High Refractive Errors Research Articles

The Effect of High-Dose Erythropoietin Perinatally on Retinal Function in School-Aged Children Born Extremely or Very Preterm

To investigate the long-term effects of high-dose recombinant human erythropoietin (rhEPO) administered during the perinatal period on retinal and visual function in children born extremely or very preterm. Randomized, double-blind clinical trial follow-up plus cohort study. Setting: Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland. Extremely or very preterm-born children aged 7 to 15 years, previously randomized to receive either high-dose rhEPO or placebo in the perinatal period. participation in an ongoing neuropediatric study (EpoKids), written informed consent. previous ocular trauma or surgery; retinal or developmental disease unrelated to prematurity. Healthy control (HC) children of comparable age were recruited. term birth, informed consent. any ocular/visual abnormality, high refractive error. Intervention status (rhEPO/placebo) was unknown to examiners and subjects at examination, with examiners unblinded only after completion of all analyses. The electroretinogram (ERG) was performed with the RETeval device (LKC Technologies, Inc). Ophthalmological and orthoptic examinations excluded comorbidity in the prematurely born cohort and ocular diseases in the HC group. Scotopic and photopic ERG response amplitudes and peak times (6 amplitudes; 6 peak times). Secondary outcomes were habitual visual acuity and color discrimination performance (for descriptive summary only). No differences in ERG parameters between EPO (n = 52; 104 eyes) and placebo (n = 35; 70 eyes) subgroups were observed (all corrected P > .05). Two cone system-mediated peak times were slightly slower in the placebo than HC (n = 52; 104 eyes) subgroup (coefficient/95% confidence interval = 0.53/0.21-0.85 and 0.36/0.13-0.60; P = .012 and .022); a predominantly rod system-mediated peak time was slightly faster in the EPO than the HC subgroup (coefficient/95% confidence interval = -4.33/-6.88 to -1.78; P = .011). Secondary outcomes were comparable across subgroups. Administration of high-dose rhEPO to infants born extremely or very preterm during the perinatal period has no measurable effects on retinal function in childhood compared to placebo. Premature birth may cause small, likely clinically insignificant effects on retinal function in childhood, which may be partially mitigated by administration of rhEPO during the perinatal period.

The Effect of Perinatal High‐Dose Erythropoietin on Retinal Structural and Vascular Characteristics in Children Born Preterm

To study the long-term effects of perinatal high-dose recombinant human erythropoietin (rhEPO) on macular structural and vascular development in preterm children. Randomized, double-blind clinical trial follow-up plus cohort study. Setting: Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland. extremely or very preterm born children aged 7-15 years from an ongoing neuropediatric study (EpoKids). These had been previously randomized to receive either high-dose rhEPO or placebo perinatally. participation in the EpoKids Study, written informed consent (IC). previous ocular trauma or surgery; retinal or developmental disease unrelated to prematurity. Term-born children of comparable age were enrolled as a healthy control (HC) group. term birth, IC. any ocular or visual abnormality, high refractive error. Examiners were blinded regarding intervention status until completion of all analyses. (Participants/guardians remain blinded). Spectral-domain OCT scans (Heidelberg Spectralis system) and OCTA imaging (Zeiss PlexElite 9000) were obtained. Ophthalmological and orthoptic examinations excluded ocular comorbidities. OCT (central retinal thickness, CRT; total macular volume, TMV), superficial plexus OCTA (foveal avascular zone, FAZ; vessel density, VD; vessel length density, VLD) parameters and foveal hypoplasia grade according to published criteria. Macular vessel density parameters (VD and VLD) were significantly lower (p =0.015, CI-95: 0.01 to 0.06 and p=0.015, CI-95: 0.74 to 3.64) in the EPO group (n= 52) when compared to placebo (n=35). No other significant differences were observed between the EPO and placebo group. When comparing the intervention subgroups to HC we found six significant differences in OCT and OCTA parameters (FAZ, VD, VLD and CRT comparing HC and EPO group; FAZ and CRT when comparing HC and placebo group). Early high-dose rhEPO in infants born extremely or very preterm affects macular vessel density parameters compared to placebo. Premature birth (regardless of intervention status) affects retinal structure and vascular development. Our findings on macular vascular development do not contraindicate the administration of early high-dose EPO in preterm infants. For further understanding of the role of EPO on macular development and its clinical significance, future studies are needed.

Pediatric refractive surgery: current opinion in ophthalmology.

Pediatric refractive surgery has been growing at a steady pace since its introduction in the early 1980 s. This article will review common laser refractive surgeries performed on pediatric patients along with controversies regarding the practice. Pediatric refractive surgery is reserved for a small population of children who fail amblyopic treatment due to high anisometropic refractive errors. Publications over the years have treated these children with various types of laser refractive surgery. Laser pediatric refractive surgery appears to be well tolerated and effective for the population of children that need it. It provides an alternative for anisometropic amblyopia treatment for children who would have otherwise not been able to improve their vision.

Comparative Analysis of Visual Outcomes and Complications in Intraocular Collamer Lens, Small-Incision Lenticule Extraction, and Laser-Assisted In Situ Keratomileusis Surgeries: A Comprehensive Review.

This review provides a comparative analysis of visual outcomes and complications associated with three prominent refractive surgical techniques: intraocular collamer lens (ICL) implantation, small-incision lenticule extraction (SMILE), and laser-assisted in situ keratomileusis (LASIK). Refractive surgeries aim to correct myopia, hyperopia, and astigmatism, offering patients an alternative to corrective lenses. The review highlights the importance of comparing these procedures to guide clinical decision-making effectively. Each technique is described, emphasizing its unique advantages and considerations. While LASIK remains widely favored for its rapid visual recovery and high patient satisfaction, ICL is suitable for patients with higher refractive errors or corneal irregularities. Although relatively newer, SMILE shows promise with potential benefits such as corneal biomechanical stability and a reduced risk of dry eye syndrome. However, each procedure carries its distinct complications, reinforcing the need for personalized patient care and informed decision-making. Understanding these techniques' relative efficacy and safety profiles is essential for optimizing outcomes and enhancing patient satisfaction. Continued advancements in technology and surgical techniques promise further improvements in refractive surgery outcomes, underscoring the importance of ongoing research and innovation.

Recalled Age of Myopia Onset May Predict Risk of High Adult Myopia in Chinese Adults

Introduction: This study aimed to investigate the relationship between the age of myopia onset and high myopia and to explore if age of onset mediated the associations of high myopia with parental myopia and time spent on electronics. Methods: This cross-sectional study enrolled 1,118 myopic patients aged 18–40. Information was obtained via a detailed questionnaire. Multivariable logistic regression and linear regression models were utilized to assess age of onset in relation to high myopia and spherical equivalent refractive error, respectively. Structural equation models examined the mediated effect of onset age on the association between parental myopia, time spent on electronics, and high myopia. Results: An early age at myopia onset was negatively correlated with spherical equivalent refractive power. Subjects who developed myopia before the age of 12 were more likely to suffer from high myopia than those who developed myopia after the age of 15. Age of myopia onset was the strongest predictor of high myopia, with an area under the curve in receiver operator characteristic analysis of 0.80. Additionally, the age of myopia onset served as a mediator in the relationships between parental myopia, electronic device usage duration, and the onset of high myopia in adulthood. Conclusions: Age of myopia onset might be the single best predictor for high myopia, and age at onset appeared to mediate the associations of high myopia with parental myopia and time spent on electronics.

Hybrid Contact Lenses in Children: a Case Series

Introduction : Refractive errors in children have experienced a significant increase in recent years. Contact lenses are a therapeutic option when spectacles can no longer provide maximum results. One of the most advanced design than suitable for children is Hybrid Contact Lenses (HCL). HCL feature a Rigid Gas Permeable (RGP) center surrounded by a soft ridge or skirt. This design enable the hard center to provide clear vision while the soft skirt offers the comfort and ease of a soft lens. However, this type of contact lens has not been widely used in Indonesia.
 Case Illustration : Three patients were prescribed HCL to correct their high refractive errors and amblyopia; 1. 5 years old female: OD: S-14.50 C-3.25x180 6/24 OS: S-12.5 C-3.50x10 6/24; 2. 5 years old female with anisometropia: OD: S-6.00 C-2.50x180 6/12 OS: S-0.75 C-2.000x170 6/6; 3. 8 years old male with pseudophakic eyes: OD: S-9.00 C-2.00x180 6/30 OS: S-8.00 C-2.00x175 6/30. Visual Acuity after 1 month of HCL average from 6/9 to 6/6 both eyes (BE).
 Discussion : In this study, we can notice several advantages of using HCL. The main outcome are significantly increased visual acuity and reduced amblyopia. HCL also has a higher comfort level compare to RGP Lenses, the soft hydrogel skirt really help the neophyte patient to ease their initial adaptation.
 Conclusion : Contact lenses are a mainstay of optical correction options for children with special refractive needs. Due to it's design and comfort, HCL is one of the most suitable contact lenses for children. Therefore, socialization and further introduction about this contact lens is needed.

Impact of stimulant treatment on refractive errors and pupil diameter in attention deficit hyperactivity disorder.

The relationship between attention deficit hyperactivity disorder (ADHD) and visual impairment remains poorly understood, and the impact of visual impairment on the development of ADHD is uncertain. The aim of this study was to investigate the refractive profile and ocular biometric characteristics in patients diagnosed with ADHD and compare them with a control group. Additionally, we aimed to explore the potential influence of sex and medication intake. A cohort of 100 participants, including 50 individuals with ADHD and 50 age- and sex-matched control subjects, was included in this study. Ocular biometric parameters were measured, and refractive error was assessed using cycloplegic and non-cycloplegic autorefraction. Subgroup analyses were performed within the ADHD group based on sex, medication intake and age to investigate potential associations with the ocular findings. We observed no statistically significant differences in axial length, corneal topography parameters or anterior chamber characteristics between ADHD and control subjects. However, subgroup analysis within the ADHD group revealed that the prevalence of ametropia under cycloplegia was significantly higher in unmedicated (69.6%) compared to medicated (37.5%) (X2(2) = 7.320, p = 0.026) participants. Pupil diameter was significantly larger in medicated (3.91 mm) compared to unmedicated (3.58 mm; p = 0.017) individuals. Males had flatter (p = 0.004) and thicker (p = 0.008) corneas than females. Older ADHD participants had higher refractive error (p = 0.008 for non-cycloplegic and p = 0.0.003 for cycloplegic), axial length (p = 0.002) and corneal astigmatism (p = 0.049). Our study provides compelling evidence that individuals diagnosed with ADHD exhibit a similar incidence of refractive errors and ocular parameters compared to normal subjects. Nonetheless, the prevalence of refractive errors appears to be higher in unmedicated ADHD patients, suggesting the potential benefit of stimulant treatment. Additionally, stimulant use is associated with an increase in pupil diameter.

Refractive Changes After Horizontal Strabismus Surgery

Purpose To investigate the changes in refractive status after surgery in patients with horizontal strabismus and high refractive error. Methods This was a prospective study of patients with horizontal strabismus and high refractive error. The patients were divided into a horizontal rectus recession group (group 1) and a horizontal rectus recession combined with horizontal rectus resection group (group 2). The postoperative follow-up duration was 3 months. The refractive status of the patients was evaluated at each postoperative examination, and the refractive changes in the two groups were compared. Results The spherical equivalent in group 1 changed by –0.26 D at 3 months postoperatively relative to the preoperative value (p = 0.078), indicating gradual progression toward myopia over time, but the difference was not significant; however, the postoperative cylinder in group 1 significantly increased by 0.34 D at 3 months postoperatively relative to the preoperative value (p = 0.03). The spherical equivalent in group 2 also indicated progression toward myopia; compared with the preoperative value, the spherical equivalent significantly decreased by –0.28 D (p = 0.019) at 1 month postoperatively and decreased by –0.21 D at 3 months postoperatively. The regression line drawn among the points also indicated a progression in the spherical equivalent toward myopia. In group 2, the cylinder increased by 0.30 D (p = 0.004) from the preoperative level at 1 month postoperatively, peaked, then decreased by 3 months postoperatively. Conclusions Patients with high refractive error who undergo horizontal strabismus correction will experience myopic shift. Patients who undergo rectus recession surgery should be fully informed of the possibility of changes in astigmatism preoperatively. For patients who undergo horizontal rectus recession combined with horizontal rectus resection, it is not recommended that glasses be changed within 1 month after surgery.

Retinopathy of prematurity screening: prevalence and risk factors of ophthalmic complications in non-treated preterm infants.

Retinopathy of prematurity (ROP) is a vision-threatening disease of premature infants. Practice guidelines recommend that all infants screened for ROP receive follow-up eye examinations to screen for ophthalmic complications.1 The purpose of this study was to identify risk factors for the development of strabismus, amblyopia, high refractive error, and cataracts among ROP-screened, non-treated infants. Retrospective single-centre study of ROP-screened, non-treated premature infants with ophthalmic follow-up. Clinical variables were screened for association with ocular findings at follow-up. Multivariable logistic regression was used to determine the risk factors associated with ocular findings. 309 patients were seen for follow-up at 0.97 (0.69) [mean (SD)] years after neonatal intensive care unit (NICU) discharge. Strabismus was predicted by occipitofrontal circumference (OFC) z-score at NICU discharge (OR 0.61; 95% CI [0.42, 0.88]; p = 0.008), intraventricular haemorrhage (IVH) grade III or IV (OR 3.18; 95% CI [1.18, 8.54]; p = 0.02), and exclusive formula feeding at NICU discharge (OR 2.20; 95% CI [1.07, 4.53]; p = 0.03). Significant predictors of amblyopia were OFC z-score at discharge (OR 0.55; 95% CI [0.31, 0.96]; p = 0.03) and necrotising enterocolitis (NEC) (OR 6.94; 95% CI [1.38, 35.00]; p = 0.02). NEC was a significant risk factor for high refractive error (OR 7.27; 95% CI [1.39, 37.94]; p = 0.02). Among premature infants screened but not treated for ROP, severe IVH, NEC, low OFC z-score, and exclusive formula feeding at NICU discharge were risk factors for ocular morbidity. These findings affirm the value of ophthalmic follow-up for all ROP-screened infants, particularly those with the identified risk factors.

Subnormal visual acuity after compliant amblyopia therapy: residual/refractory amblyopia or co-existing pathology? - a retrospective analysis

ABSTRACT Purpose: To assess the prevalence of alternate etiology/co-existing pathology among patients with amblyopia, and to characterize factors contributing to over-diagnosis of amblyopia. Methods: We retrospectively reviewed records of children (from 1 January 2016 to 31 December 2019) who were initially diagnosed as “amblyopia” but later an alternate diagnosis for subnormal vision was established. Patients who had a best corrected visual acuity (BCVA) of ≤20/32 (0.2 logMAR) after compliant amblyopia therapy were divided into 2 groups: those with refractory amblyopia (BCVA improvement from baseline <1 logMAR line) and residual amblyopia (BCVA improvement from baseline >1 logMAR line). Data was collected for presence/absence of amblyogenic risk factors, history, ocular examination, and investigations leading to the final alternate diagnosis. We analyzed the factors that contributed to the initial over-diagnosis of amblyopia using the diagnostic error evaluation and research (DEER) taxonomy tool. Results: During the study period, 508 children with an initial diagnosis of amblyopia met the study criteria. Among these 508 children, 466 were diagnosed to have amblyopia alone, while 26 children (5.1%, median age: 7 years, 17 boys: 9 girls) were revised to have an alternate diagnosis/co-existing pathology. These 26 patients comprised of 2 groups: children referred to us as amblyopia but rediagnosed to have an alternate diagnosis; and a second subset, initially diagnosed by us to have amblyopia, but later found to have alternate diagnosis/co-existing pathology. Subclinical optic neuritis (50%, 13 children), and occult macular dystrophy (OMD) (38.4%, 10 children) were the most frequent alternative diagnoses. Children with ametropic amblyopia (8/26, 30.7%) were most frequently misdiagnosed. Risk factors that led to an initial diagnosis of amblyopia were: high refractive error and heterotropia in 7 patients each (26.9%), anisometropia in 12 (46.1%), and prior pediatric cataract surgery in 4(15.3%). No improvement in BCVA in 21/26 (80.7%) children led to suspicion of co-existing etiology. Other clues were optic disc pallor (11), subnormal color vision (7), history of parental consanguinity in 7, and preceding febrile illness/rhinitis in 1 child. The DEER taxonomy tool suggested that the most common reasons for diagnostic errors were over-emphasis on amblyopia. Conclusion: Our study suggests that 5% of children diagnosed with amblyopia might have co-existing/alternate etiology. Most common co-existing etiologies were subclinical optic neuropathy, and OMD. No improvement in BCVA, subtle history and examination findings prompted further workup. Not considering co-existing etiologies was the most common reason for an initial overdiagnosis of amblyopia.

Noncompliance to spectacle wear among adults − Delta region, Egypt

Background Spectacles are one of the corrective measures for different errors of refraction. A great proportion of people worldwide are blind due to having a high-refractive error while they neglect using the appropriate corrective measures. The aim is to study the prevalence of noncompliance to spectacle wear and its predisposing factors among adults with refractive errors in Menoufia and Gharbeya Governorates. Patients and methods A cross-sectional study performed in Menoufia and Gharbeya Governorates included 290 adults recruited from government and private ophthalmological centers. Data were collected through a predesigned questionnaire asking about personal data, ophthalmological data, measures of compliance, and predisposing factors to noncompliance. Results About 38% of the participants were noncompliant to eyeglasses wearing. Among noncompliant, personal causes represented 63.1%, followed by spectacle-related causes (22.5%) and financial causes (14.4%). Rural residence and illiteracy were significantly higher among noncompliant participants. Multivariate analysis revealed that secondary and higher education were independent protective factors against noncompliance. Conclusion Noncompliance to spectacle wear was high and was significantly related to rural residence and higher levels of education.

Refractive surgery to correct visual impairments in 267 children with autism spectrum and related neuro-developmental disorders: improvements in vision and behavior.

Children with autism spectrum disorder (ASD) may have impaired vision owing to high refractive errors and aversion to spectacles or contact lenses. Visual blurring is caused by near-sighted myopia, far-sighted hyperopia, or astigmatism in one or both eyes. Refractive surgery can restore sharp vision and eliminate the need for spectacles and contact lenses. Restoration of sharp vision may improve ASD behavior. We aimed to determine the refractive outcomes in this cohort using ophthalmic measures and behavioral and school performance alterations after refractive surgery by employing parent-proxy reports. This interventional, retrospective case series included data from 267 children with refractive errors and neurodevelopmental disorders (NDDs) diagnosed as ASD alone or NDD with ASD-like behaviors over a 15-year period. One of three refractive surgery methods was employed, with the choice of method uniquely tailored to the child's eye anatomy. Laser photorefractive keratectomy (PRK) was performed in 131 children, implantation of a phakic intraocular lens (pIOL) in 115 children, and removal of the crystalline lens and implantation of an intraocular lens (refractive lens exchange, RLE) in 21 children. All procedures were performed under brief general anesthesia, with the child returning home on the same day. The median age at surgery was 10.9 years and the median follow-up period was 3.1 years. Pre-operative refractive errors ranged from a mean (standard deviation) +7.5 (0.09) D to -14.3 (4.8) D. Surgery corrected 87% of the children to normal focal length (± 1 D). Visual acuity improved an average of 0.6 logarithm of the minimum angle of resolution, the equivalent of 6 lines on a standard eye chart. Change in visual acuity was significant (all P < 0.01) between baseline and the most recent follow-up examination in each of subgroups. Change in spherical equivalent refractive error at 3, 12, 24, 36, 60, and > 60 months post-operatively were significant (all P < 0.01) between baseline and each follow-up visit in each of subgroups. Social interactions and ASD behaviors improved in 72% (192) of the treated children (P < 0.01). The incidence of sight-threatening complications was low. Refractive surgery improves both visual function and behavior in most children with ASD and major myopia, hyperopia, or astigmatism. The PRK, pIOL, and RLE procedures appear to be effective and reasonably safe methods for improving refractive error, visual acuity, and behavior in many ametropic children with ASD and ASD-like NDDs.

Observation of the effect of posterior scleral reinforcement combined with orthokeratology and 0.01% atropine in the treatment of congenital myopia: a case report

BackgroundMyopia has recently emerged as a significant threat to global public health. The high and pathological myopia in children and adolescents could result in irreversible damage to eye tissues and severe impairment of visual function without timely control. Posterior scleral reinforcement (PSR) can effectively control the progression of high myopia by limiting posterior scleral expansion, improving retrobulbar vascular perfusion, thereby stabilizing the axial length and refraction of the eye. Moreover, orthokeratology and low concentrations of atropine are also effective in slowing myopia progression.Case presentationA female child was diagnosed with binocular congenital myopia and amblyopia at the age of 3 and the patient’s vision had never been rectified with spectacles at the first consultation. The patient’s ophthalmological findings suggested, high refractive error with low best corrected visual acuity, longer axial length beyond the standard level of her age, and fundus examination suggesting posterior scleral staphyloma with weakened hemodynamics of the posterior ciliary artery. Thereby, PSR was performed to improve fundus health and the combination of orthokeratology and 0.01% atropine were performed to control the development of myopia. Following up to 8 years of clinical treatment and observations, the progression of myopia could be well controlled and fundus health was stable.ConclusionIn this report, 8-year of clinical observation indicated that PSR could improve choroidal thickness and hemodynamic parameters of the retrobulbar vessels, postoperative orthokeratology combined with 0.01% atropine treatment strategy may be a good choice for myopia control effectively.

Changes in relative peripheral refraction and optical quality in Chinese myopic patients after small incision lenticule extraction surgery.

To observe changes in retinal refraction difference values (RDV) and aberrations after small incision lenticule extraction (SMILE) surgery and evaluate their correlations. This study recruited 112 patients (112 eyes) who underwent SMILE for myopia. Participants were classified into the Low and Moderate Myopia group (LM, -0.50 to -6.0 D) and High Myopia group (HM, >-6.0 D) according to the central spherical equivalent (SE). RDVs in the five retinal eccentricities from 0° to 10°, 10° to 20°, 20° to 30°, 30° to 40°, and 40° to 53° are recorded as RDV-(0-10), RDV-(10-20), RDV-(20-30), RDV-(30-40), and RDV-(40-53), respectively; additionally, RDVs have four sectors, i.e., RDV-Superior (RDV-S), RDV-Inferior (RDV-I), RDV-Temporal (RDV-T), and RDV-Nasal (RDV-N). With a 3-month follow-up, changes in RDV (ΔRDV) and changes in aberrations [Δtrefoil, Δcoma, Δspherical aberration (SA), and Δtotal higher-order aberrations (HOA)] after surgery were recorded. No significant differences were observed in total RDV (TRDV), RDV-(0-53), RDV-S, RDV-I, RDV-N, trefoil, coma, and SA between the two groups before SMILE surgery. However, after SMILE, hyperopic defocus values [TRDV, RDV-(20-53), RDV-S, RDV-T, and RDV-N] in the LM group and hyperopic defocus values [TRDV, RDV-(20-53), RDV-S, and RDV-N] in the HM group were significantly lower at 3 months postoperatively than preoperatively, and the RDV-(40-53), RDV-S, and RDV-N were lower in the HM group than in the LM group. Aberrations [trefoil (vertical), coma, and HOA] in the LM group and aberrations (trefoil, coma, SA, and HOA) in the HM group were significantly higher at 3 months postoperatively than preoperatively, and the coma, trefoil(horizontal), SA, and HOA were higher in the HM group than in the LM group. In the multivariate analysis, ΔRDV-(40-53) was significantly correlated with ΔSA, and ΔRDV-T and ΔRDV-N were significantly correlated with Δcoma (horizontal). Our findings suggest that SMILE reduces retinal peripheral hyperopic defocus but introduces some higher-order aberrations, especially in people with high myopia refractive errors.

Amputated ICL haptic

23 yrs/F, high myope wanted to get rid of glasses. Ophthalmic examination revealed Best Corrected Visual Acuity (BCVA) of 6/12 (P), N6 with −15.50 DS/−2.50DC × 180° in right eye (RE), and 6/18(P), N6 with −18 DS/−1.50DC × 180° in left eye. Due to high refractive error, she was planned for BE toric Implantable Collamer Lens (ICL) implantation. Necessary investigations were done, and she underwent RE ICL implantation first. During ICL implantation, the foam tip applicator entrapped one trailing haptic in the injector nozzle resulting in an amputation of a part of the right trailing haptic. The ICL with three intact haptics was implanted in the sulcus. Next day, VA in RE was 6/18, N6. Slit-lamp examination and anterior segment OCT showed ICL in place with normal vaulting and no tilt.

Systematic ophthalmologic evaluation in cardio-facio-cutaneous syndrome: A genotype-endophenotype correlation.

Cardio-facio-cutaneous syndrome (CFCS) is a rare genetic disorder belonging to the RASopathies, a group of developmental syndromes caused by upregulated RAS/MAPK signaling. Pathogenic variants affecting four genes, KRAS, BRAF, MAP2K1 and MAP2K2, encoding core signal transducers of the pathway, underlie the condition. Major clinical features include a distinctive facies, ectodermal and cardiac anomalies, reduced postnatal growth, intellectual disability, and musculoskeletal abnormalities. Similar to other RASopathies, reports of visual impairment, high refractive error, optic nerve pallor, and other ocular abnormalities have been anecdotally reported in the literature. The aim of our study is to report the prevalence of ophthalmologic abnormalities in a large monocentric cohort of individuals affected by CFCS and explore the occurrence of genotype-endophenotype correlations in this series of patients. We observed that BRAF mutations are associated to a higher prevalence of anisometropia >3D (11.8% vs. 0%) and high astigmatism (29.4% vs. 0%; both p < 0.001) while patients with mutations in other genes had a significantly higher prevalence of myopia >6 D (60% vs. 5.9%; p = 0.012). Pale optic disc was associated with higher prevalence of inferior oblique muscle (IO) overaction (33.3% vs. 0%) and lower prevalence of ptosis (0% vs. 11.8%; both p < 0.001). Combined exotropia, IO overaction and nystagmus were frequent in patients with pale optic nerve. Our findings might suggest the need for earlier ophthalmologic referral for CFCS patients due to high risk of amblyopia, especially those expressing BRAF mutations.

Correlation of the macular microvasculature to the axial length in pediatric patients with high axial refractive errors.

To compare the vascular density (VD) of the macular superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC), and the foveal avascular zone (FAZ) among high hyperopic, high myopic, and emmetropic children using optical coherence tomography angiography (OCTA). This was a cross sectional comparative study of otherwise healthy children with different refractive errors. Patients were recruited from Cairo University Children's Hospital. OCTA imaging was performed using the RTVue XR Avanti device with AngioVue software. Both the 3 × 3 and 6 × 6 mm macular scans were utilized. Automated measurements were obtained from the built-in machine software. Axial length (AL) measurements were done using Lenstar LS 900 optical biometer. Ninety eyes from 51 healthy children were included. Among high myopes, there was significant thinning of the parafovea (p < 0.001). SCP-VD was also lower in high myopes in all areas except the fovea (all p < 0.001). The DCP-VD was significantly lower in high myopes in the parafovea and perifovea. High hyperopes had lower subfoveal CC-VD. Despite high myopes showing a significantly lower OCTA signal strength, linear regression analysis revealed that AL was an independent and significant predictor for the FAZ-area, as well as parafoveal and perifoveal SCP and DCP-VD. High myopia results in a reduction of VD in both the SCP and DCP, which can be non-invasively detected and monitored using OCTA. While lower VD may, in part, be attributed to lower OCTA image quality, our findings demonstrate that AL independently and significantly predicts macular vascular parameters on OCTA in children.

Ocular residual astigmatism (ORA) does not seem to correlate with baseline refractive error among refractive surgery candidates.

Ocular residual astigmatism (ORA) is defined as the difference between refractive astigmatism and anterior corneal astigmatism. A high ORA may be correlated with poorer results in patients undergoing corneal-based laser surgery. Is a high baseline refractive error related to a higher degree of ORA? This was a retrospective analytical study including 181 right eyes of an equal number of refractive surgery candidates. Manifest subjective refraction was measured, along with a Pentacam AXL Wave corneal tomography. Via a vector analysis with this methodology, subjective cylinder was translated into the corneal plane and a vectorial subtraction was performed in order to measure ORA. Spearman's rank order test, one-way ANOVA and Chi-square were used to determine whether different levels of baseline refractive error correlate with different levels of ORA. Mean age was 28.33 ± 4.71years with a female preponderance (65.7%). Mean ORA was 0.74 ± 0.39 D, with 33.1% of eyes having an ORA ≥ 0.90 D. There was not a correlation between ORA and level of myopia (rho = - 0.022; p = 0.764), nor between ORA and spherical equivalent (rho = 0.009; p = 0.903). Refractive astigmatism did not demonstrate to be correlated with ORA level either (rho = 0.078; p = 0.329). One-way ANOVA tests failed to demonstrate an association between different classifications of refractive error and level of ORA. In the studied population, ORA is not correlated with baseline refractive error. Every patient presenting for possible corneal-based laser refractive surgery should be evaluated for a possible high level of ORA, irrespective of their baseline ametropia level.

Progression and incidence of myopia among schoolchildren in the post-COVID-19 pandemic period: a prospective cohort study in Shantou, China

ObjectivesTo determine the progression and incidence of myopia in Chinese schoolchildren in the post-COVID-19 pandemic period in Shantou, China.DesignProspective cohort study.SettingShantou Myopia Study, China.Participants1-year follow-up data were available for 621...

Clear lens extraction and refractive lens exchange for the treatment of amblyopia.

Treatment of amblyopia typically involves spectacles or penalization of the nonamblyopic eye with occlusive patching or pharmacological penalization. However, these methods can be ineffective or difficult in certain children who may be unable to tolerate or are poorly compliant with such therapies. Untreated high refractive error can result in dense amblyopia, and thus, other treatment methods are necessary in this subset of children. With technological advances in ocular surgery, clear lens extraction (CLE) and refractive lens exchange (RLE) have emerged as popular alternative treatments for amblyopia, as they may avoid some of the challenges surrounding traditional methods. CLE involves lensectomy for refractive purposes in patients without cataracts, while RLE involves lensectomy followed by intraocular lens implantation. The purpose of this review was to summarize the use of CLE and RLE for the treatment of amblyopia in the pediatric population, discussing indications, techniques, treatment outcomes, safety, and potential complications.