Prescribing patterns for paediatric hyperopia among paediatric eye care providers.

PURPOSE: To survey paediatric eye care providers to identify current patterns of prescribing for hyperopia. METHODS: Paediatric eye care providers were invited, via email, to participate in a survey to evaluate current age-based refractive error prescribing practices. Questions were designed to determine which factors may influence the survey participant's prescribing pattern (e.g., patient's age, magnitude of hyperopia, patient's symptoms, heterophoria and stereopsis) and if the providers were to prescribe, how much hyperopic correction would they prescribe (e.g., full or partial prescription). The response distributions by profession (optometry and ophthalmology) were compared using the Kolmogorov-Smirnov cumulative distribution function test. RESULTS: Responses were submitted by 738 participants regarding how they prescribe for their hyperopic patients. Most providers within each profession considered similar clinical factors when prescribing. The percentages of optometrists and ophthalmologists who reported considering the factor often differed significantly. Factors considered similarly by both optometrists and ophthalmologists were the presence of symptoms (98.0%, p = 0.14), presence of astigmatism and/or anisometropia (97.5%, p = 0.06) and the possibility of teasing (8.3%, p = 0.49). A wide range of prescribing was observed within each profession, with some providers reporting that they would prescribe for low levels of hyperopia while others reported that they would never prescribe. When prescribing for bilateral hyperopia in children with age-normal visual acuity and no manifest deviation or symptoms, the threshold for prescribing decreased with age for both professions, with ophthalmologists typically prescribing 1.5-2 D less than optometrists. The threshold for prescribing also decreased for both optometrists and ophthalmologists when children had associated clinical factors (e.g., esophoria or reduced near visual function). Optometrists and ophthalmologists most commonly prescribed based on cycloplegic refraction, although optometrists most commonly prescribed based on both the manifest and cycloplegic refraction for children ≥7 years. CONCLUSION: Prescribing patterns for paediatric hyperopia vary significantly among eye care providers.

A high prevalence of biallelic RPE65 mutations in Costa Rican children with Leber congenital amaurosis and early-onset retinal dystrophy.

BACKGROUND: Leber congenital amaurosis (LCA) and early-onset retinal dystrophy (EORD), are primary causes of inherited childhood blindness. Both are autosomal recessive diseases, with mutations in more than 25 genes explaining approximately ~70% of cases. However, the genetic cause for many cases remains unclear. Sequencing studies from genetically isolated populations with increased prevalence of a disorder has proven useful for rare variant studies, making Costa Rica an ideal place to study LCA/EORD genetics. MATERIALS AND METHODS: Twenty-eight affected children (25 LCA, three EORD) and their immediate family members, totaling 52 individuals (30 affected) from 22 families, were sequenced. Whole exome sequencing was performed on all affected individuals. Available parents were analyzed either by whole exome sequencing (WES) or Sanger sequencing to determine transmission. RESULTS: All affected individuals demonstrated compound heterozygous or homozygous mutations in known Inherited Retinal Disease (IRD) associated genes. Twelve variants were identified in at least one individual in three genes, RDH12, RPE65, and USH2A. Four recurrent RPE65 mutations were observed in 97% of individuals and 95% of families. All patients with LCA and two of the three individuals with EORD had biallelic mutations in RPE65; one child with EORD had a homozygous RDH12 mutation. CONCLUSIONS: These data suggest that the majority of LCA/EORD in Costa Rica is due to four founder mutations in RPE65 which have been maintained in this genetically isolated population. This finding is of great clinical significance due to the availability of gene therapy recently approved in the US and European Union for patients with biallelic RPE65 defects.

Effectiveness of the GoCheck Kids Vision Screener in Detecting Amblyopia Risk Factors.

PURPOSE: The GoCheck Kids smartphone photoscreening app (Gobiquity Mobile Health, Scottsdale, Arizona, USA), introduced in 2014, is marketed to pediatricians with little published validation. We wished to evaluate the GoCheck Kids Screener for accuracy in detecting amblyopia risk factors (ARF) using 2013 American Association for Pediatric Ophthalmology and Strabismus guidelines. DESIGN: Validity assessment. METHODS: Children 6 months to 6 years of age presenting from October 2016 to August 2017 were included. Children were screened with the GoCheck preloaded Nokia Lumia 1020, software version 4.6 with image processing version R4d, prior to undergoing a comprehensive eye examination by a pediatric ophthalmologist masked to the screener results. Determination of the presence of age-specific ARF was made based upon the examination and compared with the GoCheck recommendation. RESULTS: A total of 206 children were included (average age 43 months). When compared to examination, GoCheck had a sensitivity of 76.0% and specificity of 67.2% in detecting ARF. Positive predictive value was 57.0% and negative predictive value 83.0%. The screener results of 13 children were changed from "no risk factors" to "risk factors identified" based on the GoCheck remote review process. Four images remained "not gradable" and screening was unsuccessful in 3 children. CONCLUSION: In our high-risk population, this version of the Gocheck Kids smartphone app was useful in identifying ARF in children who are often not able to cooperate with visual acuity testing. This study informs pediatricians about the efficacy of this new screener as they make decisions about how to best detect vision problems in young children.

Use of the Spot Vision Screener for patients with developmental disability.

PURPOSE: To determine whether the Spot Vision Screener effectively detects amblyopia risk factors (ARFs) in patients with developmental disability using the 2013 guidelines of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS). METHODS: Children with developmental disability presenting for complete pediatric ophthalmologic examination were prospectively enrolled between June 2012 and March 2016. The following data were analyzed: presence of ARFs according to the AAPOS guidelines, refraction, eye alignment, and other ocular pathology. RESULTS: A total of 100 children (average age, 5.7 years; range, 2.2- 9.2 years) were included. The prevalence of ARFs in this cohort was 38%. The sensitivity of the Spot Vision Screener in detecting amblyopia risk factors was 84%; the specificity, 62%. The positive predictive value was 58%; the negative predictive value, 86%. CONCLUSIONS: In our study cohort the Spot Vision Screener provided good sensitivity and adequate specificity for a screening examination. Automated screeners may be useful in screening children with developmental disability.

Performance of the Spot Vision Screener in Children Younger Than 3 Years of Age.

PURPOSE: To evaluate the use of the Spot Vision Screener (Spot; Welch Allyn, Skaneateles Falls, New York, USA) for detection of amblyopia risk factors in children aged 6 months to 3 years, as defined by the 2013 guidelines of the American Association for Pediatric Ophthalmology and Strabismus. DESIGN: Reliability analysis. METHODS: In this study, children seen from June 1, 2012, to April 30, 2016 were tested with the Spot during a routine visit. Enrolled children underwent a comprehensive eye examination including cycloplegic refraction and sensorimotor testing within 6 months of the testing date by a pediatric ophthalmologist masked to the Spot results. RESULTS: A total of 184 children were included. The Spot successfully obtained readings in 89.7% of patients. Compared with the ophthalmologist's examination, the Spot had an overall sensitivity of 89.8% and a specificity of 70.4%. CONCLUSION: The Spot achieved good sensitivity and specificity for detection of amblyopia risk factors in this young cohort, particularly in the older subgroup. Our data offer support for automated vision screening in young children.

A pilot study evaluating the use of EyeSpy video game software to perform vision screening in school-aged children.

PURPOSE: To compare the vision-screening results of school-aged children tested with EyeSpy software and those of children examined by a pediatric ophthalmologist. We also compared combined results of an electronic visual acuity (EVA) tester and stereopsis testing to the results of a professional eye examination. METHODS: In this pilot study, all children were tested with an EyeSpy and ETDRS EVA tester, followed by ocular examination including stereopsis assessment and cyclopegic refraction. The order of presentation of the EVA and EyeSpy assessments was assigned randomly. The EyeSpy test was performed twice (with an occlusive eyepatch and red-blue dissociative goggles). EyeSpy registered pass or refer results for visual acuity testing at a threshold of 20/32 visual acuity and stereopsis of 300 arcsec. Similar threshold values were used in the EVA/stereopsis testing. RESULTS: The average age of 72 subjects was 11.4 +/- 2.2 years. Prevalence of visual impairment was 25 (34.7%) of 72 as reported by the professional examination. The sensitivity, specificity, and conventional positive likelihood ratio were 88%, 87%, and 6.8 when EyeSpy was used with a patch; 88%, 74%, and 3.44 when EyeSpy was used with goggles; and 88%, 94%, and 13.79 for EVA/stereospsis, respectively, compared with the gold-standard professional eye examination. EyeSpy screening results using a patch were not significantly different than those of a professional examination (p = 0.508). The 2 results concurred in 63 (87.5%) of 72 subjects. CONCLUSIONS: EyeSpy software has potential for use as a vision-screening device. The use of EyeSpy with an occlusive patch outperformed EyeSpy with dissociative glasses.

New techniques and technologies for pediatric cataract surgery.

PURPOSE OF REVIEW: This paper surveys the literature from April 2004 to April 2005 and addresses trends in pediatric cataract surgery techniques. RECENT FINDINGS: The evolution of pediatric cataract surgery continues, with many adult cataract surgical techniques being applied to children with minor technical adjustments. More and more, surgeons are implanting intraocular lenses in younger children. One of the most widely reported technologic advances in 2004-2005 was the use of hydrophobic acrylic intraocular lenses in children. Predicting axial growth, and refractive change that accompanies it, is one of the major remaining challenges for the long-term care of children who have had cataract surgery. SUMMARY: Automation and intraocular lenses have helped to provide better anatomic and functional outcome for cataract surgery in children. Two major challenges in the coming years will be to find a way to obviate invasive procedures like posterior capsulotomy and vitrectomy and to study the growth of eyes in a well designed prospective study that will help to design new intraocular lens power calculation formulas specifically suited for children's eyes.

Interocular axial length difference in eyes with pediatric cataracts.

PURPOSE: We sought to report interocular axial length difference (IALD) between the operated eye and fellow eye at the time of pediatric cataract surgery. METHODS: A retrospective analysis of 171 cataractous (operated) eyes (mean patient age at surgery, 56.9 months; range, 0.2-230.0) was undertaken. Descriptive statistics, analysis of covariance, and regression analysis were used in statistical analysis. RESULTS: The mean IALD was 0.05 +/- 1.15 mm. If absolute values were used, mean IALD was 0.76 +/- 0.86. Nearly half of the subjects (48.0%) showed an IALD of >/=0.5 mm. Forty-one patients (24.0%) had operated (cataractous) eyes that were >/=0.5 mm shorter than the fellow eye, and 41 patients (24.0%) had operated eyes that were >/=0.5 mm longer than the fellow eye. The mean IALD in unilateral and bilateral cases was 0.10 +/- 1.33 and -0.03 +/- 0.76, respectively (P = 0.4). If absolute values were used, it was 0.98 +/- 0.90 and 0.40 +/- 0.65 in unilateral and bilateral cases, respectively (P < 0.001). IALD was more predictable in unilateral than in bilateral cases (P < 0.001). In unilateral cases, age at surgery (P = 0.04) and AL of the operated eye (P < 0.001) correlated with IALD. IALD did not correlate with gender, ethnicity, or type of cataract in either unilateral or bilateral cases. CONCLUSIONS: An IALD of >/=0.5 mm occurred in 48.0% of patients with pediatric cataracts and occurred more often with unilateral cataractous patients than with bilateral cases. In patients with unilateral cataract, age at surgery and AL of the operated eye could help predict the IALD.

Opacification of the visual axis after cataract surgery and single acrylic intraocular lens implantation in the first year of life.

PURPOSE: To report the incidence and risk factors for secondary surgical intervention to treat visual axis opacification (VAO) after cataract surgery and acrylic intraocular lens (IOL) implantation during the first year of life. METHODS: A retrospective review of 29 eyes of 20 patients receiving a hydrophobic acrylic (AcrySof; Alcon, Fort Worth, TX) IOL implantation was conducted. All eyes underwent primary posterior capsulectomy and anterior vitrectomy. Statistical analysis was performed using SPSS for Windows (SPSS, Chicago, IL). RESULTS: Average age at surgery was 4.8 +/- 3.7 months, and average follow-up was 33.4 +/- 16.1 months. Eleven of 29 (37.9%) eyes developed VAO requiring secondary surgical intervention at a median of 4.8 months (95% confidence interval 3.4 to 6.2). Average age at surgery for eyes that subsequently opacified was 3.8 +/- 3.0 months compared with 5.4 +/- 4.0 months for those whose visual axis remained clear (P = 0.26). The relative risk of subsequent VAO surgery was 2.7 for primary surgery performed at or before the first 6 months of life. Opacification was significantly related to eyes with associated ocular anomalies (eg, anterior segment dysgenesis, iris hypoplasia, or persistent fetal vasculature) with a relative risk of 8.6 (P < 0.001). Proliferation of cortex was the most common form of VAO, followed by mixed-type with predominantly fibrous, fibrous alone, or Elschnig pearls. When secondary surgery was required, it occurred primarily during the first 6 months (ie, 9 of 11 patients) after the initial cataract surgery. CONCLUSIONS: When cataract and IOL surgery was undertaken within the first year of life, a secondary surgical procedure was required in 37.9% of eyes to maintain a clear visual axis. Most secondary surgery for VAO occurred within the first 6 months after surgery. Postoperative opacification was most common in eyes with associated ocular anomalies.

Clinical outcomes of pars plana capsulotomy with anterior vitrectomy in pediatric cataract surgery.

PURPOSE: The management of the posterior capsule in pediatric cataract surgery remains challenging. The purpose of our study was to evaluate the role and clinical outcomes of primary pars plana capsulotomy and pars plana anterior vitrectomy during pediatric cataract surgery with intraocular lens (IOL) implantation. METHODS: Consecutive cases of pars plana capsulotomy and anterior vitrectomy during pediatric cataract surgery were retrospectively reviewed. The surgical technique was the same in all patients and involved pars plana capsulotomy with anterior vitrectomy after the IOL was implanted in the capsular bag. RESULTS: Seventy-six pediatric cataract cases in 61 patients performed from 1994 through 1999 used the pars plana posterior capsulotomy technique and were included in this analysis. A posterior chamber IOL was implanted in all cases. There were no intraoperative complications associated with the surgery. Median age at the time of surgery was 21 months. All patients had at least 1 year of follow-up and mean follow-up was 29.4 months. Among patients old enough to read Snellen's letters (53 eyes), final best-corrected visual acuity was 20/40 or better in 32 eyes (60%) and 20/50 to 20/200 in 21 eyes (40%). Poor final visual acuity was associated with deprivation amblyopia or optic nerve dysplasia or hypoplasia. There were no postoperative retinal complications or cases of IOL dislocation during the follow-up period. Seven eyes (9.2%) of five patients with a median age of 2.5 months developed reopacification of the visual axis at a mean of 4.1 months postoperatively. This event occurred in 6 of 28 eyes with an age of 6 months or less (21.4%), versus only 1 of 48 eyes with an age greater than 6 months (2.0%) (P =.006, Fisher exact test). The visual axis was restored by pars plana membranectomy in 6 eyes and 1 eye of one patient underwent neodymium:YAG laser membrane discission. CONCLUSIONS: Pars plana capsulotomy with pars plana anterior vitrectomy is a safe, effective method of managing the posterior capsule in pediatric cataract surgery with IOL implantation. Visual axis reopacification is associated with a very young age at the time of surgery. Continued follow-up of these patients is important to assess the long-term outcomes of this surgical approach.