 to August 2017.Main outcome measuresThemes and categories. The interviews were audio-recorded, transcribed verbatim, coded and analysed using grounded theory. Data collection was closed when new themes did not emerge in subsequent dialogues.ResultsThe factors affecting the timely visits included insufficient awareness of CEL, shame on hereditary disease, lack of effective doctor-patient communication, lack of reliable information online and daily stressors.ConclusionContinuing medical education of severe and rare disease, reforming the pattern of medical education, constructing an interactive platform of the disease on the internet and improving healthcare policy are effective ways to improve the diagnosis and treatment status of CEL in China.
| S-EPMC7069255 | biostudies-literature
Project description:PurposeTo investigate the characteristics of anterior chamber angle parameters in congenital ectopia lentis (CEL) patients and to evaluate the sensitivity and specificity of anterior segment parameters in distinguishing CEL from healthy controls. Setting. Zhongshan Ophthalmic Center, Guangzhou, China.DesignCross-sectional study.Methods35 CEL patients and 35 age- and sex-matched healthy controls were recruited. Axial length (AL) and anterior segment parameters including anterior chamber width (ACW), angle open distance (AOD), angle recess area (ARA), trabecular-iris space area (TISA), and trabecular-iris angle (TIA) were measured. All the above parameters and the ratio index of angle parameters, which was defined as the angle parameter value of the narrower side to that of the contralateral side, were compared between CEL and controls. Receiver operating characteristic (ROC) curves were also plotted to evaluate the diagnostic performance of anterior chamber angle parameters in CEL patients.ResultsAll angle parameters of the contralateral side to the dislocated lens side were significantly smaller than those of the dislocated lens side in CEL (all P < 0.05). For the diagnostic performance of anterior chamber angle parameters, the ratio index of TIAr500 combined with TIAr750 had the best diagnostic performance for CEL screening (AUC = 0.798), and TIAr500 of 0.887 and TIAr750 of 0.917 were detected to be the optimal cut-off points, representing a sensitivity of 89.8% and specificity of 68.7%.ConclusionThe contralateral side to the dislocated lens side in the CEL had a narrower anterior chamber angle. TIAr500 combined with TIAr750 is the optimal combination strategy for ectopia lentis screening.
| S-EPMC9192328 | biostudies-literature
Project description:PurposeTo identify the spectrum and frequency of five candidate genes in Chinese patients with congenital ectopia lentis (EL).MethodsForty consecutive and unrelated congenital probands with EL were collected and underwent ocular, skeletal, and cardiovascular examinations. Sanger sequencing was used to analyze all of the coding and adjacent regions of five candidate genes: FBN1, ADAMTS10, ADAMTSL4, TGFBR2, and CBS. Mutation analysis was performed to evaluate the pathogenic variants and to identify the cause of congenital EL.ResultsThe FBN1 gene screen revealed 25 pathogenic variants in 34 of the 40 families with congenital EL, including three novel (c.1955G>T, c.2222delA, and c.4381T>C) and 22 known mutations. The ADAMTSL10 gene screen revealed a compound heterozygous variant (c.1586G>A and c.2485T>A) in a family with Weill-Marchesani syndrome (WMS). In the remaining five probands, no pathogenic variant was detected in any of the five screened genes.ConclusionsIn this study, we identified three novel and 22 known mutations in FBN1 in 34 of 40 EL families. The results expand the mutation spectrum of the FBN1 gene and suggest that FBN1 mutations may be the major cause of congenital EL in Chinese patients.
| S-EPMC4105116 | biostudies-literature
Project description:PurposeThe purpose of this study was to develop an artificial intelligence (AI)-based intraocular lens (IOLs) power calculation formula for improving the accuracy of IOLs power calculations in patients with congenital ectopia lentis (CEL).MethodsA total of 651 eyes with CEL that underwent IOLs implantation surgery were included in this study. An AI-based ensemble formula-the Jiang Formula, was developed using a training dataset of 520 eyes and evaluated on a testing dataset of 131 eyes. A five-fold cross-validation and a two-layer ensemble learning model were constructed. The formula was then tested in a test set and compared against five current classic formulas.ResultsThe cohort included young patients (mean age = 14.38 ± 13.35 years). The Jiang Formula showed the lowest prediction error (PE; = 0.08 ± 1.01 diopters [D]), absolute error (AE; = 0.77 ± 0.65 D), median absolute error (MedAE; = 0.66 D), and root mean square error (RMSE; = 1.02 D) among six formulas (P < 0.001). Moreover, 68.00% of the eyes in the test set had AE within 1.0 D in the Jiang Formula.ConclusionsAI-integrated two-layer ensemble learning model demonstrates promising applications in IOLs power calculations for patients with CEL, not only providing higher predictive accuracy than current classic formulas but also accommodating extreme values and variations in surgical techniques.Translational relevanceThe Jiang Formula, an AI-integrated two-layer ensemble learning model, enhances IOLs power calculation accuracy in patients with CEL, ultimately improving surgical outcomes and supporting more effective, personalized treatment in this unique patient group.
| S-EPMC11801393 | biostudies-literature
Project description:PurposeTo derive an effective nomogram for predicting Marfan syndrome (MFS) in children with congenital ectopia lentis (CEL) using regularly collected data.MethodsDiagnostic standards (Ghent nosology) and genetic test were applied in all patients with CEL to determine the presence or absence of MFS. Three potential MFS predictors were tested and chosen to build a prediction model using logistic regression. The predictive performance of the nomogram was validated internally through time-dependent receiver operating characteristic curves, calibration curves, and decision curve analysis.ResultsEyes from 103 patients under 20 years old and with CEL were enrolled in this study. Z score of body mass index (odds ratio [OR] = 0.659; 95% confidence interval [CI], 0.453-0.958), corneal curvature radius (OR = 3.397; 95% CI, 1.829-6.307), and aortic root diameter (OR = 2.342; 95% CI, 1.403-3.911) were identified as predictors of MFS. The combination of the above predictors shows good predictive ability, as indicated by area under the curve of 0.889 (95% CI, 0.826-0.953). The calibration curves showed good agreement between the prediction of the nomogram and the actual observations. In addition, decision curve analysis showed that the nomogram was clinically useful and had better discriminatory power in identifying patients with MFS. For better individual prediction, an online MFS calculator was created.ConclusionsThe nomogram provides accurate and individualized prediction of MFS in children with CEL who cannot be identified with the Ghent criteria, enabling clinicians to personalize treatment plans and improve MFS outcomes.Translational relevanceThe prediction model may help clinicians identify MFS in its early stages, which could reduce the likelihood of developing severe symptoms and improve MFS outcomes.
| S-EPMC10959194 | biostudies-literature
Project description:AimTo 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).MethodsEight 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.ResultsMacula-on RRD was present in all individuals >18y, 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 logMAR (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 2y post-operative.ConclusionIn 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.
| S-EPMC7321954 | biostudies-literature