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American Journal of Ophthalmology Case... Dec 2023To report a pediatric patient with bilateral temporal keratoconus.
PURPOSE
To report a pediatric patient with bilateral temporal keratoconus.
OBSERVATIONS
A 14-year-old male presented with a two-year history of progressively worsening visual acuity in both eyes and suspicion for undiagnosed amblyopia in the right eye. Retinoscopy revealed a scissoring reflex in both eyes and corneal topography demonstrated high keratometry values (Kmax 57.9 D and 46.1 D in the right and left eyes, respectively), with relative temporal steepening approximately coinciding with the thinnest pachymetry in both eyes. Corneal cross-linking was recommended.
CONCLUSIONS AND IMPORTANCE
Keratoconus can present as a temporal variant with relative temporal steepening and thinning. It is important to maintain a high index of suspicion for keratoconus in pediatric patients with sub-normal visual acuities. Prompt assessment and diagnosis may prevent progression of keratoconus and development of amblyopia.
PubMed: 37546376
DOI: 10.1016/j.ajoc.2023.101900 -
JAMA Ophthalmology Aug 2023Lensectomy with primary intraocular lens (IOL) implantation is often used in the management of nontraumatic pediatric cataract, but long-term data evaluating the...
IMPORTANCE
Lensectomy with primary intraocular lens (IOL) implantation is often used in the management of nontraumatic pediatric cataract, but long-term data evaluating the association of age and IOL location with the incidence of complications are limited.
OBJECTIVE
To describe the incidence of complications and additional eye surgeries through 5 years following pediatric lensectomy with primary IOL implantation and association with age at surgery and IOL location.
DESIGN, SETTING, AND PARTICIPANTS
This prospective cohort study used Pediatric Eye Disease Investigator Group cataract registry data from 61 institution- and community-based practices over 3 years (June 2012 to July 2015). Participants were children younger than 13 years without baseline glaucoma who had primary IOL implantation (345 bilateral and 264 unilateral) for nontraumatic cataract. Data analysis was performed between September 2021 and January 2023.
EXPOSURES
Lensectomy with primary IOL implantation.
MAIN OUTCOME AND MEASURES
Five-year cumulative incidence of complications by age at surgery (<2 years, 2 to <4 years, 4 to <7 years, and 7 to <13 years) and by IOL location (sulcus vs capsular bag) were estimated using Cox proportional hazards models.
RESULTS
The cohort included 609 eyes from 491 children (mean [SD] age, 5.6 [3.3] years; 261 [53%] male and 230 [47%] female). Following cataract extraction with primary IOL implantation, a frequent complication was surgery for visual axis opacification (VAO) (cumulative incidence, 32%; 95% CI, 27%-36%). Cumulative incidence was lower with anterior vitrectomy at the time of IOL placement (12%; 95% CI, 8%-16%) vs without (58%; 95% CI, 50%-65%), and the risk of undergoing surgery for VAO was associated with not performing anterior vitrectomy (hazard ratio [HR], 6.19; 95% CI, 3.70-10.34; P < .001). After adjusting for anterior vitrectomy at lens surgery, there were no differences in incidence of surgery for VAO by age at surgery (<2 years, HR, 1.35 [95% CI, 0.63-2.87], 2 to <4 years, HR, 0.86 [95% CI, 0.44-1.68], 4 to <7 years, HR, 1.06 [95% CI, 0.72-1.56]; P = .74) or by capsular bag vs sulcus IOL fixation (HR, 1.22; 95% CI, 0.36-4.17; P = .75). Cumulative incidence of glaucoma plus glaucoma suspect by 5 years was 7% (95% CI, 4%-9%), which did not differ by age after controlling for IOL location and laterality.
CONCLUSIONS AND RELEVANCE
In this cohort study, a frequent complication following pediatric lensectomy with primary IOL was surgery for VAO, which was associated with primary anterior vitrectomy not being performed but was not associated with age at surgery or IOL location. The risk of glaucoma development across all ages at surgery suggests a need for long-term monitoring.
Topics: Child; Humans; Male; Female; Child, Preschool; Lens Implantation, Intraocular; Cohort Studies; Prospective Studies; Visual Acuity; Cataract Extraction; Cataract; Glaucoma; Ocular Hypertension
PubMed: 37347490
DOI: 10.1001/jamaophthalmol.2023.2335 -
EClinicalMedicine Apr 2024Amblyopia is a common neurodevelopmental condition and leading cause of childhood visual impairment. Given the known association between neurodevelopmental impairment...
BACKGROUND
Amblyopia is a common neurodevelopmental condition and leading cause of childhood visual impairment. Given the known association between neurodevelopmental impairment and cardiometabolic dysfunction in later life, we investigated whether children with amblyopia have increased risk of cardiometabolic disorders in adult life.
METHODS
This was a cross-sectional and longitudinal analysis of 126,399 United Kingdom Biobank cohort participants who underwent ocular examination. A subset of 67,321 of these received retinal imaging. Data analysis was conducted between November 1st 2021 and October 15th 2022. Our primary objective was to investigate the association between amblyopia and a number of components of metabolic syndrome and individual cardiometabolic diseases. Childhood amblyopia, dichotomised as or by adulthood, cardiometabolic disease and mortality were defined using ophthalmic assessment, self-reported, hospital admissions and death records. Morphological features of the optic nerve and retinal vasculature and sublayers were extracted from retinal photography and optical coherence tomography. Associations between amblyopia and cardiometabolic disorders as well as retinal markers were investigated in multivariable-adjusted regression models.
FINDINGS
Individuals with persisting amblyopia (n = 2647) were more likely to be obese (adjusted odds ratio (95% confidence interval): 1.16 (1.05; 1.28)), hypertensive (1.25 (1.13; 1.38)) and diabetic (1.29 (1.04; 1.59)) than individuals without amblyopia (controls, (n = 18,481)). Amblyopia was also associated with an increased risk of myocardial infarction (adjusted hazard ratio: 1.38 (1.11; 1.72)) and death (1.36 (1.15; 1.60)). On retinal imaging, amblyopic eyes had significantly increased venular caliber (0.29 units (0.21; 0.36)), increased tortuosity (0.11 units (0.03; 0.19)), but lower fractal dimension (-0.23 units (-0.30; -0.16)) and thinner ganglion cell-inner plexiform layer (mGC-IPL, -2.85 microns (-3.47; -2.22)). Unaffected fellow eyes of individuals with amblyopia also had significantly lower retinal fractal dimension (-0.08 units (-0.15; -0.01)) and thinner mGC-IPL (-1.14 microns (-1.74; -0.54)). Amblyopic eyes with a persisting visual deficit had smaller optic nerve disc height (-0.17 units (-0.25; -0.08)) and width (-0.13 units (-0.21; -0.04)) compared to control eyes.
INTERPRETATION
Although further research is needed to understand the basis of the observed associations, healthcare professionals should be cognisant of greater cardiometabolic dysfunction in adults who had childhood amblyopia. Differences in retinal features in both the amblyopic eye and the unaffected non-amblyopic suggest generalised versus local processes.
FUNDING
Medical Research Council (MR/T000953/1) and the National Institute for Health and Care Research.
PubMed: 38685932
DOI: 10.1016/j.eclinm.2024.102493 -
BMC Ophthalmology Jun 2024The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid...
BACKGROUND
The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid ophthalmic conditions in patients with spasmus nutans.
METHODS
We retrospectively reviewed the charts of consecutive patients diagnosed with spasmus nutans between 2000 and 2020. Demographic information, ophthalmic characteristics, and neuroimaging results were assessed over time.
RESULTS
Of the 32 patients included in the study, 13 (41%) were female. Underlying medical conditions included a diagnosis of Trisomy 21 in 6 (19%) and prematurity in 8 (25%). Twenty-one patients (66%) self-reported as a race other than Caucasian. 18 patients (56%) had non-private health insurance and 1 (3%) was uninsured. Mean age at diagnosis and resolution were 16 months (range 45 months) and 48 months (range 114 months), respectively. All 32 patients had nystagmus, 31 (97%) had head nodding and 16 (50%) had ocular torticollis. Mean follow-up was 66 months (range 185 months). On initial presentation, 6/32 (19%) had an amblyogenic refractive error and mean best-corrected visual acuity (BCVA) in the better-seeing eye was 0.78 Logarithm of the Minimum Angle of Resolution (LogMAR) (range 1.24). In a sub-analysis that included patients with > 1 exam (n = 23), 17/20 (85%) had an amblyogenic refractive error and mean BCVA in the better-seeing eye was 0.48 LogMAR (range 1.70). At the final exam, 12 patients had measurable stereopsis, eight had strabismus, and three had undergone strabismus surgery. Eight patients required treatment for amblyopia.
CONCLUSIONS
We found a high prevalence of amblyogenic refractive error, strabismus and amblyopia among patients with spasmus nutans. Children with spasmus nutans benefit from ongoing ophthalmic follow-up until they are past the amblyopic age range, even after resolution of nystagmus.
Topics: Humans; Female; Male; Retrospective Studies; Visual Acuity; Child, Preschool; Child; Infant; Follow-Up Studies; Nystagmus, Pathologic; Amblyopia; Strabismus
PubMed: 38867186
DOI: 10.1186/s12886-024-03494-7 -
JAMA Ophthalmology Mar 2024Amblyopia can result in permanent vision loss if not properly treated before age 7 years. In 2017, the US Preventive Services Task Force recommended that vision...
IMPORTANCE
Amblyopia can result in permanent vision loss if not properly treated before age 7 years. In 2017, the US Preventive Services Task Force recommended that vision screening should occur at least once in all children aged 3 to 5 years to detect amblyopia.
OBJECTIVE
To understand trends and factors associated with screening, referral, or diagnosis of amblyopia before and after photoscreening expansion across a relatively large health care system in late 2017.
DESIGN, SETTING, AND PARTICIPANTS
This is a retrospective cohort study of electronic health record data from patients with a well child care visit at approximately age 3 years (ages 2.75-3.25 years) in a relatively large, multispecialty group practice in Northern California and linked census data between 2015 and 2022. Data were extracted and analyzed from October 2022 through August 2023.
EXPOSURES
Patient sex, race and ethnicity, immunization records, previous well child care visits, and census-level median household income.
MAIN OUTCOMES AND MEASURES
Vision screening, pediatric ophthalmology referral, or amblyopia diagnosis, compared using adjusted odds ratios (AORs).
RESULTS
The study included 2015-2017 data from 23 246 patients aged 3 years with at least 1 well child care visit (11 206 [48.2%] female) compared with 2018-2022 postexpansion data from 34 281 patients (16 517 [48.2%] female). The screening rate increased from 5.7% (424 of 7505) in 2015 to 72.1% (4578 of 6354) in 2022. The referral rate increased from 17.0% (1279 of 7505) in 2015 to 23.6% (1836 of 7792) in 2018. The diagnosis rate was 2.7% (200 of 7505) in 2015, peaked at 3.4% (263 of 7792) in 2018, and decreased to 1.4% (88 of 6354) in 2022. Compared with White patients, patients who were Asian, Black, or Hispanic were less likely to be screened (Asian: AOR, 0.80; 95% CI, 0.72-0.88; Black: AOR, 0.71; 95% CI, 0.53-0.96; Hispanic: AOR, 0.88; 95% CI, 0.80-0.97). Compared with White patients, patients who were Asian or Hispanic were more likely to be referred (Asian: AOR, 1.49; 95% CI, 1.36-1.62; Hispanic: AOR, 1.32; 95% CI, 1.18-1.48) and were more likely to be diagnosed (Asian: AOR, 1.29; 95% CI, 1.07-1.56; Hispanic: AOR, 1.67; 95% CI, 1.33-2.11).
CONCLUSIONS AND RELEVANCE
In this study, increased availability of photoscreeners was associated with an increase in overall rates of vision screening for children aged 3 years in a relatively large health care system. Given that US rates of visual impairment are predicted to increase, additional targeted interventions would be needed to address remaining disparities in amblyopia care along patient- and clinician-level factors.
Topics: Humans; Female; Male; Amblyopia; Retrospective Studies; Ethnicity; Vision Disorders; Vision Screening
PubMed: 38300546
DOI: 10.1001/jamaophthalmol.2023.6434 -
Archivos de La Sociedad Espanola de... Dec 2023Nanophthalmos is a rare congenital condition of the eyeball that is characterised by a smaller size of the anterior and posterior segments without associated ocular... (Review)
Review
Nanophthalmos is a rare congenital condition of the eyeball that is characterised by a smaller size of the anterior and posterior segments without associated ocular malformations. Typical features that have traditionally been described in these eyes are short axial length, thickened sclera, cornea with a smaller diameter, narrow anterior chamber, and an increased lens to globe volume ratio. However, at present, there is still a lack of recognised diagnostic criteria for nanophthalmos and a classification of its severity. Its clinical relevance stems from the increased risk of multiple ocular conditions, such as high hyperopia, amblyopia, angle-closure glaucoma, retinal detachment, and cataracts. Likewise, in relation to surgery in these eyes, there are particularities in cataract and glaucoma surgery and with a greater risk of associated intra- and postoperative complications. In this way, the treatment of nanophthalmos focuses on controlling the associated eye conditions and reducing and controlling surgical complications. This review aims to update what has been published in recent years regarding nanophthalmos.
Topics: Humans; Microphthalmos; Glaucoma, Angle-Closure; Lens, Crystalline; Hyperopia; Vision, Ocular; Cataract
PubMed: 37813187
DOI: 10.1016/j.oftale.2023.10.003 -
Clinical & Experimental Optometry Nov 2023Understanding the quality-of-life (QoL) impacts of amblyopia and strabismus from the perspectives of patients using validated tools would enable eye care practitioners...
CLINICAL RELEVANCE
Understanding the quality-of-life (QoL) impacts of amblyopia and strabismus from the perspectives of patients using validated tools would enable eye care practitioners to provide better clinical management.
BACKGROUND
Item banks are advanced patient reported outcome measures with several advantages over traditional QoL assessment. This paper describes the development of amblyopia and strabismus-specific QoL item banks for two distinct country settings: Australia (high-income) and India (low-middle income) and examines the unique QoL issues.
METHODS
Using a bottom-up systematic approach, the content for the item banks was identified from three sources: existing self-report amblyopia and strabismus questionnaires ( = 22), qualitative literature ( = 5) and prospective qualitative studies in Australia ( = 49) and India ( = 30). The initial item pool underwent item evaluation, construction, and pre-testing to form optimal sets of representative items. The Indian item pools were first developed in English and translated into Hindi and Tamil using a rigorous translation protocol. The differences in QoL experiences that emanated from the qualitative studies and the number of common and unique items in the final item pools were compared.
RESULTS
The final Australian and Indian item pools comprised 312 and 277 items, respectively, covering 11 QoL domains. Two hundred and sixty items (79%) were common to both countries, and 21% were unique. Of the 11 domains, except for activity limitation (64.5%), visual symptoms (73.3%) and emotional impact (75.5%) domains, all other domains had over 80% of items common to both countries. The unique items can be attributed to differences in QoL experiences, individual perspectives, culture, lifestyle, country setting and health systems.
CONCLUSION
Amblyopia has a multifaceted impact on QoL irrespective of the country settings. Despite a huge overlap in QoL impacts between Australia and India, both countries had unique issues, especially activity limitations. The study developed comprehensive, country-specific item pools for Australia and India.
PubMed: 37980905
DOI: 10.1080/08164622.2023.2281470 -
Investigative Ophthalmology & Visual... Apr 2024Individuals with amblyopia experience central vision deficits, including loss of visual acuity, binocular vision, and stereopsis. In this study, we examine the...
PURPOSE
Individuals with amblyopia experience central vision deficits, including loss of visual acuity, binocular vision, and stereopsis. In this study, we examine the differences in peripheral binocular imbalance in children with anisometropic amblyopia, strabismic amblyopia, and typical binocular vision to determine if there are systematic patterns of deficits across the visual field.
METHODS
This prospective cohort study recruited 12 participants with anisometropic amblyopia, 10 with strabismic amblyopia, and 10 typically sighted controls (age range, 5-18 years). Binocular imbalance was tested at 0°, 4°, and 8° eccentricities (4 angular locations each) using band-pass filtered Auckland optotypes (5 cycles per optotype) dichoptically presented with differing contrast to each eye. The interocular contrast ratio was adjusted until the participant reported each optotype with equal frequency.
RESULTS
Participants with anisometropic and strabismic amblyopia had a more balanced contrast ratio, or decreased binocular imbalance, at 4° and 8° eccentricities as compared with central vision. Participants with strabismic amblyopia had significantly more binocular imbalance in the periphery as compared with individuals with anisometropic amblyopia or controls. A linear mixed effects model showed a main effect for strabismic amblyopia and eccentricity on binocular imbalance across the visual field.
CONCLUSIONS
There is evidence of decreased binocularity deficits, or interocular suppression, in the periphery in anisometropic and strabismic amblyopia as compared with controls. Notably, those with strabismic amblyopia exhibited more significant peripheral binocular imbalance. These variations in binocularity across the visual field among different amblyopia subtypes may necessitate tailored approaches for dichoptic treatment.
Topics: Humans; Amblyopia; Vision, Binocular; Male; Female; Child; Prospective Studies; Adolescent; Strabismus; Visual Acuity; Visual Fields; Child, Preschool; Anisometropia; Depth Perception
PubMed: 38652649
DOI: 10.1167/iovs.65.4.36 -
Acta Ophthalmologica Nov 2023The purpose of the current study was to evaluate the prevalence of residual amblyopia in adults in a population screened and treated in childhood. We also wanted to...
PURPOSE
The purpose of the current study was to evaluate the prevalence of residual amblyopia in adults in a population screened and treated in childhood. We also wanted to evaluate the association of amblyopia on school success, level of education, and quality of life.
METHODS
This is a follow-up study of 2708 subjects of the Northern Finland Birth Cohort. At the age of 46, the subjects took part in ophthalmic examinations, including the measurement of best-corrected visual acuity (BCVA) and refraction. Residual amblyopia was defined as BCVA 20/30 or less (logMAR ≥0.2) in one or both eyes or a two-line interocular visual acuity difference and absence of any pathological ocular factors. The quality of life was assessed with a 15D questionnaire, and educational outcome, school success, and episodic memory with a CANTAB-PAL (paired associates learning) test were evaluated.
RESULTS
The prevalence of amblyopia in the current adult population aged 46 years was 1.3% (n = 36). At 14 years, the amblyopia subjects had had significant differences in mean spherical equivalent between the amblyopic and fellow eye and strabismus more often than controls. No significant differences were observed in the CANTAB-PAL test or in educational outcome. However, amblyopia subjects had significant difficulties in the 15D questionnaire in terms of vision (54% vs. 34%, p = 0.01).
CONCLUSION
Due to screening and treatment in childhood, the number of adults with residual amblyopia was low. Despite minor visual impairment and discomfort, they cope very well in life in terms of educational outcome and quality of life.
Topics: Adult; Humans; Amblyopia; Follow-Up Studies; Birth Cohort; Prevalence; Quality of Life; Finland; Educational Status
PubMed: 36924319
DOI: 10.1111/aos.15660 -
Journal of Clinical Cases & Reports Oct 2023This is a descriptive summary of the case of a patient with Axenfeld-Rieger syndrome associated with a congenital malformation of the iris and consequent pupillary...
SUMMARY
This is a descriptive summary of the case of a patient with Axenfeld-Rieger syndrome associated with a congenital malformation of the iris and consequent pupillary morphological alteration of an atypical characteristic reported. This anomaly is unique in scientific literature and exhibits a peculiarity that we have called pseudoacorea: Hidden pupil. Other associated abnormal clinical findings were posterior embryotoxon, astigmatism, amblyopia, and exotropia. Diagnosis was achieved by instilling ocular mydriatics into the cul-de-sac that revealed this peculiarity. It is necessary to make a differential diagnosis with other pupillary pathologies such as corectopia, acorea and microcoria. Early detection of pathology and surgical management is necessary, since it would lead to a better visual prognosis for both amblyopia and strabismus.
BACKGROUND
Among the malformations of the pupil, we can find polycoria (more than one pupil), dyscoria (abnormal pupil shape), corectopia (abnormal pupil position) and acorea (absence of pupil). In addition, morphologically normal pupils can denote other anomalies such as the microcoria described by Holth in 1923. Acorea is a rare anomaly, congenital or acquired, characterized by an absolute absence of the pupil both at rest and in mydriasis. In our case we prefer to differentiate it and name it pseudoacorea, since although there is a total absence of the pupil at rest thanks to the application of ocular mydriatics, a micropupil with discoric and corectopic characteristics is achieved. It is worth noting that we have not detected in the scientific literature any case described as the one that we will develop here.
CONCLUSION
The case of a patient with Axenfeld-Rieger syndrome associated with a congenital malformation of the iris and consequent atypical pupillary morphological alteration is presented. This anomaly is unique in the scientific literature and presents a peculiarity that we have called pseudoacorea: Hidden pupil. Early detection of pathology and surgical management is necessary, since it would lead to a better visual prognosis for both amblyopia and strabismus.
PubMed: 37663013
DOI: 10.46619/joccr.2023.6-1145