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.

Presentation of TRPM1-Associated Congenital Stationary Night Blindness in Children.

Importance: Congenital stationary night blindness (CSNB) implies a stable condition, with the major symptom being nyctalopia present at birth. Pediatric clinical presentation and the course of different genetic subtypes of CSNB have not, to our knowledge, been well described in the era of molecular genetic diagnosis. Objective: To describe the presentation and longitudinal clinical characteristics of pediatric patients with molecularly confirmed TRPM1-associated complete CSNB (cCSNB). Design, Setting, Participants: This study was conducted at the University of Iowa from January 1, 1990, to July 1, 2015, and was a retrospective, longitudinal case series of 7 children (5 [71.4%] female) with TRPM1-associated cCSNB followed up for a mean (SD) of 11.1 (2.8) years. Main Outcomes and Measures: History, ophthalmologic examination findings, full-field electroretinogram (ffERG) results, full-field stimulus threshold testing results, Goldmann visual field results, optical coherence tomography results, and molecular genetic results were evaluated. Presenting symptoms and signs, the correlation of refractive error with electroretinography, and clinical evolution were analyzed. Results: Seven patients (5 [71.4%] female) presented early in childhood with strabismus (n = 6 [86%]), myopia (n = 5 [71%]), and/or nystagmus (n = 3 [43%]). The mean (SD) age at presentation was 8 (4) months and for receiving a diagnosis by ffERG was 7.3 years, with molecular diagnosis at 9.7 years. The mean (SD) length of follow-up was 11 (2.8) years. The best-corrected visual acuity at the most recent visit averaged 20/30 in the better-seeing eye (range, 20/20-20/60). The mean (SD) initial refraction was -2.80 (4.42) diopters (D) and the mean refraction at the most recent visit was -8.75 (3.53) D (range, -4.00 to -13.75 D), with the greatest rate of myopic shift before age 5 years. Full-field electroretinogram results were electronegative, consistent with cCSNB, without a significant change in amplitude over time. No patient or parent noted night blindness at presentation; however, subjective nyctalopia was eventually reported in 5 of 7 patients (71%). The full-field stimulus threshold testing results were moderately subnormal (-29.7 [3.8] dB; normal -59.8 [4.0] dB). Goldmann visual field results were significant for full I-4e, but constricted I-2e isopter. Eight different mutations or rare variants in TRPM1 predicted to be pathogenic were detected, with 3 novel variants. Conclusions and Relevance: Children with TRPM1-associated cCSNB presented before school age with progressive myopia as well as strabismus and nystagmus (but not nyctalopia), with stable, electronegative ffERG results, mildly subnormal full-field stimulus threshold testing results, and a constricted I2e isopter on perimetry. These findings suggest that ffERG and cCSNB genetic testing should be considered for children who present with early-onset myopia, especially in the presence of strabismus and/or nystagmus, and that TRPM1-associated cCSNB is a channelopathy that may present without complaints of night blindness in childhood.

Use of a Supplemental Oxygen Protocol to Suppress Progression of Retinopathy of Prematurity.

Purpose: To compare progression of retinopathy of prematurity (ROP) before and after institution of an oxygen therapy protocol to inhibit active proliferation and progression of ROP in premature infants. Methods: A retrospective cohort study was performed of premature infants undergoing ROP screening before (cohort A) and after (cohort B) implementation of an oxygen therapy protocol to inhibit further progression for those with stage 2 ROP or worse. Statistical analysis with χ2, Fisher's exact test, or Wilcoxon rank sum test was performed; and logistic regression models were created to determine the odds ratio of cohort B developing ROP progression beyond stage 2, compared to cohort A, adjusting for other risk factors for ROP. Results: In cohort A, without oxygen therapy protocol (2002-2007), 44% (54/122) of infants progressed beyond stage 2, compared to 23% (24/103) of infants after protocol implementation (cohort B, 2008-2012) (P = 0.001). No significant differences between cohort A and B were found for gestational age, birth weight, survival, sepsis, bronchopulmonary dysplasia, oxygen at discharge, or need for diuretics. Infants with stage 2 ROP in cohort B, with oxygen therapy protocol, had significantly decreased risk of ROP beyond stage 2 (odds ratio 0.37, 95% confidence interval 0.20-0.67; P = 0.0013), compared to cohort A, correcting for differences in birth weight and necrotizing enterocolitis. Conclusions: Progression from stage 2 to stage 3 ROP in premature infants was significantly decreased after implementation of an oxygen therapy protocol, without a corresponding increase in pulmonary morbidity. This study suggests that appropriate oxygen therapy may play a role in inhibiting progression of stage 2 ROP, potentially decreasing the risk of lifelong visual loss in this vulnerable population.

Description and validation of a structured simulation curriculum for strabismus surgery.

PURPOSE: To describe and validate a Web-based structured simulation curriculum of strabismus surgery for residents in training using noncadaveric eye models. METHODS: A pre- and posttest of cognitive skills, objective wet laboratory structured assessment of technique, and summative global evaluation form were implemented as part of a systematic ophthalmology wet lab (OWL) curriculum. Strabismus techniques were taught using a structured simulation method on noncadaveric models of eyes. Likert scale questionnaires were administered to assess the comfort level with strabismus surgery of residents before and after participation in the wet laboratory. Statistical analysis was performed using the paired t test. RESULTS: Seven residents participated in the curriculum. Average test scores improved from 65% to 91% (P = 0.0002). Resident comfort level improved from an average score (assessed by Likert scale of 1-5) with standard deviation of 2.6 ± 1.0 to 4.3 ± 0.5 for passing scleral sutures (P = 0.0008), of 2.6 ± 0.5 to 4.3 ± 0.5 for isolating and suturing muscles (P = 0.00004), and of 2.7 ± 1.0 to 4.6 ± 0.5 for comfort with naming instruments (P = 0.0007). CONCLUSIONS: In this study of 7 residents, knowledge and comfort level with strabismus surgery statistically improved with a structured simulation curriculum.

Validity of a layperson-administered Web-based vision screening test.

PURPOSE: To determine the sensitivity and specificity of the Web-based vision-screening test (WBT) VisionForKids.org when administered by an untrained layperson in a controlled environment. METHODS: Visual acuities were obtained by an untrained layperson using the WBT under observation and by an ophthalmic professional using the Electronic Visual Acuity (EVA) Tester. Subjects were randomized to which method was used first. Subjects were considered to have failed by the following criteria: in children <48 months, 20/50 vision; in children between 48 and 59 months, 20/40 or worse; in children ≥60 months, 20/30 or worse. The results of the vision examinations by the WBT and the EVA Tester were compared; sensitivity and specificity of the WBT and the correlation coefficient to detect normal and abnormal vision compared to EVA testing were determined. RESULTS: Visual acuities were obtained on 203 children between ages 3 and 12 years (average age, 7.4 years). Sensitivity of the WBT was 78.7% (95% CI, 66.0%-87.7%), specificity was 89.4% (95% CI, 82.9%-93.8%). The correlation coefficient of EVA testing with the WBT was 0.89 (P < 0.001). CONCLUSIONS: This layperson-administered WBT, VisionForKids.org, is valid for identifying amblyopia in a controlled environment, making it possible for cost-effective and easily accessible vision screening to be performed by laypersons.

Cataract surgery in children with uveitis: retrospective analysis of intraocular lens implantation with anterior optic capture.

PURPOSE: To present experience with cataract extraction in 9 eyes of 7 pediatric patients with chronic uveitis and compare the technique of anterior optic capture in 5 eyes that underwent cataract extraction without optic capture of the intraocular lens (IOL) or were left aphakic. METHODS: A retrospective review of pediatric patients with chronic uveitis undergoing cataract surgery was performed, examining the preoperative and postoperative visual acuity, immunosuppressive therapy, surgical technique, complications, subsequent procedures, and need for escalation of systemic immunosuppressive therapy. The technique of anterior optic capture is described in detail. RESULTS: Of the 9 eyes, 5 underwent cataract extraction with IOL placement with the haptics in the capsular bag and optic prolapsed through the anterior capsulorhexis. One eye underwent cataract extraction with IOL implantation in the bag. Three eyes had lensectomy without IOL placement. The eyes with anterior optic capture had no adverse outcomes and uveitis flares were controlled with topical medications and systemic immunosuppressants; the eye with IOL placement without optic capture had recurrent membranes and uveitis flares, necessitating increased systemic immunosuppression. All eyes achieved best-corrected visual acuity of 20/60 or better by 6 months following surgery and 20/30 or better at the most recent follow-up. CONCLUSIONS: The technique of cataract extraction with IOL placement and anterior prolapse of the optic through the anterior capsulorhexis shows promise to be a safe and viable option for pediatric patients with chronic uveitis treated with systemic immunotherapy.

Quantitative measurement of retinal hemorrhages in suspected victims of child abuse.

PURPOSE: Child abuse is one of the leading causes of death in early childhood. The presence of retinal hemorrhages often supports the diagnosis. The purpose of this study was to determine whether the specific measurement of retinal hemorrhages when present on fundus photography correlates with other clinical findings typically seen in children suspected of having been abused. METHODS: The medical records of children with retinal hemorrhages who were suspected of being victims of abusive head trauma from June 2003 to June 2013 and who had widefield retinal photography performed were retrospectively reviewed. Data collected included hemorrhage-covered percentage (HCP) of the central retina (posterior pole or 40° circle centered on fovea) measured by ImageJ in relation to death, length of hospital stay, presence of abnormal findings on neuroimaging or skeletal survey, and definite versus possible abuse. RESULTS: Significant difference in retinal hemorrhage measured on fundus photography was found in patients with axial skeletal fracture (P = 0.016), signs of severe brain trauma on neuroimaging (P = 0.014) and definite versus possible abuse (P = 0.023). No correlation of quantitative measurement of the retinal hemorrhage to length of hospital stay, death, or the presence of skull fracture was found in this cohort. CONCLUSIONS: The quantitative measurement of total retinal hemorrhage when present on fundus photography centered on posterior pole in children suspected of having been abused correlated with some but not all findings typically seen in abused children.

Cryptic respiratory epithelial cyst of the lateral orbit in a 3 year old with strabismus and amblyopia.

Although choristomatous cystic orbital lesions are not uncommon in the pediatric population, these lesions rarely cause strabismus without being clinically obvious. Common choristomas, such as dermoid cysts and epidermoid cysts, usually cause vision loss through amblyopia or changes in refractive error. A 3-year-old girl with a history of esotropia and presumed lipodermoid who subsequently developed an abduction deficit and worsening of her visual acuity to 20/70 from a baseline of 20/50 is described. Magnetic resonance imaging of the orbits was performed, which showed a large cyst of the lateral orbit. On surgical removal, a developmental cyst lined with respiratory epithelium was identified and, based on its temporal location and history, characterized as a choristomatous lesion. Subsequently, the patient's visual acuity improved to 20/25 without patching. This patient had a larger than expected developmental cyst of respiratory epithelium that was originally thought to be a lipodermoid, contributing to her strabismus and amblyopia. Although orbital imaging is not routine management prior to strabismus surgery in the presence of a lipodermoid, it may be useful in cases when an abduction deficit is present or amblyopia treatment fails, because an underlying process may be responsible.

Primary peripheral retinal nonperfusion in a family with Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is a connective tissue disorder associated with aggressive arterial aneurysms; rarely, it can have clinical features similar to those of Marfan syndrome, with retinal detachment, myopia, and ectopia lentis. A 19-month-old boy with history of LDS was found to have peripheral retinal nonperfusion in both eyes and a combined traction and exudative retinal detachment of the left eye. Ocular findings in the father, who also had LDS, were normal, but the patient's 34-month-old sister with LDS was also found to have less extensive peripheral retinal nonperfusion. To our knowledge, this is the first report of LDS associated with peripheral retinal nonperfusion in siblings with the same LDS mutation.

Retinal hemorrhages in intubated pediatric intensive care patients.

PURPOSE: To define the diagnoses associated with the presence of retinal hemorrhages, to clarify the association between retinal hemorrhages, cardiopulmonary resuscitation (CPR), and coagulopathy, and to describe the type of retinal hemorrhages found in intubated critically ill patients <4 years of age. METHODS: This was a prospective observational study of intubated patients <4 years of age admitted to the pediatric intensive care unit of a tertiary care center from March 2010 to May 2012. The presence and description of retinal hemorrhages was recorded along with diagnoses, international normalized ratio (INR), CPR (in minutes), and mortality. RESULTS: A total of 85 intubated, critically ill patients (37 females) were included; average patient age was 8.2 months (range, 0.1-46.8 months). Of the 85 patients, 6 (7%) had retinal hemorrhages (95% CI, 0.029-0.153). Of the 6 patients with RH, abusive head trauma (AHT) was diagnosed in 4 patients, 1 patient had direct head trauma, and 1 had CPR. There were 8 patients with CPR out of the 85. AHT was highly associated with severe multilayered retinal hemorrhages (P = 0.0001) but coagulopathy (P = 0.2671) and CPR (P = 0.5342) were not. CONCLUSIONS: Severe multilayered retinal hemorrhages were associated with AHT in this cohort of patients. Without a history of trauma, retinal hemorrhages occurred in only 1 of 85 patients; in this case the hemorrhages were mild, confined to the posterior pole, and found only in the retinal layer.

Resident and fellow participation in strabismus surgery: effect of level of training and number of assistants on operative time and cost.

OBJECTIVE: To investigate the effect of the level of training and number of assistants on operative time for uncomplicated, 2-muscle, horizontal strabismus surgery at an academic institution. DESIGN: Comparative case series. PARTICIPANTS: A total of 993 children and adults between the ages of 6 months and 75 years. METHODS: Retrospective chart review of strabismus surgeries performed between July 1, 2008, and December 31, 2012, by any of 3 attending surgeons assisted by a resident in the postgraduate year 3 (PGY3), fellow in the postgraduate year 5 (PGY5), or both. MAIN OUTCOME MEASURES: Operative time (minutes) and associated operative cost (dollars). RESULTS: There were 373 cases with 1 assistant and 44 cases with 2 assistants. Of all cases with 1 assistant, there were 200 cases with a PGY3 assistant an average operative time of 62.5 minutes (standard deviation [SD], 15.1) and 173 cases with a PGY5 assistant an average operative time of 59.0 minutes (SD, 14.7); the difference of 3.5 minutes was statistically significant (P = 0.02). The average operative time for all cases with 2 assistants (both PGY3 and PGY5) was 10.6 minutes longer than all cases with 1 assistant (P = 0.0002). No statistically significant variation in operative times was demonstrated when comparing cases with a PGY3 (P = 0.29) and PGY5 (P = 0.44) assistant in their respective first and last halves of the academic year, but operative times within individual quarters of the academic year were significant for PGY3 (P = 0.03) but not for PGY5 (P = 0.24) assistant cases. Operative times were significantly different for individual PGY3 (P = 0.03) but not PGY5 (P = 0.22) assistant cases. Cost per PGY3 assistant per year for additional operative time is $3141.95. CONCLUSIONS: Operative time in strabismus surgery increased with PGY3 participation and further increased with both assistants over either assistant alone. Operative times earlier in the year did not vary from those later in the year for PGY3 or PGY5 assistants. The difference in quarterly and individual PGY3 but not PGY5 assistant operative times suggests that efficiency in strabismus surgery varies by assistants with less experience or interest.

Inadvertent intrastromal injection of ophthalmic viscoelastic.

Viscoelastic is an ophthalmic viscosurgical device used to protect ocular tissue and maintain intraocular space during surgical procedures such as cataract removal. To date, the only cases published regarding inadvertent viscosurgical device injection have caused Descemet's membrane detachments. We report the case of a 4-year-old girl who underwent complicated lensectomy with prior history of uveitis and posterior synechia in which intrastromal ophthalmic viscoelastic was inadvertently injected into the stroma, leaving an off-centered opacity. At the time of surgery, no Descemet's membrane detachment was seen. The lensectomy and planned anterior vitrectomy were performed without complication and visual acuity has improved from 20/200 preoperatively to 20/70 at 3 months' follow-up. The corneal opacity resolved within 1 week of surgery, with no evidence of residual visual impairment.

Transient leopard spot corneal endothelial staining with trypan blue during cataract surgery in a child with congenital rubella syndrome.

We report the complication of corneal endothelial staining with trypan blue that limited the surgical view during cataract extraction in a 10-month-old boy. The boy had presented with a pigmentary retinopathy, microphthalmia, and a dense, white, unilateral congenital cataract. He was suspected of having, and was later diagnosed with, congenital rubella syndrome. We hypothesize that the corneal staining may have resulted from virally induced corneal endothelial damage. To our knowledge, this is the first reported case of trypan blue adversely affecting congenital cataract surgery.

Nd:YAG laser capsulotomy under general anesthesia in the sitting position.

Neodymium-yttrium-aluminum-garnet (Nd:YAG) laser capsulotomy is commonly used to treat posterior capsule opacification after cataract surgery in adults. Children and adults with developmental delay, however, are not always cooperative, and the procedure must be performed under general anesthesia. We describe a technique for Nd:YAG capsulotomy under general anesthesia in the sitting position using the standard widely available Nd:YAG laser. Anesthesia was induced in the supine position and the patient was then transferred to the sitting position with chin in the slit-lamp laser delivery system. The procedure was performed without complications. We report our experience in 4 eyes of 3 patients, including 2 children. This technique is a viable option for those without access to an overhead-mounted Nd:YAG laser and an alternative to secondary posterior capsulotomy in selected patients.

Practical community photoscreening in very young children.

OBJECTIVE: Recent US Preventive Services Task Force recommendations on vision screening reported insufficient data to recommend vision screening in children <3 years of age. The Iowa photoscreening program, KidSight, has screened children from 6 months of age and older since 2000. We report our experience with vision screening in these children and compare the results of the photoscreens in children younger than 3 years with those of children of preschool age and older. METHODS: A retrospective review of results from the Iowa KidSight database using the MTI PhotoScreener containing results of children screened between May 1, 2000, and April 30, 2011. RESULTS: During the 11 years of the study, 210 695 photoscreens on children were performed at 13 750 sites. In the <3-year age group, the unreadable rate was 13.0%, the referral rate was 3.3%, and the overall positive-predictive value was 86.6%. In the 3- to 6-year-old children, the unreadable rate was 4.1%, the referral rate was 4.7%, and the overall positive-predictive value was 89.4%. CONCLUSIONS: No statistically significant difference was found in screening children from 1 to 3 years old compared with screening children >3 years old. These results confirm that early screening, before amblyopia is more pronounced, can reliably detect amblyogenic risk factors in children younger than 3 years of age, and we recommend initiation of photoscreening in children aged 1 year and older.

Effect of occlusion amblyopia after prescribed full-time occlusion on long-term visual acuity outcomes.

PURPOSE: To investigate the incidence and characteristics of occlusion amblyopia with prescribed full-time patching and determine its effect on long-term visual acuity outcomes. METHODS: The records of patients younger than 10 years diagnosed as having amblyopia between 1970 and 2000 were retrospectively reviewed. Patients were prescribed full-time occlusion and observed until completion of therapy. RESULTS: Of 597 patients treated for amblyopia by full-time patching, 115 were diagnosed as having occlusion amblyopia (19.3%). Seventy-five percent (86 of 115) developed occlusion amblyopia during the first episode of full-time patching. Occlusion amblyopia occurred more frequently in children prescribed full-time patching at an earlier age (P = .0002), with an odds ratio of 8.56 (95% confidence interval: 2.73, 26.84) in children younger than 36 months and 2.66 (95% confidence interval: 0.96, 7.37) in children between 36 and 59 months old. Seven of the patients with occlusion amblyopia did not reverse fixation and continued to fixate with the initially amblyopic eye after treatment. Final visual acuity in these eyes with occlusion amblyopia was 20/30 or better. After cessation of treatment, the final interocular difference in visual acuity was less in patients with a history of occlusion amblyopia (P = .003). CONCLUSION: Occlusion amblyopia occurred at all ages, but the incidence decreased with increasing age. Patients who developed occlusion amblyopia with prescribed full-time occlusion had less interocular visual acuity difference than patients who did not, suggesting that development of occlusion amblyopia can indicate the potential for the development of better vision in the originally amblyopic eye.