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Purpose: To examine the long‐term risk of glaucoma after cataract surgery in childhood. Methods: This study took place from January 2022 until December 2022 and included patients from a large family with hereditary childhood cataract who had cataract surgery before 18 years of age. Patients underwent an ophthalmologic examination to determine the presence of glaucoma or ocular hypertension (OHT). Patients who did not want to participate in the examination could contribute with a medical journal from their treating ophthalmologist. The risk of long‐term glaucoma was determined using survival analysis, and risk factors were assessed using a Cox proportional hazards regression model. Results: We included 68 patients (133 eyes) with a median age at cataract surgery of 7 years (IQR: 5–10). The median follow‐up time after cataract surgery to glaucoma/OHT or the latest ophthalmologic examination was 35 years (IQR: 15–48). Twelve patients (18 eyes) had glaucoma, and five patients (eight eyes) had OHT, resulting in 15 patients with glaucoma/OHT. The long‐term risk of glaucoma/OHT diagnosed in adulthood was 47.7% (CI: 21.8–70.9) at the age of 70 years of patients who were free of glaucoma before their 18th year. We could not confirm or dismiss an association between glaucoma/OHT and sex, age at surgery, number of ocular interventions before 18 years of age or glaucoma after cataract surgery in a first‐degree relative. Conclusion: Cataract surgery in childhood is associated with a high risk of late‐onset glaucoma. Regular lifelong follow‐up is important to ensure early diagnosis and prevent extensive vision loss. [ABSTRACT FROM AUTHOR]

Introduction: We previously reported a case of a patient with bilateral congenital cataract identified in the Ogasawara village, Bonin Islands, Japan, on a visit by an ophthalmologist and describe its course over 17 years from initial surgery. Here, we report on a secondary intraocular lens (IOL) implantation that was subsequently performed at 22 years and 5 months of age. Methods: After cataract surgery at 7 months, the aphakic patient began amblyopia treatment using extended-wear soft contact lenses (SCLs). After 10 years of age, SCLs were chosen to achieve crossed monovision considering the cosmetic appearance when facing other people. At 22 years of age, a secondary IOL implantation was performed. Secondary implant in the patient considered appearance and postoperative vision without glasses, we selected the crossed monovision method using a monofocal IOL. The postoperative targeted refraction for the right (non-dominant eye) and left eyes (dominant eye) planed − 0.33D and − 2.25D, respectively. Results: At 3 months after surgery, the corrected distance visual acuity (CDVA) with IOLs for the right and left eyes was 20/16 and 20/60, respectively, and the binocular visual acuity was 20/16 for distant vision and 20/25 for near vision. The CDVA for the right eye was: 20/13 × IOL = sph−0.25D and that for the left eye was: 20/13 × IOL = sph −1.75D D/cyl −0.50D/Ax170°. Although the exotropia was complicated by dissociated horizontal deviation and dissociated vertical deviation, there were no significant changes in ocular position before and after surgery. The patient was satisfied with achieving independence from spectacles and SCLs. Conclusion: The use of the crossed monovision method with monofocal IOLs in this patient and defective binocular function created a visual environment with no inconvenience in everyday life after secondary IOL implantation. In terms of secondary implant after amblyopia treatment, the IOL type or postoperative targeted refraction must be chosen to maintain or improve the visual environment obtained with the amblyopia treatment. [ABSTRACT FROM AUTHOR]

Purpose Methods Results Conclusion Treatment with glucocorticoids following paediatric cataract surgery is crucial to prevent inflammation, but may lead to secondary glaucoma, and hypothalamic–pituitary–adrenal axis suppression. We wish to compare glaucoma outcomes following high‐dose and low‐dose glucocorticoid treatment after paediatric cataract surgery.This cohort study included Danish children undergoing cataract surgery before 10 years of age, receiving either a low‐dose or high‐dose postoperative glucocorticoid treatment. Case identification and collection of a standardized dataset were retrospective, from 1 January 2010 to 31 December 2016, and prospective thereafter, until 31 December 2021. High‐dose treatment included 0.5–1.0 mg subconjunctival depot dexamethasone or methylprednisolone, followed by 6–8 drops of dexamethasone for 1 week, tapered by one drop weekly. Low‐dose treatment included 6 drops for 3 days, followed by 3 drops for 18 days. Sustained (>3 months) ocular hypertension or glaucoma was compared between the two groups.Overall, 267 children (388 eyes) were included in the study. Ninety‐five children (133 eyes) had received high‐dose treatment and had a median follow‐up time of 89 months (IQR: 57.2–107.4), while 173 children (255 eyes) had received the low‐dose treatment and had a median follow‐up time of 40.5 months (IQR: 22.9–60.4). Survival curves showed a lower risk of glaucoma in the low‐dose group for children with axial lengths ≥18 mm.Low‐dose glucocorticoid treatment was associated with a lower risk of glaucoma in children with axial lengths ≥18 mm. The same effect was not observed in children with shorter eyes. High‐dose glucocorticoid should be limited in children undergoing cataract surgery. [ABSTRACT FROM AUTHOR]

Background: Congenital cataract is a common cause of blindness. Genetic factors always play important role. Material and Methods: This study identified a novel missense variant (c.1412C>T (p.P471L)) in the EZR gene in a four-generation Chinese family with nuclear cataract by linkage analysis and whole-exome sequencing. A knockout study in zebrafish using transcription activator-like effector nucleases was carried out to gain insight into candidate gene function. Results: Conservative and functional prediction suggests that the P-to-L substitution may impair the function of the human ezrin protein. Histology showed developmental delays in the ezrin-mutated zebrafish, manifesting as multilayered lens epithelial cells. Immunohistochemistry revealed abnormal proliferation patterns in mutant fish. Conclusions: The study suggests that ezrin may be involved in the enucleation and differentiation of lens epithelial cells. [ABSTRACT FROM AUTHOR]

Background: Congenital cataracts, which can arise due to a combination of factors like environmental influences and genetic predisposition, significantly impact children's visual health globally. The occurrence rate of congenital cataracts varies from 0. 63 to 9.74 per 10,000 births. There are 7.4 instances per 10,000 children, with the highest occurrence seen in Asia. Symptoms of the disease include clouding of the lens and visual impairment. Timely identification of the condition plays a crucial role in the management and outlook of pediatric patients. Objective: This investigation aimed to discover causative mutations in four separate Chinese family lineages. Methods: The detailed clinical data and family history of four Chinese families with autosomal dominant congenital cataracts were carefully documented. Examination of the Whole Exome Sequencing was utilized to identify the genetic anomalies present in the familial cases. Subsequent validation of the identified mutations was carried out using PCR and Sanger sequencing. Following this, various computational predictive programs were utilized to evaluate how the mutations impact the structure and function of the protein. Results: The sequencing results reveal four potential disease-causing mutations: c.436G > A (p.V146M) of CRYBB2 Family 1, c.26G > T (p.R9I) of GJA3 in family 2, c.227G > A (p.R76H) of GJA8 in family 3, c.-168G > T of FTL in family 4. Among them, the causative mutation in Family GJA3 is novel, and Family FTL is a rare cataract syndrome. These familial mutations showed complete co-segregation with the affected individuals, with no presence in unaffected family members or the 100 controls. Several bioinformatic prediction tools also support the likely pathogenicity of these mutations. Conclusion: Our findings expand the mutational and phenotypic spectrum of genes associated with congenital cataracts and provide clues to the pathogenesis of congenital cataracts. These data also demonstrate the importance of NGS technology for the molecular diagnosis of congenital cataract patients. [ABSTRACT FROM AUTHOR]

Copyright of MN Opthalmology / MN Oftalmoloji is the property of Medical Network and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

AIM: To evaluate the diopter errors and safety of secondary intraocular lens(IOL)implantation in the ciliary sulcus using a modified incision procedure in children with bilateral aphakia.METHODS: The data of 12 children(24 eyes)with bilateral aphakia who had undergone secondary foldable IOL implantation in the ciliary sulcus using a modified incision procedure from 2020 to 2021 were retrospectively reviewed. Incision-related complications were analyzed intraoperatively and at 1 d and 1 mo postoperatively. Best-corrected visual acuity(BCVA), axial length(AL), white-to-white(WTW), and refractive outcomes in terms of preoperative reserved diopters(PRD)and actual equivalent spherical diopters(AESD). The patients were divided into two groups by age(≤7 and >7 a), AL(11.5mm), and the diopter errors between their AESD and PRD predicted based on the measurement results were compared.RESULTS: The patients comprised 9(75%)boys and 3(25%)girls. Two(17%)patients had anterior subcapsular cataracts, 4(33%)had posterior polar cataracts, and 6(50%)had nuclear cataracts. The mean age at cataract extraction was 6.4±1.61(3.4-8.9)mo. The mean interval between cataract extraction and secondary IOL implantation was 6.8±1.82(4.4-11.5)a. The mean preoperative BCVA was 0.49±0.33(1.0-0.1)LogMAR. The mean postoperative BCVA was 0.38±0.32(1.0-0.0)LogMAR. The mean AL was 23.56±1.96(19.00-26.38)mm, and the mean WTW was 11.5±0.92(9.3-13.9)mm. The mean PRD was 1.57±0.60(0.73-2.77)D, the mean AESD was 0.57±0.55(-0.50 to 1.75)D, and the mean difference between the AESD and PRD was -0.99±0.52(-2.22 to 0.32)D. The differences in the AESD and PRD between the groups according to age, AL and WTW were not statistically significant(P=0.59, 0.56, and 0.53, respectively).CONCLUSION: IOL implantation in the ciliary sulcus after a modified incision is safe and feasible for children with aphakia. It is necessary to subtract approximately 1 D of IOL power from the formula-selected power when implanting an IOL in the ciliary sulcus. Age, AL, and WTW do not significantly affect the difference.

The current genetic diagnostic workup of congenital cataract (CC) is mainly based on NGS panels, whereas exome sequencing (ES) has occasionally been employed. In this multicentre study, we investigated by ES the detection yield, mutational spectrum and genotype–phenotype correlations in a CC cohort recruited between 2020 and mid‐2022. The cohort consisted of 67 affected individuals from 51 unrelated families and included both non‐syndromic (75%) and syndromic (25%) phenotypes, with extra‐CC ocular/visual features present in both groups (48% and 76%, respectively). The functional effect of variants was predicted by 3D modelling and hydropathy properties changes. Variant clustering was used for the in‐depth assessment of genotype–phenotype correlations. A diagnostic (pathogenic or likely pathogenic) variant was identified in 19 out of 51 probands/families (~37%). In a further 14 probands/families a candidate variant was identified: in 12 families a VUS was detected, of which 9 were considered plausibly pathogenic (i.e., 4 or 5 points according to ACMG criteria), while in 2 probands ES identified a single variant in an autosomal recessive gene associated with CC. Eighteen probands/families, manifesting primarily non‐syndromic CC (15/18, 83%), remained unsolved. The identified variants (8 P, 12 LP, 10 VUS‐PP, and 5 VUS), half of which were unreported in the literature, affected five functional categories of genes involved in transcription/splicing, lens formation/homeostasis (i.e., crystallin genes), membrane signalling, cell–cell interaction, and immune response. A phenotype‐specific variant clustering was observed in four genes (KIF1A, MAF, PAX6, SPTAN1), whereas variable expressivity and potential phenotypic expansion in two (BCOR, NHS) and five genes (CWC27, KIF1A, IFIH1, PAX6, SPTAN1), respectively. Finally, ES allowed to detect variants in six genes not commonly included in commercial CC panels. These findings broaden the genotype–phenotype correlations in one of the largest CC cohorts tested by ES, providing novel insights into the underlying pathogenetic mechanisms and emphasising the power of ES as first‐tier test. [ABSTRACT FROM AUTHOR]

Abstract Objective To develop a comprehensive compliance assessment scale for postoperative visual function rehabilitation in children with congenital cataracts and to assess its reliability and validity. Method Drawing on the Interactive Model of Health Behavior, we conducted a literature review and semi-structured interviews to create a pool of 36 items. The items underwent rigorous evaluation through the Delphi method, face validity checks, and item analysis, leading to a reduction to 18 items. To assess the scale's reliability and validity, we collected data from 225 parents of children with congenital cataracts. We employed SPSS version 25.0 for data analysis and evaluated construct validity using exploratory factor analysis, content validity, internal consistency reliability, and test–retest reliability. Results The compliance scale for postoperative visual function rehabilitation in children with congenital cataracts comprises 5 dimensions and 18 items. Exploratory factor analysis extracted 5 common factors, with a cumulative variance contribution rate of 68.178%. Item-level content validity index ranged from 0.730 to 1.000, and the content validity index of the scale was 0.963. The total Cronbach's alpha coefficient, split-half reliability, and test–retest reliability of the scale were 0.855, 0.778, and 0.859, respectively. Conclusions The compliance assessment scale for postoperative visual function rehabilitation in children with congenital cataracts demonstrates acceptable reliability and validity. It serves as a valuable reference for developing standardized nursing programs for these children in clinical practice.

Mawaddah Sabr,1,&ast; Valmore A Semidey,2 Marcos J Rubio-Caso,2 Renad Aljuhani Jnr,3 Gorka Sesma1,&ast; 1Pediatric Ophthalmology and Strabismus Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 2Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 3Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia&ast;These authors contributed equally to this workCorrespondence: Gorka Sesma, Pediatric Ophthalmology and Strabismus Division, King Khaled Eye Specialist Hospital, Al Urubah Branche Road, West Building 2nd Floor, Riyadh, 11462, Saudi Arabia, Tel +966114849700, Fax +966114821908, Email gsesma@kkesh.med.saBackground: Retinal detachment is a major postsurgical threat in pediatric cataract surgery; however, the effect of axial length remains unclear. This study aimed to assess the relationship between axial length and detachment risk in vulnerable patients.Methods: This retrospective cohort study analyzed 132 eyes of 84 pediatric cataract surgery patients aged < 20 years old. Axial length was measured preoperatively, and the incidence of retinal detachment was recorded over a median follow-up of 4 years. Logistic regression analysis was used to examine the axial length-detachment relationship.Results: Twenty eyes had postoperative retinal detachments. The median axial length was longer in the detachment group (23.6 mm) than in the non-detachment group (21.6 mm). Eyes with axial length ≤ 23.4 mm had 0.55-fold decreased odds of detachment compared to longer eyes. Preexisting myopia and glaucoma confer heightened risk. Approximately half of the patients retained some detachment risk eight years postoperatively.Conclusion: Shorter eyes (axial length ≤ 23.4 mm) appear to be protected against pediatric retinal detachment after cataract surgery, whereas myopia, glaucoma, and axial elongation > 23.4 mm elevate the postoperative risk. Understanding these anatomical risk profiles requires surgical planning and follow-up care of children undergoing lensectomy.Plain language Summary: This study investigated the protective role of a shorter axial length in preventing retinal detachment after pediatric cataract surgery. This highlights the correlation between smaller eye sizes and reduced detachment risk, emphasizing the need for careful consideration of anatomical factors in surgical planning and patient monitoring, particularly for patients with preexisting myopia or postoperative glaucoma. Keywords: pediatric cataract surgery, axial length, retinal detachment, myopia, congenital glaucoma, congenital cataract

AIM: To identify genetic defects in a Chinese family with congenital posterior polar cataracts and assess the pathogenicity. METHODS: A four-generation Chinese family affected with autosomal dominant congenital cataract was recruited. Nineteen individuals took part in this study including 5 affected and 14 unaffected individuals. Sanger sequencing targeted hot-spot regions of 27 congenital cataract-causing genes for variant discovery. The pathogenicity of the variant was evaluated by the guidelines of American College of Medical Genetics and InterVar software. Confocal microscopy was applied to detect the subcellular localization of fluorescence-labeled ephrin type-A receptor 2 (EPHA2). Co-immunoprecipitation assay was implemented to estimate the interaction between EphA2 and other lens membrane proteins. The mRNA and protein expression were analyzed by reverse transcription-polymerase chain reaction (qRT-PCR) and Western blotting assay, respectively. The cell migration was analyzed by wound healing assay. Zebrafish model was generated by ectopic expression of human EPHA2/p.R957P mutant to demonstrate whether the mutant could cause lens opacity in vivo. RESULTS: A novel missense and pathogenic variant c.2870G>C was identified in the sterile alpha motif (SAM) domain of EPHA2. Functional studies demonstrated the variant's impact: reduced EPHA2 protein expression, altered subcellular localization, and disrupted interactions with other lens membrane proteins. This mutant notably enhanced human lens epithelial cell migration, and induced a central cloudy region and roughness in zebrafish lenses with ectopic expression of human EPHA2/p.R957P mutant under differential interference contrast (DIC) optics. CONCLUSION: Novel pathogenic c.2870G>C variant of EPHA2 in a Chinese congenital cataract family contributes to disease pathogenesis.

Introduction: Congenital cataract is the most common treatable cause of blindness during infancy and is responsible for 5%–20% of blindness in children worldwide. Materials and Methods: It was a retrospective descriptive study analyzing the causes of congenital cataracts in infants referred to the pediatric neurology department of a tertiary care institution, in South India. Results: Thirty-two infants with congenital cataracts underwent evaluation. Thirteen (40.6%) were

Congenital cataract is among the main causes of treatable vision loss in childhood. The first weeks and months of life are a critical time for the development of vision. Therefore, early cataract surgery and effective multifaceted treatment of the resulting aphakia in the early stages of life are of great value for the management of vision development. Among the treatment models, contact lenses (CL) have an important place in infancy and early childhood up to the age of 2 years. Although good visual gains were not considered very likely, especially in unilateral aphakia, important steps have been taken in the treatment of pediatric aphakia thanks to the surgical techniques developed over time and the increasing experience with optical correction systems, especially CLs. This review examines current developments in the types of CL used in pediatric aphakia, their application features, comparison with other optical systems, the features of amblyopia treatment in the presence of CL, and the results obtained with family compliance to CL wear and occlusion therapy in the light of existing studies.

Objective: To develop a comprehensive compliance assessment scale for postoperative visual function rehabilitation in children with congenital cataracts and to assess its reliability and validity. Method: Drawing on the Interactive Model of Health Behavior, we conducted a literature review and semi-structured interviews to create a pool of 36 items. The items underwent rigorous evaluation through the Delphi method, face validity checks, and item analysis, leading to a reduction to 18 items. To assess the scale's reliability and validity, we collected data from 225 parents of children with congenital cataracts. We employed SPSS version 25.0 for data analysis and evaluated construct validity using exploratory factor analysis, content validity, internal consistency reliability, and test–retest reliability. Results: The compliance scale for postoperative visual function rehabilitation in children with congenital cataracts comprises 5 dimensions and 18 items. Exploratory factor analysis extracted 5 common factors, with a cumulative variance contribution rate of 68.178%. Item-level content validity index ranged from 0.730 to 1.000, and the content validity index of the scale was 0.963. The total Cronbach's alpha coefficient, split-half reliability, and test–retest reliability of the scale were 0.855, 0.778, and 0.859, respectively. Conclusions: The compliance assessment scale for postoperative visual function rehabilitation in children with congenital cataracts demonstrates acceptable reliability and validity. It serves as a valuable reference for developing standardized nursing programs for these children in clinical practice. [ABSTRACT FROM AUTHOR]

Purpose Methods Results Conclusion To investigate visual development and long‐term complications after cataract surgery in childhood.This cross‐sectional study included patients from a family with Marner's hereditary cataracts who had cataract surgery before 18 years of age. The study was conducted from 1 January 2022 until 31 December 2022. The patients contributed to their medical files and participated in an updated ophthalmologic examination.We included 52 patients (101 eyes, 34 females). The median age at cataract surgery was 7 years (IQR: 5–10) and the age at examination was 40 years (IQR: 21–54). Primary and secondary intraocular lens implantation had been performed in 47.5% (25 patients, 48 eyes) and 16.8% (10 patients, 17 eyes). Visual acuity was ≤0.3 logMAR in 77% (78 eyes), and <0.5 logMAR in 8% (8 eyes). Glaucoma was present in 17% (9 patients, 12 eyes), ocular hypertension in 6% (3 patients, 4 eyes), and 10% (5 patients, 5 eyes) had prior retinal detachment. Mild visual field loss (2 < mean defect (MD) ≤ 6 dB) was found in 62% (63 eyes) and moderate to advanced visual field loss (MD > 6 dB) in 24% (24 eyes). Thirty‐five patients (67%) held a driver's licence, and three were not allowed to drive due to low visual function. All patients were employed.After cataract surgery in childhood, many patients achieve normal visual acuity, but mild visual field loss is common. Long‐term follow‐up is important due to the high risk of glaucoma. [ABSTRACT FROM AUTHOR]

Stickler syndrome (STL) is a collagenopathy caused by pathogenic variants in collagen-coding genes, mainly COL2A1 or COL11A1 associated with Stickler syndrome type 1 (STL1) or type 2 (STL2), respectively. Affected individuals manifest ocular, auditory, articular, and craniofacial findings in varying degrees. Previous literature and case reports describe high variability in clinical findings for patients with STL. With this case report, we broaden the clinical spectrum of the phenotype. Case report on two members of a family (mother and son) including clinical examination and genetic testing using targeted trio whole exome sequencing (trio-WES). A boy and his mother presented with microphthalmia, congenital cataract, ptosis, and moderate-to-severe sensorineural hearing loss. Trio-WES found a novel heterozygote missense variant, c.4526A>G; p(Gln1509Arg) in COL11A1 in both affected individuals. We report a previously undescribed phenotype associated with a COL11A1-variant in a mother and son, expanding the spectrum for phenotype-genotype correlation in STL2, presenting with microphthalmia, congenital cataract, and ptosis not normally associated with Stickler syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Glaucoma is one of the world's leading causes of irreversible vision loss. It is often asymptomatic until it reaches an advanced stage, which can have a significant impact on patients' daily lives. This paper describes the case of a 50-year-old female patient who presented with acute onset of ocular pain, photophobia, and loss of visual acuity in her right eye (RE). The patient's medical history includes congenital cataracts, surgical aphakia, nystagmus, strabismus, amblyopia, and secondary glaucoma. Ophthalmological examination showed BCVA RE-hand movement, left eye (LE)—0.08 with an intraocular pressure (IOP) of 30 mmHg in RE and 16 mmHg in LE. Biomicroscopic examination of RE showed corneal graft, epithelial and endothelial edema, endothelial precipitates, corneal neovascularization, aphakia, and Ahmed valve superotemporally. Despite maximal topical and systemic treatment, Ahmed valve, and trabeculectomy, secondary glaucoma in the right eye remained refractory. Reimplantation of an Ahmed valve was performed. This resulted in a favorable outcome with increased visual acuity and controlled intraocular pressure. The combination of aphakia, penetrating keratoplasty, and secondary glaucoma is a challenge for any surgeon. It is important that both the perioperative risks and the possible complications are carefully assessed in each patient, especially if associated pathology is present. [ABSTRACT FROM AUTHOR]

Congenital cataract is among the main causes of treatable vision loss in childhood. The first weeks and months of life are a critical time for the development of vision. Therefore, early cataract surgery and effective multifaceted treatment of the resulting aphakia in the early stages of life are of great value for the management of vision development. Among the treatment models, contact lenses (CL) have an important place in infancy and early childhood up to the age of 2 years. Although good visual gains were not considered very likely, especially in unilateral aphakia, important steps have been taken in the treatment of pediatric aphakia thanks to the surgical techniques developed over time and the increasing experience with optical correction systems, especially CLs. This review examines current developments in the types of CL used in pediatric aphakia, their application features, comparison with other optical systems, the features of amblyopia treatment in the presence of CL, and the results obtained with family compliance to CL wear and occlusion therapy in the light of existing studies. [ABSTRACT FROM AUTHOR]

Intraocular lens (IOL) implantation is currently the most effective clinical treatment for cataracts. Nevertheless, due to the growth of the eye axis in patients with congenital cataracts during the process of growth and development, the progressive incapacity of an IOL with a fixed focus does not meet the demands of practical usage, leading to the occurrence of ametropia. This work describes an innovative class of an IOL bulk material that offers good biosafety and light-controlled refractive index adjustment. Acrylate materials were synthesized for the preparation of IOLs by free radical polymerization of ethylene glycol phenyl ether methacrylate (EGPEMA), hydrophilic monomer 2-(2-ethoxyethoxy) ethyl acrylate (EA), and functional monomer hydroxymethyl coumarin methacrylate (CMA). Under 365/254 nm ultraviolet (UV) irradiation, the coumarin group could adjust the polymer material's refractive index through reversible photoinduced dimerization/depolymerization. Meanwhile, the potential for the IOL use is enabled by its satisfactory biosafety. Such a light-induced diopter adjustable IOL will be more appropriate for implantation during cataract surgery since it will not require the correction needed for ametropia and will offer more accurate and humane treatment. • A coumarin group containing-copolymer was synthesized and fabricated into intraocular lenses (IOL). • A functional IOL is diopter adjustable by light irradiation through triggering the [2 + 2] cycloaddition of the coumarin groups. • Such light-triggered diopter-adjustable copolymer IOLs have a great potential in clinical application. [Display omitted] [ABSTRACT FROM AUTHOR]

AIM: To identify disease-causative mutations in families with congenital cataract. METHODS: Two Chinese families with autosomal-dominant congenital cataract (ADCC) were recruited and underwent comprehensive eye examinations. Gene panel next-generation sequencing of common pathogenic genes of congenital cataract was performed in the proband of each family. Sanger sequencing was used to valid the candidate gene mutations and sequence the other family members for co-segregation analysis. The effect of sequence changes on protein structure and function was predicted through bioinformatics analysis. Major intrinsic protein (MIP)-wildtype and MIP-G29R plasmids were constructed and microinjected into zebrafish single-cell stage embryos. Zebrafish embryonic lens phenotypes were screened using confocal microscopy. RESULTS: A novel heterozygous mutation (c.85G>A; p.G29R) in the MIP gene was identified in the proband of one family. A known heterozygous mutation (c.97C>T; p.R33C; rs864309693) in MIP was found in the proband of another family. In-silico prediction indicated that the novel mutation might affect the MIP protein function. Zebrafish embryonic lens was uniformly transparent in both wild-type PCS2+MIP and mutant PCS2+MIP. CONCLUSION: Two missense mutations in the MIP gene in Chinese cataract families are identified, and one of which is novel. These findings expand the genetic spectrum of MIP mutations associated with cataracts. The functional studies suggest that the novel MIP mutation might not be a gain-of-function but a loss-of-function mutation.

AIM: To establish a classification for congenital cataracts that can facilitate individualized treatment and help identify individuals with a high likelihood of different visual outcomes. METHODS: Consecutive patients diagnosed with congenital cataracts and undergoing surgery between January 2005 and November 2021 were recruited. Data on visual outcomes and the phenotypic characteristics of ocular biometry and the anterior and posterior segments were extracted from the patients' medical records. A hierarchical cluster analysis was performed. The main outcome measure was the identification of distinct clusters of eyes with congenital cataracts. RESULTS: A total of 164 children (299 eyes) were divided into two clusters based on their ocular features. Cluster 1 (96 eyes) had a shorter axial length (mean±SD, 19.44±1.68 mm), a low prevalence of macular abnormalities (1.04%), and no retinal abnormalities or posterior cataracts. Cluster 2 (203 eyes) had a greater axial length (mean±SD, 20.42±2.10 mm) and a higher prevalence of macular abnormalities (8.37%), retinal abnormalities (98.52%), and posterior cataracts (4.93%). Compared with the eyes in Cluster 2 (57.14%), those in Cluster 1 (71.88%) had a 2.2 times higher chance of good best-corrected visual acuity [

Abstract Background To explore the changes of cytokines expression in aqueous humor (AH) of eyes of patients with congenital cataract (CC) who underwent delayed sequential bilateral cataract surgery (DSBCS). Methods 28 patients with CC underwent DSBCS. AH samples were collected from each eye before surgery. The contents of cytokines in AH were detected by Luminex xMAP Technology. Results There was no significant difference in the expression of IL-8, IP-10, MCP-1 and PDGFAA in the AH of the first and second eyes (P = 0.35, 0.39, 0.17, respectively). The level of IL-8 in the first-eye AH was negatively correlated with age (ρ=– 0.519, P = 0.008). IP-10 and MCP-1 in the second-eye AH were negatively correlated with age (ρ=– 0.483, P = 0.009; ρ=– 0.445, P = 0.018,respectively). Conclusion The first-eye surgery in patients with CC may not cause the change of cytokines in the contralateral eye. The expression of IL-8, IP-10 and MCP-1 in the AH was negatively correlated with the age of patients. Trial registration The study was registered at www.chictr.org.cn on March 22, 2022 and the clinical trial number is ChiCTR2200057927.

Objectives:To evaluate the predictive factors of complications and visual acuity outcomes in pediatric cataract patients.Materials and Methods:This retrospective, observational clinical study included 80 eyes of 50 patients treated for pediatric cataracts between 2010 and 2020. The eyes were divided into Group I (congenital cataracts, n=38) and Group II (developmental cataracts, n=42). Group II was also divided into Group IIA (aphakic, n=21) and Group IIB (pseudophakic, n=21). The effects of the age, laterality, cataract morphology, intraocular lens implantation, preoperative nystagmus/strabismus, and intraoperative anterior hyaloid rupture on complications and final best-corrected visual acuity (BCVA; logMAR) were evaluated.Results:The median (interquartile range) age and follow-up time were 28 (5-79) months and 60 (29-84) months, respectively. There was a significant difference in mean final BCVA between Group I (0.79±0.46) and Group II (0.57±0.51) (p=0.047); however, no difference was observed between Group IIA and Group IIB (p=0.541). Having congenital cataract (p=0.045), preoperative nystagmus/strabismus (p=0.042), total/mature cataract (p

Abstract Background Cranio-lenticulo-sutural dysplasia (CLSD) is a rare dysmorphic syndrome characterized by skeletal dysmorphism, late-closing fontanels, and cataracts. CLSD is caused by mutations in the SEC23A gene (OMIM# 607812) and can be inherited in either an autosomal dominant or autosomal recessive pattern. To date, only four mutations have been reported to cause CLSD. This study aims to identify the disease-causing variants in a large cohort of congenital cataract patients, to expand the genotypic and phenotypic spectrum of CLSD, and to confirm the association between SEC23A and autosomal recessive CLSD (ARCLSD). Methods We collected detailed medical records and performed comprehensive ocular examinations and whole-exome sequencing (WES) on 115 patients with congenital cataracts. After suspecting that a patient may have CLSD based on the sequencing results, we proceeded to conduct transmission electron microscopy (TEM) on the cultured skin fibroblasts. The clinical validity of the reported gene-disease relationships for the gene and the disease was evaluated using the ClinGen gene curation framework. Results Two novel compound heterozygous variants (c.710A > C p.Asp237Ala, c.1946T > C p.Leu649Pro) of the SEC23A gene, classified as variant of uncertain significance, were identified in the proband with skeletal, cardiac, ocular, and hearing defects. The observation of typical distended endoplasmic reticulum cisternae further supported the diagnosis of CLSD. Application of the ClinGen gene curation framework confirmed the association between SEC23A and ARCLSD. Conclusion This study expands the genotypic and phenotypic spectrum of CLSD, proposes TEM as a supplemental diagnostic method, and indicates that congenital cataracts are a typical sign of ARCLSD.

A fatal viral infection called rubella, often known as German or three-day measles, is spread by the Rubivirus genus of the Togaviridae family. The disorder known as Congenital Rubella Syndrome (CRS) has the potential to cause a wide range of multisystem problems. Every organ in the developing foetus can be damaged; the most prevalent involvements are cardiac, ophthalmic, and auditory abnormalities. This is a case report of an 11-year-old patient who was hospitalised at a tertiary care facility with severe signs and symptoms of CRS, which were identified at birth. Ocular abnormalities included congenital cataracts and salt and pepper retinopathy. The lens aspiration and Intraocular Lens (IOL) implantation were done. This case serves as an example of the significance of the rubella immunisation program.

Congenital cataract (CC), the most prevalent cause of childhood blindness and amblyopia, necessitates prompt and precise genetic diagnosis. The objective of this study is to identify the underlying genetic cause in a Swiss patient with isolated CC. Whole exome sequencing (WES) and copy number variation (CNV) analysis were conducted for variant identification in a patient born with a total binocular CC without a family history of CC. Sanger Sequencing was used to confirm the variant and segregation analysis was used to screen the non-affected parents. The first de novo missense mutation at c.391T>C was identified in exon 3 of CRYGC on chromosome 2 causing the substitution of a highly conserved Tryptophan to an Arginine located at p.Trp131Arg. Previous studies exhibit significant changes in the tertiary structure of the crystallin family in the following variant locus, making CRYGC prone to aggregation aggravated by photodamage resulting in cataract. The variant can be classified as pathogenic according to the American College of Medical Genetics and Genomics (ACMG) criteria (PP3 + PM1 + PM2 + PS2; scoring 10 points). The identification of this novel variant expands the existing knowledge on the range of variants found in the CRYGC gene and contributes to a better comprehension of cataract heterogeneity. [ABSTRACT FROM AUTHOR]

Purpose: The study evaluates the rate of postoperative formation of a pupillary membrane (PM) and posterior visual axis opacification (PVAO) in infants with and without primary IOL implantation during the first 4 months of infancy. Methods: Medical records for 144 eyes (101 infants) operated between 2005 and 2014 were evaluated. A posterior capsulectomy and anterior vitrectomy were performed. Primary IOL implantation was performed in 68 eyes, while 76 eyes were left aphakic. There were 16 bilateral cases in the pseudophakic group and 27 in the aphakic group. The follow-up period was 54.3 ± 21.05 months and 49.1 ± 18.60 months, respectively. Fisher's exact test was used for statistical analysis. The two-sample t-test with equal variance was used to compare surgery age, follow-up period and time intervals of complications. Results: The mean age of surgery was 2.1 ± 0.85 months in the pseudophakic and 2.2 ± 1.01 months in the aphakic group. PM was diagnosed in 40% pseudophakic and 7% aphakic eyes. A second surgery for PVAO was performed in 72% pseudophakic and 16% aphakic eyes. Both were significantly higher in the pseudophakic group. In the pseudophakic group, the number of PVAO was significantly higher in infants operated before 8 weeks of age compared to surgery age 9–16 weeks. The frequency of PM was not age-dependent. Conclusion: Although it remains feasible to implant an IOL during the primary surgery, even in very young infants, there should always be solid arguments for this decision since it puts the child at higher risk of repeated surgeries under general anaesthesia. [ABSTRACT FROM AUTHOR]

Beaded filament structural protein 1 (BFSP1) gene plays important role in the development of congenital cataract. We aimed to investigate and analyze the molecular mechanism of congenital cataract caused by D348N mutation of BFSP1 gene, and to provide evidence for the intervention of congenital cataract. BFSP1 and CP49 genes were cloned, wild type and mutant expression plasmids of BFSP1 were constructed and transfected into 293T cells. The BFSP1 wild type and mutant (D348N) gene sequence (NM_001195) were constructed into pEGFP-N1 vector by the restriction site NheI/KpnI. The effect of mutation on cell proliferation and apoptosis was analyzed. There was no significant change between the expression site of BFSP1 D348N mutation and the wild type. The expression of BFSP1 protein in wild group was higher than that in mutant group. CCK8 detection showed that the proliferation ability of 293T cells in mutant group was weaker than that in BFSP1 group. The mutation led to an increase in apoptosis. BFSP1 mutation significantly decreases the expression of BFSP1 protein, weakened the ability of cell proliferation and increased apoptosis. BFSP1 D348N mutation may be closely associated with congenital cataract and is of great significance to the investigations of the mechanism and intervention of congenital cataract. [ABSTRACT FROM AUTHOR]

Purpose: The present study aimed to identify the molecular etiology of non-syndromic congenital cataract (CC) using whole-exome sequencing (WES) analysis. Methods: In the present study, ophthalmologic results and pedigree analysis of the families of 12 patients with non-syndromic CC were evaluated. WES analysis was conducted after DNA was isolated from peripheral blood samples obtained from the patients. Results: Twelve non-syndromic probands (10 males and 2 females) with bilateral CC were included in the study. Patient age ranged between 1 and 11 months. WES analysis showed pathogenic/likely pathogenic variant in 7 (58%) of the 12 families and variant of unknown significance (VUS) in 5 (42%) of them. All the 13 different variants detected in 9 different CC-related genes were co-segregated with the disease. Autosomal dominant inheritance was found in 7 (58%) of the families and autosomal recessive inheritance was found in 5 (42%) of them. Conclusion: To the best of our knowledge, the present research is one of the limited numbers of studies in the Turkish population in which genetically heterogeneous non-syndromic CC was investigated using WES analysis. Novel variants that we identified in DNMBP, LSS, and WFS1 genes, which are rarely associated with the CC phenotype, have contributed to the mutation spectrum of this disease. Identifying the relevant molecular genetic etiology allows accurate genetic counseling to be provided to the families. [ABSTRACT FROM AUTHOR]

Background: To explore the changes of cytokines expression in aqueous humor (AH) of eyes of patients with congenital cataract (CC) who underwent delayed sequential bilateral cataract surgery (DSBCS). Methods: 28 patients with CC underwent DSBCS. AH samples were collected from each eye before surgery. The contents of cytokines in AH were detected by Luminex xMAP Technology. Results: There was no significant difference in the expression of IL-8, IP-10, MCP-1 and PDGFAA in the AH of the first and second eyes (P = 0.35, 0.39, 0.17, respectively). The level of IL-8 in the first-eye AH was negatively correlated with age (ρ=– 0.519, P = 0.008). IP-10 and MCP-1 in the second-eye AH were negatively correlated with age (ρ=– 0.483, P = 0.009; ρ=– 0.445, P = 0.018,respectively). Conclusion: The first-eye surgery in patients with CC may not cause the change of cytokines in the contralateral eye. The expression of IL-8, IP-10 and MCP-1 in the AH was negatively correlated with the age of patients. Trial registration: The study was registered at www.chictr.org.cn on March 22, 2022 and the clinical trial number is ChiCTR2200057927. [ABSTRACT FROM AUTHOR]

Pediatric ocular examinations are often a challenge in the outpatient setting due to limited cooperation of the child. Hence an evaluation under anesthesia (EUA) or sedation is important for a holistic ophthalmic examination. It can be combined with short procedures, such as suture removal and corneal scrappings, both for diagnosis and for the management of several ophthalmic disorders. It can also be performed before planning a surgical intervention to record the baseline characters and formulate or refine a surgical plan. Every EUA must be used as a chance to perform a complete ophthalmic examination rather than perform a single task such as recording the intraocular pressure. This article aims to provide a protocol that can be followed for a complete EUA. [ABSTRACT FROM AUTHOR]

Background: Cranio-lenticulo-sutural dysplasia (CLSD) is a rare dysmorphic syndrome characterized by skeletal dysmorphism, late-closing fontanels, and cataracts. CLSD is caused by mutations in the SEC23A gene (OMIM# 607812) and can be inherited in either an autosomal dominant or autosomal recessive pattern. To date, only four mutations have been reported to cause CLSD. This study aims to identify the disease-causing variants in a large cohort of congenital cataract patients, to expand the genotypic and phenotypic spectrum of CLSD, and to confirm the association between SEC23A and autosomal recessive CLSD (ARCLSD). Methods: We collected detailed medical records and performed comprehensive ocular examinations and whole-exome sequencing (WES) on 115 patients with congenital cataracts. After suspecting that a patient may have CLSD based on the sequencing results, we proceeded to conduct transmission electron microscopy (TEM) on the cultured skin fibroblasts. The clinical validity of the reported gene-disease relationships for the gene and the disease was evaluated using the ClinGen gene curation framework. Results: Two novel compound heterozygous variants (c.710A > C p.Asp237Ala, c.1946T > C p.Leu649Pro) of the SEC23A gene, classified as variant of uncertain significance, were identified in the proband with skeletal, cardiac, ocular, and hearing defects. The observation of typical distended endoplasmic reticulum cisternae further supported the diagnosis of CLSD. Application of the ClinGen gene curation framework confirmed the association between SEC23A and ARCLSD. Conclusion: This study expands the genotypic and phenotypic spectrum of CLSD, proposes TEM as a supplemental diagnostic method, and indicates that congenital cataracts are a typical sign of ARCLSD. [ABSTRACT FROM AUTHOR]

Objective: Our aim to examine the results of surgical treatment and follow-up of patients diagnosed with congenital cataract and who are under our clinical follow-up. Material and Method: In this study, a diagnosis of congenital cataract was made, anterior continuous curvilinear capsulorhexis, phacoaspiration,posterior continuous curvilinear capsulorhexis, anterior vitrectomy operation and/or intraocular lens implantation was performed in the ciliary sulcus, apakic follow-up was performed, and patients aged 0-34 years at the time of surgery. 84 eyes of 50 patients were evaluated Results: The mean follow-up period was 30.9±23months.After an average of 33 months of follow-up in 16 eyes, intraocular lens was applied to the ciliary sulcus in 57 eyes at an average of 45 months. During the follow-up period, secondary glaucoma was observed in 7eyes, intraocular lens dislocation in 9 eyes, pupillary irregularity and localized iris atrophy in 17 eyes,and anterior vitreous membrane development in 5 eyes. The preoperative best corrected visual acuity of the patients was 0.08±0.02 (P+P+ or hand motion-5m counting finger), and the postoperative best corrected visual acuity was 0.4±0.3, which was statistically significant(P<0.001).Apakic follow-up and secondary intraocular lens were applied to 16 patients, with an average visual acuity of 0.2. Conclusion: In the surgical treatment of congenital cataracts, anterior continuous curvilinear capsulorhexis, phacoaspiration, posterior continuous curvilinear capsulorhexis, anterior vitrectomyand/or intraocular lens implantation into the ciliary sulcus is an effective, reliable, and good visual technique in preventing posterior capsule occlusion and minimizing surgical complications. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the predictive factors of complications and visual acuity outcomes in pediatric cataract patients. Materials and Methods: This retrospective, observational clinical study included 80 eyes of 50 patients treated for pediatric cataracts between 2010 and 2020. The eyes were divided into Group I (congenital cataracts, n=38) and Group II (developmental cataracts, n=42). Group II was also divided into Group IIA (aphakic, n=21) and Group IIB (pseudophakic, n=21). The effects of the age, laterality, cataract morphology, intraocular lens implantation, preoperative nystagmus/strabismus, and intraoperative anterior hyaloid rupture on complications and final best-corrected visual acuity (BCVA; logMAR) were evaluated. Results: The median (interquartile range) age and follow-up time were 28 (5-79) months and 60 (29-84) months, respectively. There was a significant difference in mean final BCVA between Group I (0.79±0.46) and Group II (0.57±0.51) (p=0.047); however, no difference was observed between Group IIA and Group IIB (p=0.541). Having congenital cataract (p=0.045), preoperative nystagmus/strabismus (p=0.042), total/ mature cataract (p<0.001), and postoperative complications (p=0.07) were significantly associated with final BCVA. However, in multivariate analysis, only total/mature cataract (β: 0.52, p<0.001) and having any complication (β: 0.24, p=0.018) were associated with final BCVA. Congenital cataract and intraoperative anterior hyaloid rupture were the only significant risk factors of postoperative complications on univariate (p=0.027 and p=0.003, respectively) and binary logistic regression analysis (odds ratio [OR]: 2.95 [95% confidence interval: 1.07-8.15], p=0.036 and OR: 4.28 [95% confidence interval: 1.55-11.77], p=0.005, respectively). Conclusion: Total/mature cataract and the presence of any postoperative complication adversely affected the final BCVA. Having a congenital cataract and intraoperative anterior hyaloid membrane rupture increased the risk of complications. [ABSTRACT FROM AUTHOR]

BackgroundCongenital cataracts stand as the primary cause of childhood blindness globally, characterized by clouding of the eye’s lens at birth or shortly thereafter. Previous investigations have unveiled that a variant in the V-MAF avian musculoaponeurotic-fibrosarcoma oncogene homolog (MAF) gene can result in Ayme-Gripp syndrome and solitary cataract. Notably, MAF mutations have been infrequently reported in recent years.MethodsIn this investigation, we recruited a Chinese family with non-syndromic cataracts. Whole exome sequencing and Sanger sequencing were applied to scrutinize the genetic anomaly within the family.ResultsThrough whole exome sequencing and subsequent data filtration, a new mutation (NM_005360, c.901T>C/p.Y301H) in the MAF gene was detected. Sanger sequencing validated the presence of this mutation in another affected individual. The p.Y301H mutation, situated in an evolutionarily preserved locus, was not detected in our 200 local control cohorts and various public databases. Additionally, multiple bioinformatic programs predicted that the mutation was deleterious and disrupted the bindings between MAF and its targets.ConclusionHence, we have documented a new MAF mutation within a Chinese family exhibiting isolated congenital cataracts. Our study has the potential to broaden the spectrum of MAF mutations, offering insights into the mechanisms underlying cataract formation and facilitating genetic counseling and early diagnosis for congenital cataract patients.

ObjectivesTo investigate the characteristic cytokine profile of the aqueous humor in eyes with congenital cataract and pre-existing posterior capsule dysfunction (PCD).MethodsIn this cross-sectional study, the enrolled eyes with congenital cataract and PCD were included in the PCD group, while those with an intact posterior capsule were included in the control group. Demographic data and biometric parameters were recorded. The levels of 17 inflammatory factors in the aqueous humor collected from the enrolled eyes were detected using Luminex xMAP technology, and intergroup differences in the collected data were analyzed.ResultsThe PCD group comprised 41 eyes from 31 patients with congenital cataract and PCD, whereas the control group comprised 42 eyes from 27 patients with congenital cataract and an intact posterior capsule. Lens thickness was significantly thinner in the PCD group than in the control group. However, the levels of monocyte chemoattractant protein-1 (MCP-1), transforming growth factor-β2 (TGF-β2), and vascular endothelial growth factor (VEGF) were significantly higher in the PCD group than in the control group. Multivariate logistic regression confirmed that lens thickness and TGF-β2 level were independent risk factors for PCD.ConclusionA thinner lens thickness in eyes with congenital cataract and PCD could serve as a biometric feature of these eyes. The higher levels of MCP-1, TGF-β2, and VEGF in eyes with PCD indicated a change in their intraocular inflammatory microenvironment, which possibly led to cataract progression. Lens thickness and TGF-β2 level are independent risk factors for PCD.

Congenital cataract refers to the presence of any opacity in the lens at birth or during early childhood. Cataracts in children can manifest as unilateral or bilateral, congenital or acquired, with specific inheritance patterns or occurring sporadically. Additionally, the condition can be stable or progressive. Congenital cataracts can be associated with certain systemic diseases, or they may occur in the eye of an otherwise healthy child in isolation. Congenital cataract is one of the main causes of blindness in childhood. According to the report of the World Health Organization in 2001, congenital cataract is responsible for about 5 to 20 percent of cases of childhood blindness in the world based on different geographical regions. Untreated cataract in children leads to a tremendous social, economic and emotional burden for the child, family and society. Common complaints associated with congenital cataracts often include leukocoria (white pupil), reduced vision, a child's inability to track objects up close, light sensitivity leading to eye closure, squinting, the presence of either small eyes (microphthalmos) or large eyes (buphthalmos), strabismus (eye deviation), and abnormal eye movements such as nystagmus. Childhood cataract-related blindness can be cured with early detection and appropriate management. Managing pediatric cataracts is a challenge. Increased intraoperative complications compared to adults, tendency to increase postoperative inflammation, associated with complications such as aphakic glaucoma and persistent fetal vasculature, change in refractive status of the eye, and tendency to develop amblyopia, all complicate the achievement of good vision. Congenital cataract has a good prognosis if it is diagnosed early and surgery is performed before 6 weeks. Factors that may adversely affect the outcome, include the presence of unilateral cataract, presence of nystagmus, strabismus, or any ocular defect such as microphthalmos and PFV. Pediatric cataract surgery has evolved over the years, and with improved knowledge about myopic shift and axial growth, outcomes for these patients have become more predictable. Optimal results depend not only on effective surgery, but also on careful postoperative care and visual rehabilitation. Hence, it is the combined effort of parents, surgeons, anesthesiologists, pediatricians and optometrists that can make the difference.

Background: The most frequent surgical approach for cataracts is a micro-incision aspiration with intraocular lens (IOL) implantation. Multiple factors are involved in the long-standing effect of Pediatric cataract surgery. Objective: The purpose of this study was to assess the visual outcomes following secondary implantation of IOL in children who have undergone primary cataract surgery for congenital cataracts. Study type, settings & duration:This was a cross-sectional study performed at the Pediatric section of Al-Ibrahim Eye Hospital, Karachi from January 2018 to December 2019. Methodology:Fifty-seven files were retrieved for data collection. All children who had congenital cataracts and were aged 1-3 years and had undergone primary cataract surgery were included. Children with congenital cataract had undergone a primary surgery at six months to one year after which a secondary IOL implant had been done on them aged 15-36 months. Results:Visual acuity improved to 6/12 in 40.4% patients, 6/9.5 in 33.3% patients, and 6/15 in 10.5% patients. The final visual outcome through log MAR chart revealed that most of the children improved visual acuity. Of the total 19(33.3%) had a 0.2 score that is equivalent to 6/9.5 on the Snellen chart. Likewise, 23(40.4%) had 0.3 score that is equivalent to 6/12 on the Snellen chart, 6(10.5%) had 0.4 on the log MAR chart. Conclusion:The bilateral congenital cataract was more prevalent. Overall an improvement in the visual outcome was seen after secondary Intraocular lens implantation in patients with bilateral congenital cataracts.