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.

Ocular, cardiovascular, and genetic characteristics and their associations in children with Marfan syndrome and related fibrillinopathies.

PURPOSE: Congenital ectopia lentis (CEL) and heart abnormalities are common clinical symptoms in patients with Marfan syndrome (MFS) and related fibrillinopathies, which is caused by mutations in fibrillin-1 (FBN1) gene. This study aims to explore the ocular and cardiovascular characteristics and their association with genotype in children with MFS and related fibrillinopathies. METHODS: Seventy-nine children diagnosed with CEL and with FBN1 mutations confirmed via whole-exome sequencing were included for genotypes and phenotypes analysis. The axial length (AL), corneal curvature, and refractive status were included for ocular phenotypes analysis. The cardiovascular examination was performed by echocardiography, and aortic root Z score was calculated to evaluate the severity of aortic dilatation. The heart disorders were classified as aortic root dilatation, valvular disorders, and others. Both the ocular and cardiac manifestations were collected for comprehensive analysis and compared among patients with different genotypes, including the mutation involving cysteine substitution or mutation in different regions. RESULTS: In CEL children with FBN1 mutations, 77.2% patients could be diagnosed as MFS. It was observed that children with mutations in exons 22-42 had significant higher aortic root Z score (P = 0.003) and higher incidence of cardiovascular disorders (P = 0.004). Additionally, children with cysteine substitution mutations had significant higher aortic root Z score (P = 0.011), and the aortic root Z score was positively associated with axial length (AL) in children under 6 years old (P = 0.035). Those with long AL (≥ 26 mm) had significant higher incidence of valve disorders (P = 0.023). In addition, nearly half the children with CEL (46.8%) were diagnosed with cardiovascular disease for the first time. CONCLUSIONS: CEL children with FBN1 mutations involving cysteine substitution or mutations in exons 22-42 or with long AL had higher risks of severe cardiovascular complications. Knowing the phenotype may help in anticipating severe cardiovascular disease in CEL patients.

A Modified Knotless Transscleral Intraocular Lens Fixation Technology for Congenital Ectopia Lentis.

INTRODUCTION: This 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). METHODS: This 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. RESULTS: For 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. CONCLUSION: Modified 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.

Longitudinal Changes of Axial Length and Associated Factors in Congenital Ectopia Lentis Patients.

Purpose: To investigate the longitudinal changes and associated factors of axial length (AL) in congenital ectopia lentis (CEL) patients. Methods: In this retrospective study, medical records of CEL patients were reviewed from January 2014 to December 2019 at the Zhongshan Ophthalmic (ZOC) in China. Patients were divided into the surgery group and the nonsurgery group. Data of refractive power, best-corrected visual acuity (BCVA), and intraocular pressure (IOP) as well as ocular biometrics including AL, corneal curvature, white-to-white (WTW), and central corneal thickness (CCT) were collected at baseline and each follow-up visit. Multiple linear regression was performed to assess the potential associated factors for axial length growth in congenital ectopia lentis patients. Results: Compared with the nonsurgery group, the change rate of AL among children aged 3 to 6 years old was slower in the surgery group (0.443 ± 0.340 mm/year vs. 0.278 ± 0.227 mm/year, P < 0.05). However, no statistically significant difference for the change rate of AL was detected between the surgery group and the nonsurgery group (P > 0.05) among patients aged 7 years or older. For the surgery group, the results of the linear regression model showed that a higher change rate of AL was associated with younger age (older age: ß = -0.009, 95% CI: -0.014 to -0.003, and P=0.002) and worse baseline BCVA (logMAR) (ß = 0.256, 95% CI: 0.072 to 0.439, and P=0.007). As for the nonsurgery group, younger baseline age (older age: ß = -0.027, 95% CI: -0.048 to -0.007, and P=0.01) and longer baseline AL (ß = 0.073, 95% CI: 0.023 to 0.122, and P=0.006) were associated with a higher change rate of AL. Conclusions: The AL change rate was clearly associated with age both in the surgery group and in the nonsurgery group. Intervention strategies such as surgery should be performed earlier for CEL that meets the surgical criteria. Worse baseline BCVA and longer baseline AL are associated factors that would affect the growth rate of AL in the surgery and nonsurgery group, respectively.

Evaluation of Intraocular Lens Tilt and Decentration in Congenital Ectopia Lentis by the Pentacam Scheimpflug System.

Purpose: The purpose of this study was to quantify the characteristics of the tilt and decentration of the IOL after trans-scleral suture fixation surgery in congenital ectopia lentis (CEL) patients. Methods: The clinical characteristics of 70 CEL patients at Zhongshan Ophthalmic Center in China were retrospectively analyzed. The tilt and decentration of intraocular lens (IOL) were measured by using a Pentacam and compared between different axial length (AL) subgroups. The correlation between IOL tilt, decentration, and ocular characteristics was investigated using Spearman's correlation analysis. Results: The postoperative IOL position of CEL patients was mainly located nasally inferiorly. The average tilt of the IOL in CEL patients was less than 7° (for temporal: 2.21 ± 1.53°, for nasal: -1.84 ± 2.04°, for superior: 2.22 ± 2.18°, and for inferior: -1.70 ± 1.62°), and the average decentration of the IOL in CEL patients was larger than 0.4 mm (for temporal: 0.49 ± 0.38 mm, for nasal: -0.69 ± 0.46 mm, for superior: 0.72 ± 0.58 mm, and for inferior: -0.68 ± 0.54 mm). The decentration of CEL patients in the AL ≥ 26 subgroup was greater than those with AL < 24 mm and AL 24 to 26 mm subgroups (for superior: 0.72 ± 0.28 mm vs. 0.46 ± 0.25 mm and 0.48 ± 0.22 mm, all P < 0.05; for inferior: -0.94 ± 0.56 mm vs. -0.44 ± 0.26 mm and -0.44 ± 0.46 mm, all P < 0.05). IOL decentration was positively correlated with AL (for superior: r = 0.44, P=0.019; for inferior: r = 0.54, P=0.006). IOL tilt was positively correlated with AL on the superior side (r = 0.38, P=0.041). Conclusions: The extent of IOL decentration after trans-scleral suture fixation was great in CEL patients, and the IOL decentration in CEL patients was significantly associated with AL.

Accuracy of intraocular lens power formulas for eyes with scleral-sutured intraocular lenses in congenital ectopia lentis.

PURPOSE: To compare the accuracy of intraocular lens (IOL) power calculation formulas in eyes with congenital ectopia lentis (CEL) that underwent scleral-fixated IOL implantation. SETTING: Zhongshan Ophthalmic Center, Guangzhou, China. DESIGN: Retrospective consecutive case-series study. METHODS: 158 eyes from 158 patients diagnosed from December 12, 2017, to November 16, 2020, with CEL and undergoing a lensectomy and scleral fixation of a Rayner 920H or 970C model IOL were retrospectively reviewed. The prediction errors (PEs) of the spherical equivalent of 8 formulas, Barrett Universal II (BUII), Emmetropia Verifying Optical (EVO), Haigis, Hoffer Q, Holladay 1, Kane, Hill-RBF 3.0, and SRK/T, were compared. RESULTS: For CEL patients with scleral-sutured IOL, all 8 formulas yielded myopic PEs without constant optimization. After such optimization, the performance of each formula ranked by median absolute error (MedAE) from the lowest to highest in diopter (D) was as follows: SRK/T (0.47), EVO (0.48), Kane (0.52), BUII (0.53), Hoffer Q (0.58), Holladay 1 (0.59), Haigis (0.61), and Hill-RBF 3.0 (0.62) formulas. The EVO and SRK/T formulas had the highest prediction accuracy concerning the percentage of cases within ±0.50 D and ±1.00 D range of PE in eyes that experienced scleral-sutured IOL surgery, respectively. CONCLUSIONS: All formulas before constant optimization produced myopic PEs. After optimization, the SRK/T and EVO formulas had the lowest MedAE and the highest percentage of PE in the range within ±0.50 D for CEL patients with scleral-sutured IOL implantations.

Endoscopy-guided in vivo evaluation of ciliary sulcus location in children with ectopia lentis.

OBJECTIVE: To assess a new method to measure the distance of the needle passage from the ciliary sulcus to the corneal limbus anterior border (CTC) in eyes with ectopia lentis directly in vivo via endoscopy and to further evaluate the correlations among the CTC, age, automated horizontal white-to-white distance (WTW), and ocular axial length (AL). METHODS: The WTW and AL were measured using an optical biometer. An intraocular endoscope was used during transscleral suture fixation of posterior chamber intraocular lenses to identify the true location of the ciliary sulcus. Linear regression analysis was used to assess the correlation between the CTC and other ocular biological parameters, including age, WTW, and AL. RESULTS: Thirty eyes of 30 children with ectopia lentis were evaluated. A statistically significant correlation was found between age and the CTC. The CTC could be predicted by the equation CTC = 0.1313 × Age + 0.9666. No statistically significant correlations were found between CTC and WTW, CTC and AL, WTW and AL, or WTW and age. CONCLUSION: Endoscopy is useful for precisely suturing intraocular lens haptics in the real ciliary sulcus. Age can be used as an equivalent parameter for prediction of the true ciliary sulcus location.

Mutation spectrum and genotype-phenotype correlations in Chinese congenital ectopia lentis patients.

PURPOSE: To identify the spectrum and frequency of mutations in congenital ectopia lentis (CEL) and to investigate the correlations between genotype and clinical phenotype in Chinese CEL patients. METHODS: Ninety-three participants with CEL were enrolled from March 2017 to April 2020. Ocular and systemic examinations were performed for each included patient. Genomic DNA from the included patients was analysed by whole-exome sequencing to detect mutations. Clinical manifestations were compared for different mutation subgroups. RESULTS: Gene mutations were detected in 79 patients. Sixty-five were FBN1-associated, and most were related to Marfan syndrome (MFS). The FBN1 mutations mainly consisted of missense mutations (49/65) and were concentrated in the 5' region. Probands with missense mutations tend to show high corneal astigmatism (χ2 = 3.98, P = 0.046) and severe lens dislocation (t = 2.90, P = 0.006) compared to premature termination codon (PTC) mutations. CONCLUSIONS: Most Chinese CEL patients were identified as having FBN1 mutations. Those with missense mutations commonly showed severe ocular phenotypes; therefore, reinforced follow-up and long-term observation are required. These correlations implicated the crucial role of missense and cysteine-involving mutations in ocular phenotypes, which might be explained by dominant-negative and nonsense-mediated mRNA decay (NMD).

Fluid-jet technique to polish the posterior capsule for phacoemulsification surgeries: efficacy and safety evaluation.

PURPOSE: To compare the efficacy and safety of a fluid-jet technique with capsular polish in reducing residual lens fibers (RLFs) in phacoemulsification surgery. SETTING: Single center. DESIGN: Prospective nonrandomized comparative study. METHODS: Patients receiving phacoemulsification were included. Consecutive eligible patients alternately underwent either removal of RLFs on the posterior capsule using capsular polish after irrigation and aspiration, and before intraocular lens (IOL) implantation, or RLF removal using a fluid-jet technique after IOL implantation. Posterior capsular images were used to quantify RLFs. Visual parameters were evaluated at 1 day, 1 week, and 1 year postoperatively. The proportion of capsule occupied by posterior capsule opacification (PCO) (area %) and incidence of protocol-driven laser posterior capsulotomy were recorded at 1 year in masked fashion. RESULTS: Seven hundred forty eyes were allocated to polishing (n = 370) or fluid-jet technique (n = 370). Polishing required 70.4 ± 17.5 seconds and fluid-jet 32.2 ± 9.9 seconds (P ≤ 0.001). Compared with the polishing group, capsular area occupied by RLFs in the fluid-jet group was significantly smaller (3.5% vs 0.5%, P = .031) at the end of surgery. One day postoperatively, the fluid-jet group had more desirable corrected distance visual acuity, objective scattering index, and Strehl ratio (all P < .05). PCO area percentage did not differ between groups (6.5% vs 4.5%, P = .252) 1 year postoperatively, but incidence of posterior capsulotomy was lower in the fluid-jet group (3.2% vs 0.8%, P = .019). CONCLUSIONS: Fluid-jet removed RLFs effectively and had lower incidence of postoperative capsulotomy than capsular polishing.

Incidence of and Risk Factors for Suspected Glaucoma and Glaucoma After Congenital and Infantile Cataract Surgery: A Longitudinal Study in China.

PRéCIS:: The overall incidence of postoperative suspected glaucoma and glaucoma after congenital cataract surgery is low; however, the identification of the associated risk factors helps to monitor susceptible individuals and to provide real-time surveillance and timely intervention. PURPOSE: Pediatric patients who have undergone surgery for congenital or infantile cataracts have a risk of developing suspected glaucoma and glaucoma, but the current evidence does not address our understanding of the incidence and associated risk factors of suspected glaucoma/glaucoma for application in clinical standard care. Therefore, this study investigated the incidence of and risk factors for suspected glaucoma/glaucoma in patients who have undergone surgery for congenital/infantile cataracts. METHODS: This study used a prospective cohort of 241 eyes from 241 patients who were 10 years of age or younger and who had undergone congenital/infantile cataract surgeries and were recruited from January 2011 to December 2016 at the Zhongshan Ophthalmic Center. Pediatric patients who underwent cataract surgery were classified into 2 groups according to intraocular lens (IOL) implantation. The patients' baseline characteristics and the incidence and risk factors for suspected glaucoma/glaucoma were assessed. RESULTS: The incidence of suspected glaucoma after cataract surgery was 10.70% [26 of 241 eyes; 95% confidence interval (CI), 6.88%-14.70%]. The rate of suspected glaucoma developing into glaucoma was 26.92% (7 of 26 eyes; 95% CI, 9.87%-43.97%), and the incidence of glaucoma was 2.90% (7 of 241 eyes; 95% CI, 0.79%-5.03%). Our study revealed that the incidence of suspected glaucoma/glaucoma in patients who did not receive IOL implantation was 13.91% (21 of 151 eyes; 95% CI, 8.39%-19.42%), and the incidence in patients who received IOL implantation was 5.56% (5 of 90 eyes; 95% CI, 8.23%-10.28%). The preoperative central corneal thickness (hazard ratio, 1.003; 95% CI, 1.001 to 1.004; P=0.004) and a family history of congenital cataracts (hazard ratio, 2.314; 95% CI, 1.004-5.331; P=0.049) were significant risk factors in patients without IOL implantation. Patient age at the time of cataract surgery was not a statistically significant risk factor for the development of suspected glaucoma/glaucoma in patients without IOL implantation. CONCLUSIONS: Identification of the incidence of and risk factors for suspected glaucoma/glaucoma may help clinicians monitor susceptible individuals and provide timely surveillance and interventions in a clinical setting. Age at the time of cataract surgery was not a risk factor for suspected glaucoma/glaucoma in patients without IOL implantation, and this may allow the timing window for pediatric cataract surgery to be expanded and support new insights into the optimal timing of surgery for standard care.

Expression of transforming growth factor ß and matrix metalloproteinases in the aqueous humor of patients with congenital ectopia lentis.

It is well known that transforming growth factor ß (TGFß), which is able to stimulate multiple intracellular signaling pathways, exerts an important role in Marfan syndrome, although the effects of TGFß on congenital ectopia lentis (CEL) have yet to be fully elucidated. In the present study, the expression levels of TGFß and matrix metalloproteinases (MMPs) were investigated in the aqueous humor of patients with ectopic lentis who differed in terms of the severity of the disease. A total of 17 CEL patients with 21 eyes (aged 12.76±9.37 years) and 12 congenital cataract (CC) patients with 17 eyes (aged 6.82±9.18 years) were randomized in the present study. The levels of active TGFß and MMPs in the aqueous humor were analyzed with Luminex xMAP® technology by using commercially available Bio­Plex Pro™ Human MMP and TGFß assays. The distance from the lens edge to the pupil edge and the white to white corneal diameter (i.e. the horizontal distance between the borders of the corneal limbus) were measured, and the ratio was calculated as the degree of lens dislocation. The association between TGFß and MMP levels and the degree of lens dislocation was analyzed using Spearman's correlation test. Compared with the patients with CC, the level of TGFß2 in the patients with CEL was increased significantly. Specifically, the level of TGFß2 in the CEL patients was 855.19 pg/ml (744.33, 1,009.24), whereas it was 557.08 (438.24, 692.71) pg/ml in the CC patients (P<0.001). In addition, it was noted that the levels of MMP­2 and ­10 in the aqueous humor of the patients with CEL were higher compared with those in the CC patients, although this increase did not reach the level of statistical significance. Notably, the levels of MMP­8 and ­9 in the aqueous humor of patients with CEL were significantly lower compared with those in the CC patients (P=0.014 and P=0.002, respectively). Furthermore, a marginal correlation was identified between the severity of ectopic lentis and the levels of TGFß2 in the aqueous humor (r2=0.379; P=0.003) of the patients with CEL. Taken together, these results demonstrated that a significant correlation existed between high levels of aqueous humor TGFß2 and the severity of ectopia lentis in patients with CEL. In addition, aqueous humor TGFß2 levels in the CEL patients were significantly higher compared with those in CC patients.

Diagnostic Efficacy and Therapeutic Decision-making Capacity of an Artificial Intelligence Platform for Childhood Cataracts in Eye Clinics: A Multicentre Randomized Controlled Trial.

BACKGROUND: CC-Cruiser is an artificial intelligence (AI) platform developed for diagnosing childhood cataracts and providing risk stratification and treatment recommendations. The high accuracy of CC-Cruiser was previously validated using specific datasets. The objective of this study was to compare the diagnostic efficacy and treatment decision-making capacity between CC-Cruiser and ophthalmologists in real-world clinical settings. METHODS: This multicentre randomized controlled trial was performed in five ophthalmic clinics in different areas across China. Pediatric patients (aged ≤ 14 years) without a definitive diagnosis of cataracts or history of previous eye surgery were randomized (1:1) to receive a diagnosis and treatment recommendation from either CC-Cruiser or senior consultants (with over 5 years of clinical experience in pediatric ophthalmology). The experts who provided a gold standard diagnosis, and the investigators who performed slit-lamp photography and data analysis were blinded to the group assignments. The primary outcome was the diagnostic performance for childhood cataracts with reference to cataract experts' standards. The secondary outcomes included the evaluation of disease severity and treatment determination, the time required for the diagnosis, and patient satisfaction, which was determined by the mean rating. This trial is registered with ClinicalTrials.gov (NCT03240848). FINDINGS: Between August 9, 2017 and May 25, 2018, 350 participants (700 eyes) were randomly assigned for diagnosis by CC-Cruiser (350 eyes) or senior consultants (350 eyes). The accuracies of cataract diagnosis and treatment determination were 87.4% and 70.8%, respectively, for CC-Cruiser, which were significantly lower than 99.1% and 96.7%, respectively, for senior consultants (p < 0.001, OR = 0.06 [95% CI 0.02 to 0.19]; and p < 0.001, OR = 0.08 [95% CI 0.03 to 0.25], respectively). The mean time for receiving a diagnosis from CC-Cruiser was 2.79 min, which was significantly less than 8.53 min for senior consultants (p < 0.001, mean difference 5.74 [95% CI 5.43 to 6.05]). The patients were satisfied with the overall medical service quality provided by CC-Cruiser, typically with its time-saving feature in cataract diagnosis. INTERPRETATION: CC-Cruiser exhibited less accurate performance comparing to senior consultants in diagnosing childhood cataracts and making treatment decisions. However, the medical service provided by CC-Cruiser was less time-consuming and achieved a high level of patient satisfaction. CC-Cruiser has the capacity to assist human doctors in clinical practice in its current state. FUNDING: National Key R&D Program of China (2018YFC0116500) and the Key Research Plan for the National Natural Science Foundation of China in Cultivation Project (91846109).

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.

Analysis of Corneal Astigmatism before Surgery in Chinese Congenital Ectopia Lentis Patients.

PURPOSE: This study aims to describe the characteristics of corneal astigmatism before surgery in congenital ectopia lentis (CEL) patients. METHODS: This retrospective study reviewed 306 CEL patients from 1st January 2006 to 31st December 2015. One eye was randomly selected from each patient when the patient had bilateral EL. The influence of sex, laterality, and Marfan syndrome on corneal astigmatism in different age subgroups was evaluated and compared. The correlation between age and corneal astigmatism was evaluated. RESULTS: Two hundred fifty-two eyes were included in this study. The mean corneal astigmatism of CEL patients was 2.00 ± 1.28 D. There was a statistical difference in corneal astigmatism between CEL eyes with and without Marfan syndrome. However, no statistical difference was found between male and female patients, or between the EL-affected eye and the unaffected eye in monocular EL patients. There was a positive correlation between age and corneal astigmatism in CEL eyes. CONCLUSIONS: This study suggests that CEL patients' corneal astigmatism is higher in patients with Marfan syndrome, and corneal astigmatism of the CEL eye increases with age. Our results are useful for surgeons to make appropriate incision and intraocular lens (IOL) choices for patients, as well as a useful reference for designs of new IOLs.

Timing and approaches in congenital cataract surgery: a four-year, two-layer randomized controlled trial.

AIM: To compare visual prognoses and postoperative adverse events of congenital cataract surgery performed at different times and using different surgical approaches. METHODS: In this prospective, randomized controlled trial, we recruited congenital cataract patients aged 3mo or younger before cataract surgery. Sixty-one eligible patients were randomly assigned to two groups according to surgical timing: a 3-month-old group and a 6-month-old group. Each eye underwent one of three randomly assigned surgical procedures, as follows: surgery A, lens aspiration (I/A); surgery B, lens aspiration with posterior continuous curvilinear capsulorhexis (I/A+PCCC); and surgery C, lens aspiration with posterior continuous curvilinear capsulorhexis and anterior vitrectomy (I/A+PCCC+A-Vit). The long-term best-corrected visual acuity (BCVA) and the incidence of complications in the different groups were compared and analyzed. RESULTS: A total of 57 participants (114 eyes) with a mean follow-up period of 48.7mo were included in the final analysis. The overall logMAR BCVA in the 6-month-old group was better than that in the 3-month-old group (0.81±0.28 vs 0.96±0.30; P=0.02). The overall logMAR BCVA scores in the surgery B group were lower than the scores in the A and C groups (A: 0.80±0.29, B: 1.02±0.28, and C: 0.84±0.28; P=0.007). A multivariate linear regression revealed no significant relationships between the incidence of complications and long-term BCVA. CONCLUSION: It might be safer and more beneficial for bilateral total congenital cataract patients to undergo surgery at 6mo of age than 3mo. Moreover, with rigorous follow-up and timely intervention, the postoperative complications in these patients are treatable and do not compromise visual outcomes.

Distribution of axial length in Chinese congenital ectopia lentis patients: a retrospective study.

BACKGROUND: Congenital ectopia lentis (CEL) usually leads to refractive error and may influence the axial length development. But few investigations have reported patient demographics and the distribution of axial length (AL) before surgery in Chinese pediatric patients with CEL. To describe the distribution of AL before surgery in CEL patients and its relationship with patients' demographics, such as age, Marfan syndrome, sex, and laterality. METHODS: This retrospective study reviewed 306 CEL patients from January 1, 2006 to December 31, 2015. One eye was randomly selected from each patient if both eyes were EL. The influences of Marfan syndrome, sex, and laterality to AL in different age subgroups were evaluated and compared. The differences of the AL between groups were assessed using the student t test or paired t-test. P-values less than 0.05 were considered statistically significant. RESULTS: Two hundred forty-seven eyes were enrolled. 58.3% of all the patients had binoculus EL, 70% of all the patients were male and 36% of all the patients were diagnosed with Marfan syndrome. The mean AL of EL patients was 25.1 ± 2.5 mm. There was no statistical difference in the AL between patients with and without Marfan syndrome, and in the AL between male and female patients. There was statistical difference in AL between the EL-affected eye and the unaffected eye in monocular EL patients younger than 12 years old. CONCLUSIONS: This study suggests that AL can be influenced by CEL, but the influence of CEL may be reduced after the age of 12 years old, which will likely provide a useful reference when considering the most appropriate time of surgery.

Dynamic response to initial stage blindness in visual system development.

Sensitive periods and experience-dependent plasticity have become core issues in visual system development. Converging evidence indicates that visual experience is an indispensable factor in establishing mature visual system circuitry during sensitive periods and the visual system exhibits substantial plasticity while facing deprivation. The mechanisms that underlie the environmental regulation of visual system development and plasticity are of great interest but need further exploration. Here, we investigated a unique sample of human infants who experienced initial stage blindness (beginning at birth and lasting for 2-8 months) before the removal of bilateral cataracts. Retinal thickness (RT), axial length (AL), refractive status, visual grating acuity and genetic integrity were recorded during the preoperative period or at surgery and then during follow-up. The results showed that the development of the retina is malleable and associated with external environmental influences. Our work supported that the retina might play critical roles in the development of the experience-dependent visual system and its malleability might partly contribute to the sensitive period plasticity.

Interocular anatomical and visual functional differences in pediatric patients with unilateral cataracts.

BACKGROUND: Congenital cataracts are often complicated by anterior segment dysgenesis. This study aims to compare bilateral anterior segment parameters, macular thickness, and best-corrected visual acuity (BCVA) in pediatric cataract patients at 3 months after unilateral cataract extraction with intraocular lens implantation. METHODS: Fifty-three pediatric patients with uncomplicated unilateral total cataracts were included. At 3 months post-surgery, bilateral corneal thickness at the thinnest location (CTTL), anterior chamber depth (ACD), and anterior chamber volume (ACV) were measured using Pentacam. Central macular thickness (CMT) was evaluated using spectral-domain optical coherence tomography. BCVA was measured by experienced optometrists concurrently. Descriptive statistics and bivariate corrections were performed to analyze the interocular differences in bilateral anatomic parameters and their relationships with BCVA. RESULTS: For all 53 included patients (mean age 5.2 ± 2.3 years), the median BCVA was 10/40 in the operated eyes and 40/40 in the contralateral eyes, which indicates a significant interocular difference. BCVA values in the contralateral eyes were significantly correlated with patient age at surgery, but this result differed for BCVA in the operated eyes. The Pentacam analysis revealed no significant interocular differences in bilateral CTTL and ACV, but significant differences were found for ACD. CONCLUSIONS: At 3 months after surgery, unilateral pediatric cataract patients exhibited no significant interocular differences in identified anatomical parameters (except for ACD), and these parameters were not significantly correlated with BCVA in bilateral eyes. Therefore, amblyopia, but not anatomical factors, might be the main cause of interocular visual functional differences in our study population. TRIAL REGISTRATION: ClinicalTrial.gov, NCT02765230 , 05/05/2016, retrospectively registered.

Visual Outcome and Related Factors in Bilateral Total Congenital Cataract Patients: A Prospective Cohort Study.

This study is to evaluate the visual outcome and identify its crucial related factors in children undergoing cataract surgery for bilateral total congenital cataract (CC). This prospective study included consecutive bilateral total cataract patients undergoing primary surgery at Zhongshan Ophthalmic Center (ZOC), Guangzhou, China from Jan 2010 to May 2014. Visual outcome was estimated by best-corrected visual acuity (BCVA) at last follow-up. Potential related factors, including gender, age at last follow-up, age at primary surgery, surgical procedure, postoperative complications (PCs), frequency of follow-up and changes in spectacles were evaluated. Eighty-eight children (176 eyes) were included in the cohort. The mean post-operative BCVA (logMAR) was 1.07 ± 0.53 at the mean follow-up duration 31.07 ± 19.36 months. Multivariable generalized estimating equations (GEEs) showed BCVA was significantly associated with PCs, age at last follow-up and age at primary surgery. Partial correlation analysis indicated age at primary surgery was positively correlated with BCVA controlling for the other factors, both for the whole age range (R = 0.415, P < 0.001) and age >6 months (R = 0.867, P < 0.001). Better visual acuity was related to early primary surgery and low PC occurrence in children with bilateral total CC. Timely surgical intervention and strict control of PCs would be potential steps to achieving better visual outcome.

[Clinical study of pars plana vitrectomy with silicone oil endotamponade in posttraumatic endophthalmitis on eyes without retinal detachment].

PURPOSE: To assess the clinical effect of pars plana vitrectomy (PPV) with intravitreous antibiotic drugs or silicone oil endotamponade in post-traumatic endophthalmitis on eyes without detachment of the retina. METHODS: Thirty cases of eyes with penetrating injury associated with endophthalmitis without retina detachment had been treated with PPV plus intravitreous antibiotic drugs or silicone oil tamponade. All the patients in the study received intravenous, subconjunctival and topical medications antibiotics. RESULTS: All the patients were followed up for 3 to 12 months. Postoperative intraocular infection of 30 eyes (30 patients) were controlled. The best-corrected visual acuity was significantly improved in both groups postoperatively (P < 0.05). The rate of postoperative retinal detachment in the PPV combined with intravitreal antibiotics group was 21.1% (4/19) vs. 9% (1/11)in the PPV combined with silicone oil There was no significant difference in postoperative endotamponade group. There was no significant difference in postoperative complications between two groups. CONCLUSIONS: PPV combined with silicone oil tamponade may have beneficial effect on the surgical treatment of traumatic bacterial endophthalmitis associated without retinal detachment, but the surgery may be used in some cases which have bad retinal condition or uncontrollable inflammation.