Effectiveness of the Spot tm Vision Screener With Variations in Ocular Pigments.

PURPOSE: To evaluate Spot in detecting American Association for Pediatric Ophthalmology and Strabismus (AAPOS) Amblyopia risk factors (ARF) and for ARF myopia and hyperopia with variations in ocular pigments. DESIGN: Diagnostic screening test evaluation. METHODS: Study population: Children presented for a complete eye examination in pediatric clinic. The study population included 1040 participants, of whom 273 had darkly pigmented eyes, 303 were medium pigmented, and 464 were light pigmented. INTERVENTION: Children were screened with the Spot vision screener before the complete eye examination. A pediatric ophthalmologist then completed an eye examination, including cycloplegic refraction. The pediatric ophthalmologist was blinded to the result of the Spot vision screener. MAIN OUTCOME: The association between Spot screening recommendation and meeting one or more ARF/ARF + Amblyopia criterion, Spot measured spherical equivalent, and ARF myopia and hyperopia detection. RESULTS: The area under the receiver operative characteristic curve (AUC) for myopia was excellent for all. The AUC for hyperopia was good (darker-pigmented: 0.92, medium-pigmented: 0.81, and lighter-pigmented: 0.86 eyes). The Spot was most sensitive for ARF myopia (lighter-pigmented: 0.78, medium-pigmented: 0.52, darker-pigmented: 0.49). The reverse was found for hyperopia; however, sensitivity was relatively poor. The Spot was found most sensitive for hyperopia in the darker-pigment group (0.46), 0.27 for medium-pigment, and 0.23 for the lighter-pigment cohort. CONCLUSIONS: While the Spot was confirmed as a sensitive screening test with good specificity in our large cohort, the sensitivity of the Spot in detecting AAPOS guidelines for myopia and hyperopia differed with variations in skin pigment. Our results support the consideration of ethnic and racial diversity in future advances in photorefractor technology.

Effectiveness of the Spot Vision Screener using updated 2021 AAPOS guidelines.

PURPOSE: To evaluate the Spot Vision Screener according to updated 2021 AAPOS Vision Screening Committee guidelines for instrument-based pediatric vision screen validation. METHODS: As part of an IRB-approved ongoing prospective study, children were screened with the Spot prior to a complete examination. RESULTS: Spot screening was successful in 1,036 of 1,090 children (95%). Forty-eight percent of participants were referred for further screening using the Spot manufacturer guidelines, and 40% of all children were found to have a 2021 amblyopia risk factor or visually significant refractive error by gold standard examination. The Spot recommendation compared reasonably well to the 2021 criteria, with an overall sensitivity of 0.88 and a specificity of 0.78. Applying updated guidelines to the Spot for hyperopia, anisometropia, and astigmatism yielded moderate-to-poor sensitivity (0.27-0.77) but excellent specificity (>0.9). The area under the curve of the receiver operating characteristic analysis demonstrates overall good prediction performance for the Spot for each diagnosis-myopia, hyperopia, astigmatism, anisometropia (range, 0.87-0.97). Results of our study suggest increasing the instrument referral criterion for astigmatism from 1.5 D (manufacturer thresholds of the screener used in this study) to 2 D in older children. Decreasing the anisometropia cut-off from 1 D to 0.75 D would improve sensitivity from 0.59 to >0.8. CONCLUSIONS: In our study population, the overall predictive ability of the Spot is good, with a sensitivity of 0.88 and a specificity of 0.78. We recommend specific device refractive referral criteria to maximize screening effectiveness using the updated AAPOS guidelines.

The blinq™ Vision Screener in Detection of Amblyopia and Strabismus.

PURPOSE: The blinq (Rebion Inc) is a new screening device designed to directly detect amblyopia and strabismus rather than amblyopia risk factors. We performed an independent assessment of the effectiveness of the blinq in detecting amblyopia and strabismus. DESIGN: Prospective clinical validity analysis of a screening device based on sensitivity and specificity. METHODS: Children presenting for examination in the pediatric ophthalmology clinic underwent screening with the blinq before examination by a pediatric ophthalmologist blinded to the screening results. Results of the blinq and examination findings of strabismus or amblyopia were compared. RESULTS: In our cohort of 267 children with an average age of 6.3 years, the sensitivity of the blinq to detect amblyopia or any constant strabismus was 87.5% (78.2%-93.8%) and specificity was 51.3% (43.9%-58.7%). Using the previously described "appropriate referral gold standard" criteria, including children with intermittent strabismus and high refractive error, the sensitivity increased to 91.3% and the specificity to 63.2%. We found a high number of children (44 [16%]) upon whom the blinq timed out and were included as automatic referrals. CONCLUSIONS: Our results support use of the blinq as a screening device to detect amblyopia and strabismus in children.

Inherited Retinal Dystrophy in Southeastern United States: Characterization of South Carolina Patients and Comparative Literature Review.

Inherited retinal dystrophies (IRDs) are a group of rare diseases involving more than 340 genes and a variety of clinical phenotypes that lead to significant visual impairment. The aim of this study is to evaluate the rates and genetic characteristics of IRDs in the southeastern region of the United States (US). A retrospective chart review was performed on 325 patients with a clinical diagnosis of retinal dystrophy. Data including presenting symptoms, visual acuity, retinal exam findings, imaging findings, and genetic test results were compiled and compared to national and international IRD cohorts. The known ethnic groups included White (64%), African American or Black (30%), Hispanic (3%), and Asian (2%). The most prevalent dystrophies identified clinically were non-syndromic retinitis pigmentosa (29.8%), Stargardt disease (8.3%), Usher syndrome (8.3%), cone-rod dystrophy (8.0%), cone dystrophy (4.9%), and Leber congenital amaurosis (4.3%). Of the 101 patients (31.1%) with genetic testing, 54 (53.5%) had causative genetic variants identified. The most common pathogenic genetic variants were USH2A (n = 11), ABCA4 (n = 8), CLN3 (n = 7), and CEP290 (n = 3). Our study provides initial information characterizing IRDs within the diverse population of the southeastern US, which differs from national and international genetic and diagnostic trends with a relatively high proportion of retinitis pigmentosa in our African American or Black population and a relatively high frequency of USH2A pathogenic variants.

AAPOS uniform guidelines for instrument-based pediatric vision screen validation 2021.

BACKGROUND: As instrument-based pediatric vision screening technology has evolved, the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) has developed uniform guidelines (2003, updated 2013) to inform the development of devices that can detect specified target levels of amblyopia risk factors (ARFs) and visually significant refractive error. Clinical experience with the established guidelines has revealed an apparent high level of over-referral for non-amblyopic, symmetric astigmatism, prompting the current revision. METHODS: The revised guidelines reflect the expert consensus of the AAPOS Vision Screening and Research Committees. RESULTS: For studies of automated screening devices, AAPOS in 2021 recommends that the gold-standard confirmatory comprehensive examination failure levels include anisometropia >1.25 D and hyperopia >4.0 D. Astigmatism >3.0 D in any meridian and myopia < -3 D should be detected in children <48 months, whereas astigmatism >1.75 D and myopia < -2 D should be detected after 48 months. Any media opacity >1 mm and manifest strabismus of >8Δ should also be identified. Along with performance in detecting ARFs and refractive error, validation studies should also report screening instrument performance with regard to presence or absence of amblyopia. Instrument receiver operating characteristic curves and Bland-Altman analysis are suggested to improve comparability of validation studies. CONCLUSIONS: Examination failure criteria have been simplified and the threshold for symmetric astigmatism raised compared to the 2013 guidelines, whereas the threshold for amblyogenic anisometropia has been decreased. After age 4 years, lower magnitudes of symmetric astigmatism and myopia are also targeted despite a low risk of amblyopia, because they can influence school performance and may warrant consideration of myopia prevention therapy.

Evaluation of the Spot Vision Screener in School-Aged Children.

PURPOSE: To determine the accuracy of the Spot Vision Screener (Welch Allyn, Skaneateles Falls, NY) in children 6 years and older and recommend device thresholds to improve its accuracy for the detection of refractive error. METHODS: The Spot Vision Screener results were compared with three gold standard conditions of increasingly narrow refractive error criterion. The sensitivity, specificity, positive predictive value, and negative predictive value of the Spot Vision Screener in detecting each gold standard criterion were calculated. The most accurate threshold setting for each parameter was identified by calculating the area under the curve receiver operating characteristic. RESULTS: The Spot Vision Screener was able to successfully evaluate 313 of 330 children (95%). The sensitivity of the Spot Vision Screener to detect American Association for Pediatric Ophthalmology and Strabismus guidelines for amblyopia risk factors was 89.5% and the specificity was 76.7%. The sensitivity decreased to 80% and the specificity increased to 75.3% with narrower refractive criteria. The sensitivity in detecting refractive criteria improved with the proposed optimized device thresholds. Estimates for the general population indicate that the positive predictive value is reasonable at 52.3% to 61.8%, depending on the stringency of the criteria, with excellent negative predictive values. CONCLUSIONS: In school-aged children, the primary screening focus shifts from preventing amblyopia to detecting visual disturbances, including refractive error, that may interfere with academic performance. In this age group, the Spot Vision Screener was an acceptable method of detecting significant refractive error with improved sensitivity with threshold optimization. [J Pediatr Ophthalmol Strabismus. 2020;57(3):146-153.].

Effectiveness of the iPhone GoCheck Kids smartphone vision screener in detecting amblyopia risk factors.

BACKGROUND: The recently released GoCheck Kids iPhone photoscreening app is designed to detect amblyopia risk factors (ARFs) in young children and includes remote review of images captured by smartphone. We aimed to evaluate the system's accuracy in detecting AAPOS guidelines ARFs. METHODS: Patients 6 months through 6 years of age at Medical University of South Carolina were recruited for this prospective study. Presence of age-specific ARF was determined based on a complete ophthalmic examination and compared with the GoCheck Kids recommendations. RESULTS: A total of 244 children were included (average age, 42 months; 51% male). Sensitivity of the GoCheck Kids iPhone with remote review to detect ARFs was 90.5%; specificity, 68.1%; positive predictive value, 56.8%; negative predictive value, 94.0%. Two of the 7 false negative results had hyperopia of >4 D, 4 had astigmatism, and 1 had anisometropia. Remote review of all images improved sensitivity. CONCLUSIONS: The GoCheck Kids app had good sensitivity and adequate specificity in detecting AAPOS ARFs in our enriched cohort of young children.

Retinopathy of prematurity: screening and treatment in Costa Rica.

AIM: To determine the recent demographic data, risk factors and results of retinopathy of prematurity (ROP) screening and treatment in Costa Rica. METHODS: The medical records of all preterm infants meeting ROP screening criteria (≤34 weeks' gestational age (GA) or birth weight (BW) ≤1750g, and those determined at risk by neonatologists) in the national healthcare system, Costa Rica, January 2010-December 2014, were retrospectively reviewed. The numbers and percentages of infants with ROP, risk factors, percentage of patients treated and treatment outcomes were determined. Comparison is made with screening criteria and literature reports of ROP incidence in other countries. RESULTS: The study population included 3018 preterm infants. Overall, 585 patients (585/3018, 19.4%) were found to have ROP. Of these, 15.4% (90 patients) required laser treatment, and 53% of those requiring treatment had BW <1000g. Five babies requiring treatment were ≥32 weeks' GA but with BW ≤1750g. Aggressive posterior disease was found in nine patients, and two infants of those screened (2/3018, 0.07%) suffered severe visual impairment during the 5-year study period. CONCLUSION: We provide comprehensive data of ROP care in Costa Rica allowing assessment and comparison of screening criteria and protocol.

Detecting High Hyperopia: The Plus Lens Test and the Spot Vision Screener.

PURPOSE: To evaluate the usefulness of the Plus Lens (Goodlite Company, Elgin, IL) test and the Spot Vision Screener (Welch Allyn, Skaneateles Falls, NY) in detecting high hyperopia in a pediatric population. METHODS: Between June and August 2015, patients were screened with the Spot Vision Screener and the Plus Lens test prior to a scheduled pediatric ophthalmology visit. The following data were analyzed: demographic data, Plus Lens result, Spot Vision Screener result, cycloplegic refraction, and examination findings. Sensitivity/specificity and positive/negative predictive values were calculated for the Plus Lens test and Spot Vision Screener in detecting hyperopia as determined by the "gold-standard" cycloplegic refraction. RESULTS: A total of 109 children (average age: 82 months) were included. Compared to the ophthalmologist's cycloplegic refraction, the Spot Vision Screener sensitivity for +3.50 diopters (D) hyperopia was 31.25% and the specificity was 100%. The Plus Lens sensitivity for +3.50 D hyperopia was 43.75% and the specificity was 89.25%. Spot Vision Screener sensitivity increased with higher degrees of hyperopia. CONCLUSIONS: In this preliminary study, the Plus Lens test and the Spot Vision Screener demonstrated moderate sensitivity with good specificity in detecting high hyperopia. [J Pediatr Ophthalmol Strabismus. 2017;54(3):163-167.].

Prospective Evaluation of Photoscreeners in the Pseudophakic Eyes of Children.

PURPOSE: To compare refractive data of Plusoptix AO9 (Plusoptix, Inc., Atlanta, GA) and Spot (Welch Allyn, Skaneateles Falls, NY) photoscreeners with retinoscopy in pseudophakic eyes of children. METHODS: In this prospective study, patients underwent testing with the Plusoptix and Spot photoscreeners prior to their examination by a pediatric ophthalmologist masked to the results of both photoscreeners. Data including testability and estimated refractions were entered into a Research Electronic Data Capture database for statistical analysis. For bilateral pseudophakia, one randomly selected eye was included in the analysis. RESULTS: Forty-four eyes were included, with a mean age of 7.4 ± 4.8 years. Refraction was estimated in all eyes using retinoscopy, but a refractive estimate was obtained in only 11.3% (5 of 44) of eyes using the Plusoptix binocularly and 63.6% (28 of 44) of eyes using the Spot. Compared to retinoscopy, the Plusoptix showed a mean difference of -0.80, 0.10, and -0.75 diopters (D) for sphere, cylinder, and spherical equivalent, respectively (P > .05). Comparable values for the Spot were 0.18, -0.26, and 0.05 D, respectively (P > .05). Compared to retinoscopy, the Plusoptix showed a mean absolute difference of 1.30, 0.80, and 1.25 D for sphere, cylinder, and spherical equivalent, respectively (P > .05). Similar values for the Spot were 1.38, 0.88, and 0.97 D, respectively (P > .05). CONCLUSIONS: Refraction was estimated in a higher number of pseudophakic eyes of children using the Spot as compared to the Plusoptix. The Spot may be useful to evaluate myopic shift in pseudophakic eyes of children. [J Pediatr Ophthalmol Strabismus. 2016;53(3):146-149.].

Detection of strabismus by the Spot Vision Screener.

BACKGROUND: The Spot Vision Screener, a new automated screener designed for use in schools and pediatric offices, refers children for strabismus based on the device's measurement of "gaze." We sought to evaluate its ability to detect manifest strabismus of ≥8(Δ) in primary position, which criteria is specified by the 2013 guidelines of the American Association for Pediatric Ophthalmology and Strabismus for detection of amblyopia risk factors by automated vision screeners. METHODS: In this prospective study, new and returning patients seen in the pediatric ophthalmology clinic underwent screening with the Spot Vision Screener (version 1.1.51) prior to a complete examination by a pediatric ophthalmologist. A Research Electronic Data Capture database was created for results of Spot referral status and examination. Patients who were referred by the Spot for gaze were included as test positives. Patients found to have constant strabismus of ≥8(Δ) in primary position by the physician were considered referral positive. RESULTS: A total of 444 children (average age, 72 months; range, 11-221 months) were included. Of these, 93 (20.9%) met the referral-positive threshold for strabismus criteria on examination by the pediatric ophthalmologist. The sensitivity of the Spot to detect AAPOS-threshold strabismus was 77.17%; the specificity, 93.73%. Positive predictive value was 76.34%; negative predictive value, 94.0%. CONCLUSIONS: The Spot demonstrates good sensitivity and excellent specificity for detecting AAPOS-threshold strabismus.

Evaluation of the Spot Vision Screener in young children in Costa Rica.

BACKGROUND: The Spot Vision Screener has demonstrated good sensitivity and specificity in the pediatric ophthalmology clinic setting. We sought to evaluate the updated Spot (version 2.0.16) in a general pediatric population through a collaboration of the Storm Eye Institute of the Medical University of South Carolina, the Clinica Dr Clorito Picado, and National Children's Hospital of Costa Rica. We compared results of screening with the Spot and pediatric ophthalmologic examination and determined sensitivity and specificity of the Spot in detecting amblyogenic risk factors (ARFs) according to the 2013 AAPOS Vision Screening Committee guidelines for automated vision screeners. METHODS: Children were screened with the Spot followed by a pediatric ophthalmologic examination. Cycloplegic refraction and motility findings were analyzed by age group to determine ARFs. RESULTS: A total of 219 subjects, averaging 60 months of age (range, 20-119 mo) were included. The prevalence of ARFs in our population was 12.3% (27/219). The most common risk factor was astigmatism, with a prevalence of 8.7% (19/219). The Spot referred 43 children (19.6%). Sensitivity of the Spot was 92.6%; specificity, 90.6%. The positive predictive value was 58.1%; the negative predictive value, 98.9%. CONCLUSIONS: The Spot demonstrated good sensitivity and specificity in detecting amblyopia risk factors in this general pediatric population.

Combining automated vision screening with on-site examinations in 23 schools: ReFocus on Children Program 2012 to 2013.

PURPOSE: The Association for the Blind and Visually Impaired (ABVI) began the ReFocus on Children Program to assist school nurses in providing vision screening for at-risk children in the Charleston County School District in South Carolina. METHODS: In 2012 to 2013, 2,750 low-income school children ages 3 to 5 years were screened using the Plusoptix Vision screener (Atlanta, GA). Further examinations were performed on 419 (56%) children referred and glasses prescribed and provided for 192 children (positive predictive value 46%). In 2013, teacher feedback questionnaires were sent to the 23 schools. RESULTS: Teacher feedback questionnaires had a 49% response rate. Of teachers responding to the questionnaire, 70% reported the children liked wearing their glasses. Teachers provided observations of positive impact, including improved academic performance. CONCLUSIONS: Nurses appreciated that the entire process was efficient and completed in school, simplifying care and follow-up. The authors encourage partnerships between schools, nonprofit agencies, and healthcare providers to improve screening and access to comprehensive vision care for young children.

The effectiveness of the Spot Vision Screener in detecting amblyopia risk factors.

PURPOSE: To evaluate the updated Spot Vision Screener (PediaVision, Welch Allyn, Skaneateles Falls, NY) in detecting amblyopia risk factors using 2013 guidelines of American Association for Pediatric Ophthalmology and Strabismus (AAPOS). METHODS: In this prospective study, patients seen from June 2012 to November 2013 were tested with the Spot prior to examination by a pediatric ophthalmologist who was masked to test results. The following data were analyzed: age, subject testability, examination findings, and systemic and ocular pathology. Children were divided into three age groups to determine gold standard results according to the AAPOS guidelines. RESULTS: A total of 444 children (average age, 72 months) were included. Compared to the ophthalmologist's examination, the Spot sensitivity was 87.7% and the specificity was 75.9% in detecting amblyopia risk factors. Sensitivity did not differ significantly between age groups, although the positive predictive value improved in the older age groups. CONCLUSIONS: In our study cohort, the Spot provided good specificity and sensitivity in detecting amblyopia risk factors according 2013 AAPOS criteria, with minor improvements with updated versions.

Photoscreeners in the pediatric eye office: compared testability and refractions on high-risk children.

PURPOSE: To compare refractive data and testability of Spot (PediaVision) and Plusoptix A09 (Plusoptix, Inc) photoscreeners and to compare each device with traditional cycloplegic retinoscopy. DESIGN: Prospective, interventional case series. METHODS: After informed consent, patients underwent testing with the Spot and Plusoptix photoscreeners before their examination by a pediatric ophthalmologist masked to the results. Data including testability and estimated refractions were entered into a Research Electronic Data Capture database for statistical analysis. RESULTS: A total of 265 children were enrolled (mean age, 6.0 ± 3.4 years). Both devices produced a computer printout result in 250 (94.3%) of the patients. The Spot photoscreener provided a refractive estimate in all computer printouts, whereas the Plusoptix, used binocularly, provided a refractive estimate in 75.2% (188/250) of the printouts. Compared with cycloplegic retinoscopy, both devices underestimated hyperopia or overestimated myopia (-1.35 diopters [D] and -0.64 D, Spot and Plusoptix, respectively) and overestimated astigmatism (0.36 D and 0.32 D, Spot and Plusoptix, respectively). The intraclass correlation coefficient for spherical equivalents indicated good agreement between cycloplegic retinoscopy and Spot (0.806) and excellent agreement between cycloplegic retinoscopy and Plusoptix (0.898). CONCLUSIONS: The Spot photoscreener provided refractive data on a greater percentage of children. The photorefractors correlated with cycloplegic retinoscopy refractive findings for sphere and spherical equivalents, but underestimated hyperopia or overestimated myopia and overestimated astigmatism. The binocular refractions of Plusoptix agreed more closely with the refractions of our pediatric ophthalmologists.

Cataracts and glaucoma in patients with oculocerebrorenal syndrome.

BACKGROUND: Oculocerebrorenal syndrome is an X-linked recessive hereditary oculocerebrorenal disorder characterized by congenital cataract, mental retardation, and Fanconi syndrome of the proximal renal tubules. Other ocular findings include glaucoma, corneal opacity (keloid), enophthalmos, and hypotonia. OBJECTIVE: To describe the treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. Method Retrospective review. RESULTS: Seven patients with oculocerebrorenal syndrome had visually significant bilateral cataracts detected on their first full ophthalmic examination. All underwent bilateral cataract surgery. The mean age (of 14 eyes) at cataract extraction was 1.25 (median, 1.1) months. Glaucoma diagnosis and treatment were more variable. The mean age (of 11 eyes) at glaucoma diagnosis was 24.1 (range, 0.2-70.0) months: the mean age was 0.2 month (of 4 eyes) when glaucoma was diagnosed before cataract extraction, and the mean age was 37.7 months (of 7 eyes) when glaucoma was diagnosed after cataract extraction. All eyes were followed up for a mean of 100.8 (range, 38-190) months. Main Outcome Measure Treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. CONCLUSIONS: Early identification and surgical removal of cataracts is recommended in patients with oculocerebrorenal syndrome. Despite this, visual acuity results will only rarely be better than 20/70, and nystagmus is likely. Patients should be monitored closely and regularly for changes in intraocular pressure, optic nerve cupping, and refractive error to rule out the development of glaucoma.