Revisiting the surgical table: An analysis of surgical dose-response in Asian exotropia.

BACKGROUND: Previous research on the factors associated with surgical dose-response in strabismus surgery for exotropia has yielded inconsistent results. This study determined the factors influencing surgical dose-response in exotropia patients who underwent recession and resection (R&R). METHODS: Exotropia patients who underwent unilateral R&R at the National Taiwan University Hospital between 2006 and 2021 were evaluated. Deviation-angle differences in prism diopters (PD) were measured preoperatively and at 1 month postoperatively. Surgical dose-response (PD/mm) was defined as the difference in deviation angle (in PD) divided by the surgical dose in millimeters. Linear and non-linear regression models were used to evaluate the influence of variables including age, sex, axial length, and preoperative deviation on surgical dose-response. RESULTS: Overall, 295 patients (162 children; 133 adults) were included. Average surgical dose-response in the pediatric and adult groups was 2.82 ± 0.60 PD/mm and 3.02 ± 0.62 PD/mm, respectively. Male sex was negatively correlated with surgical dose-response in children. The surgical dose-response was larger in adults with longer axial length (>25.64 mm) and patients with larger preoperative deviation (>42.6 PD and >38.7 PD in pediatric and adult groups, respectively). Surgical dose-responses peaked at 35.1 years. CONCLUSION: Age, axial length, and preoperative deviation have a nonlinear effect on surgical dose-responses in exotropia patients undergoing R&R. Surgical dose-responses were larger in patients in young adulthood, with longer axial length and larger preoperative deviation angle. A table with fitted values for surgical dose-response based on age, axial length, and preoperative deviation was established for clinical reference.

Influence of parental behavior on myopigenic behaviors and risk of myopia: analysis of nationwide survey data in children aged 3 to 18 years.

BACKGROUND: Preventive parental behavior may play an important role in the outcomes of children's myopia. We investigated associations between parental behavior and children's myopia status and daily activities using data from the most recent myopia survey in Taiwan. METHODS: In total, 3845 children aged 3 to 18 years who completely responded to the questionnaire were included (total score ranging from 0 to 75). A score of ≥ 50 was considered to indicate beneficial parental behavior. Time allocation data for near-work activities, using electronic devices, and outdoor activities were collected using a separate self-reported questionnaire. Associations between beneficial parental behavior and children's myopia status and activity patterns were analyzed and stratified by school level. RESULTS: Beneficial parental behavior was positively associated with children's myopia in the overall samples [adj. odds ratio (OR): 1.31, 95% confidence interval (CI): 1.08-1.59, p = 0.006)] and at the elementary school level (adj. OR: 1.43, 95% CI: 1.11-1.83, p = 0.005). However, a negative association with high myopia was observed in the overall samples (adj. OR: 0.71, 95% CI: 0.50-0.99, p = 0.049) and high school level (adj. OR: 0.62, 95% CI: 0.41-0.92, p = 0.02). Beneficial parental behavior was associated with less time spent on near work (≥ 180 min/day) and electronic device use (≥ 60 min/day), but not with outdoor activities. CONCLUSION: In Taiwan, children's myopia is associated with higher rate of parents' beneficial behaviors, which suggests that regular vision surveillance is necessary to promote better parental behavior toward children's eye care. Certain parental practices may influence children's behavior pattern and reduce the risk of children's high myopia development in the long run.

Evolution of the Prevalence of Myopia among Taiwanese Schoolchildren: A Review of Survey Data from 1983 through 2017.

PURPOSE: The aim of this study was to evaluate the changes in the prevalence of myopia in Taiwanese schoolchildren over the past few decades and to analyze the risk factors for myopia. DESIGN: Analysis of 8 consecutive population-based myopia surveys conducted from 1983 through 2017. PARTICIPANTS: An average of 8917 (5019-11 656) schoolchildren 3 to 18 years of age were selected using stratified systematic cluster sampling or by probability proportional to size sampling. METHODS: All participants underwent complete ophthalmic evaluations. Three drops of 0.5% tropicamide were used to obtain the cycloplegic refractive status of each participant. Questionnaires were used to acquire participant data from the 1995, 2005, 2010, and 2016 surveys. MAIN OUTCOME MEASURES: Prevalence of myopia (spherical equivalence of ≤-0.25 diopter [D]) and high myopia (≤-6.0 D) was assessed. Multivariate analyses of risk factors were conducted. RESULTS: The prevalence of myopia among all age groups increased steadily. From 1983 through 2017, the weighted prevalence increased from 5.37% (95% confidence interval [CI], 3.50%-7.23%) to 25.41% (95% CI, 21.27%-29.55%) for 7-year-olds (P = 0.001 for trend) and from 30.66% (95% CI, 26.89%-34.43%) to 76.67% (95% CI, 72.94%-80.40%) for 12-year-olds (P = 0.001 for trend). The prevalence of high myopia also increased from 1.39% (95% CI, 0.43%-2.35%) to 4.26% (95% CI, 3.35%-5.17%) for 12-year-olds (P = 0.008 for trend) and from 4.37% (95% CI, 2.91%-5.82%) to 15.36% (95% CI, 13.78%-16.94%) for 15-year-olds (P = 0.039 for trend). In both the 2005 and 2016 survey samples, children who spent less than 180 minutes daily on near-work activities showed significantly lower risks for myopia developing (<60 minutes: odds ratio [OR], 0.48 and 0.56; 60-180 minutes: OR, 0.69 and 0.67). In the 2016 survey, spending more than 60 minutes daily on electronic devices was associated significantly with both myopia and high myopia (OR, 2.43 and 2.31). CONCLUSIONS: The prevalence of myopia among schoolchildren increased rapidly from 1983 through 2017 in Taiwan. The major risk factors are older age and time spent on near-work activities. Use of electronic devices increased the amount of time spent on near-work and may increase the risk of developing myopia.

Refinement strategies in photoscreening for the detection of amblyopia risk factors in 12-to-24-month-old children.

PURPOSE: To establish an appropriate clinical protocol for early photoscreening in 12-to-24-month-old children at pediatric well-baby clinics. METHODS: This prospective study included a total of 277 children aged 12-24 months who visited a pediatric well-baby clinic. All participants underwent: 1) inquiry of medical history; 2) photoscreening with PlusoptiX A12; and 3) comprehensive ophthalmologic examinations. The optimal referral cut-off point for PlusoptiX was determined by receiver operating characteristic analyses. A high-risk subgroup was defined as having a birth weight <3000 g or a history of major systemic diseases, based on the results of multivariable risk factor analysis from children's medical history. A strategy of selective photoscreening focusing on the high-risk subgroup was evaluated. The main outcome measures included sensitivity, specificity, and positive and negative predictive values. RESULTS: The prevalence of amblyopia risk factors in our study population was 12.3%. A total of 172 children (62.1%) were assigned to the high-risk subgroup. Compared with the nonselective photoscreening of all 277 children, selective photoscreening of the high-risk children yielded a higher positive predictive value (59.6% vs. 46.7%, p = 0.001) while showing no difference in sensitivity (82.3% vs. 85.3%, p = 0.32), specificity (92.2% vs, 86.4%, p = 0.05), and negative predictive value (97.4% vs. 97.6%, p = 0.50). CONCLUSION: At pediatric well-baby clinics, selective photoscreening among 12-to-24-month-old children with a birth weight <3000 g or a history of major systemic diseases helped reducing the number of children need to be screened and conserving medical resources yet identifying children at risk for timely eye care.

Genome-Wide Association Study in Asians Identifies Novel Loci for High Myopia and Highlights a Nervous System Role in Its Pathogenesis.

PURPOSE: To identify novel susceptibility loci for high myopia. DESIGN: Genome-wide association study (GWAS) followed by replication and meta-analysis. PARTICIPANTS: A total of 14 096 samples from East and Southeast Asian populations (2549 patients with high myopia and 11 547 healthy controls). METHODS: We performed a GWAS in 3269 Japanese individuals (1668 with high myopia and 1601 control participants), followed by replication analysis in a total of 10 827 additional samples (881 with high myopia and 9946 control participants) from Japan, Singapore, and Taiwan. To confirm the biological role of the identified loci in the pathogenesis of high myopia, we performed functional annotation and Gene Ontology (GO) analyses. MAIN OUTCOME MEASURES: We evaluated the association of single nucleotide polymorphisms with high myopia and GO terms enriched among genes identified in the current study. RESULTS: We identified 9 loci with genome-wide significance (P < 5.0 × 10-8). Three loci were previously reported myopia-related loci (ZC3H11B on 1q41, GJD2 on 15q14, and RASGRF1 on 15q25.1), and the other 6 were novel (HIVEP3 on 1p34.2, NFASC/CNTN2 on 1q32.1, CNTN4/CNTN6 on 3p26.3, FRMD4B on 3p14.1, LINC02418 on 12q24.33, and AKAP13 on 15q25.3). The GO analysis revealed a significant role of the nervous system related to synaptic signaling, neuronal development, and Ras/Rho signaling in the pathogenesis of high myopia. CONCLUSIONS: The current study identified 6 novel loci associated with high myopia and demonstrated an important role of the nervous system in the disease pathogenesis. Our findings give new insight into the genetic factors underlying myopia, including high myopia, by connecting previous findings and allowing for a clarified interpretation of the cause and pathophysiologic features of myopia at the molecular level.

Incidence and risk of attention-deficit hyperactivity disorder in children with amblyopia: A nationwide cohort study.

IMPORTANCE: The association between visual deficits and attention disorders has been reported but remains unproven. BACKGROUND: The objective of this study was to evaluate the risk of attention-deficit hyperactivity disorder (ADHD) in children with amblyopia. DESIGN: Population-based, cohort study. PARTICIPANTS: The dataset from the Taiwan National Health Insurance Research Database in 2000 to 2010. METHODS: A total of 6817 patients aged <18 years with newly diagnosed amblyopia were identified. Four age- and sex-matched controls without amblyopia were included for each patient, that is, 27268 controls. MAIN OUTCOME MEASURES: The primary outcome was the risk of ADHD. The secondary outcomes were age at ADHD onset and use of ADHD medication. RESULTS: During a mean observation period of 7.18 years, the incidence of ADHD per 1000 person-years was 7.02 in the amblyopia group and 4.61 in the control group (P < 0.0001). The ADHD risk in the amblyopia group was 1.81 times that in the control group (hazard ratio 1.81; 95% confidence interval 1.59-2.06). After stratification by amblyopia subtype, the greatest risk was in the deprivation type (hazard ratio 2.14; 95% confidence interval 1.56-2.92) followed by the strabismic (hazard ratio 2.09; 95% confidence interval 1.15-3.79) and refractive (hazard ratio 1.76; 95% confidence interval 1.54-2.02) types. Age at ADHD onset was younger in the amblyopia group (median 8.14 vs 8.45 years; P = 0.0096). The average duration of neuropsychiatric medication use was comparable between groups (P = 0.98). CONCLUSIONS AND RELEVANCE: The ADHD risk is higher in children with amblyopia.

Outcomes of pediatric cataract surgery with triamcinolone-assisted vitrectomy.

BACKGROUND/PURPOSE: To evaluate outcomes in pediatric patients undergoing lensectomy, posterior capsulotomy, and triamcinolone-assisted vitrectomy for congenital cataract. METHODS: This retrospective study included 34 patients younger than 72 months who underwent lensectomy, posterior capsulotomy, and triamcinolone-assisted vitrectomy with or without intraocular lens (IOL) implantation for cataract at the National Taiwan University Hospital from July 2006 to December 2012. RESULTS: Fifty-one eyes from 34 patients with cataract (unilateral in 17 patients, bilateral in 17 patients) were included. The mean age at surgery was 26.74 months (range: 2-72 months). The mean postoperative follow-up was 27.8 months (range: 6-72 months). Primary IOL implantation was performed in 25 eyes, 21 of which had the IOL implanted in the capsular bag. Fifty eyes had a central round pupil. The median logarithm of the minimum angle of resolution visual acuity was 0.3 in patients with unilateral cataract and 0.1 in those with bilateral cataract. Three eyes (5.9%) developed visual axis opacification (VAO) and required further surgery. Univariate analysis using Fisher's exact test indicated that surgery in the first 12 months of life was significantly associated with development of VAO (p=0.047). The incidence of postoperative VAO was approximately 15.8% in this age group. CONCLUSION: Triamcinolone-assisted vitrectomy can be used in pediatric cataract surgery without serious long-term adverse effects. While the incidence of VAO is low, it appears unavoidable in approximately one-sixth of patients who undergo surgery before 12 months of age.

Long-term prognosis of patients with infantile-onset Pompe disease diagnosed by newborn screening and treated since birth.

OBJECTIVE: To determine the benefit of newborn screening for the long-term prognosis of patients with classic infantile-onset Pompe disease (IOPD). STUDY DESIGN: A cohort of patients with classic IOPD were diagnosed by newborn screening, treated with recombinant human acid α-glucosidase (rhGAA), and followed prospectively. Outcome measurements included survival, left ventricular mass, serum creatinine kinase, motor function, mental development, and systemic manifestations. RESULTS: Ten patients who presented with left ventricular hypertrophy at diagnosis received rhGAA infusions starting at a median age of 16 days (6-34 days). All patients were cross-reactive immunologic material-positive. After a median treatment time of 63 months (range 28-90 months), all could walk independently, and none required mechanical ventilation. All patients had motor capability sufficient for participating in daily activities, but muscle weakness over the pelvic girdle appeared gradually after 2 years of age. Ptosis was present in one-half of the patients, and speech disorders were common. Anti-rhGAA antibody titers were low (median maximal titer value 1:1600, range: undetectable ∼ 1:12,800). CONCLUSION: By studying patients treated since birth who have no significant anti-rhGAA antibody interference, this prospective study demonstrates that the efficacy of rhGAA therapy is high and consistent for the treatment of classic IOPD. This study also exposes limitations of rhGAA treatment. The etiology of the manifestations in these early-treated patients will require further study.

Clinical presentations of X-linked retinoschisis in Taiwanese patients confirmed with genetic sequencing.

PURPOSE: To investigate the clinical characteristics of X-linked retinoschisis (XLRS) and identify genetic mutations in Taiwanese patients with XLRS. METHODS: This study included 23 affected males from 16 families with XLRS. Fundus photography, spectral domain optical coherent tomography (SD-OCT), fundus autofluorescence (FAF), and full-field electroretinograms (ERGs) were performed. The coding regions of the RS1 gene that encodes retinoschisin were sequenced. RESULTS: The median age at diagnosis was 18 years (range 4-58 years). The best-corrected visual acuity ranged from no light perception to 20/25. The typical spoke-wheel pattern in the macula was present in 61% of the patients (14/23) while peripheral retinoschisis was present in 43% of the patients (10/23). Four eyes presented with vitreous hemorrhage, and two eyes presented with leukocoria that mimics Coats' disease. Macular schisis was identified with SD-OCT in 82% of the eyes (31/38) while foveal atrophy was present in 18% of the eyes (7/38). Concentric area of high intensity was the most common FAF abnormality observed. Seven out of 12 patients (58%) showed electronegative ERG findings. Sequencing of the RS1 gene identified nine mutations, six of which were novel. The mutations are all located in exons 4-6, including six missense mutations, two nonsense mutations, and one deletion-caused frameshift mutation. CONCLUSIONS: XLRS is a clinically heterogeneous disease with profound phenotypic inter- and intrafamiliar variability. Genetic sequencing is valuable as it allows a definite diagnosis of XLRS to be made without the classical clinical features and ERG findings. This study showed the variety of clinical features of XLRS and reported novel mutations.

Focal foveal atrophy of unknown etiology: clinical pictures and possible underlying causes.

BACKGROUND/PURPOSE: Focal foveal atrophy is defined as the presence of a small, focal, ill-defined, hypopigmented foveal or juxtafoveal lesion, with the remaining retina unaffected. The purpose of this study was to report the clinical characteristics and optical coherence tomography (OCT) in patients with focal foveal atrophy of unknown etiology. METHODS: The study was a retrospective observational case series. Data collected included complete ocular examination results for best corrected visual acuity (BCVA), ophthalmoscopy, fundus photography, fluorescein angiography, color sense discrimination tests, visual field tests, and OCT examinations. RESULTS: Twenty-three eyes in 21 patients were examined. The mean patient age was 49.2 ± 15.4 years. The mean BCVA was 20/25. The 21 patients were divided into three groups according to OCT results. Group 1 eyes (n = 10) had intact inner and outer hyperreflective layers (HRLs), with the signal of the inner HRL corresponding to the junction between the inner and outer photoreceptor segments and the outer HRL corresponding to the retinal pigment epithelium (RPE). Group 2 eyes (n = 9) had small hyporeflective defects with defects in the inner HRL at the fovea but an intact outer HRL. Group 3 eyes (n = 4) had small hyporeflective defects in both the inner and outer HRLs at the fovea. Groups 3 eyes had significantly lower visual acuity compared to Group 1 eyes and Group 2 eyes. There was no significant difference in visual acuity between Group 1 and Group 2 eyes. There were no significant differences among the groups with respect to color vision or foveal thickness. CONCLUSION: This is the first report of clinical presentations for patients with focal foveal atrophy of unknown etiology. OCT aided in the diagnosis and assessment of the degree of retinal structural abnormalities, but the real etiology of foveal atrophy remains unclear.

Late-Onset Abducens Nerve Palsy after Endovascular Treatment for Carotid-Cavernous Fistula: Two Case Reports.

Endovascular treatment, including transarterial embolisation and transvenous embolisation, is generally considered effective for treating carotid-cavernous fistula. Immediate cranial nerve palsy following the procedure is the most common complication, but it usually resolves spontaneously. The authors report two cases of late-onset abducens nerve palsy after successful obliteration of direct carotid-cavernous fistulas, both of which required strabismus surgery.

A Novel Device for Vergence Exercises: Testing Automatic Dual Rotational Risley Prisms on Asymptomatic Adults and Patients with Convergence Insufficiency.

INTRODUCTION: This study investigated the effects of automatic dual rotational Risley prisms (ADRRPs), a mobile phone application-operated device, on vergence abilities in young adults. METHODS: Fifty-six participants aged 20-24 performed vergence exercises. The test group used prisms with power changes from 30Δ base-out to 10Δ base-in, while the control group used plano lenses for 10 min. Ophthalmic examinations included lateral heterophoria, near point of convergence (NPC), vergence facility (VF), negative fusional vergence (NFV), and positive fusional vergence (PFV), all measured before and after the vergence exercises. Pre- and post-test results were analyzed using a paired sample t test. Additionally, three cases with convergence insufficiency (CI) performed similar exercises for 12 weeks. RESULTS: Participants were divided into the test group (n = 39; age 21.82 ± 1.10 years) and control group (n = 17; age 20.53 ± 0.51 years). In the test group, NPC improved from 6.11 ± 2.52 cm to 5.77 ± 2.30 cm (p = 0.023). VF increased from 13.75 ± 4.10 cpm to 16.50 ± 4.42 cpm (p = 0.007). PFV at 6 m and 0.4 m increased from 19.49 ± 6.77∆ to 22.19 ± 6.64∆ (p < 0.001) and 20.51 ± 7.05∆ to 22.69 ± 6.44∆ (p = 0.012), respectively. After 12 weeks, convergence insufficiency symptom survey scores for cases with CI decreased significantly, with NPC improving from 7.0 to 0 cm, 6.0 to 5.8 cm, and 6.0 to 4.7 cm. PFV increased from 10 to 25∆, 20 to 30∆, and 25 to 50∆. CONCLUSION: This preliminary study showed the effect of ADRRPs on improving vergence abilities. Further studies are needed to investigate the long-term sustainability and effects in a larger population of individuals with CI of this approach.

Genetic testing confirmed osteopetrosis with initial presentation of nystagmus.

Osteopetrosis (OS) is a rare heritable disorder characterized by osteoclast dysfunction and increased bone density on radiography. Optic nerve osseous compression is the most frequent ocular complication of OS, with nystagmus, strabismus, ptosis, proptosis, and lagophthalmos occurring less frequently. However, it is uncommon for patients to have neurological or ocular symptoms at initial presentation. Herein, we present the case of a 3-year-old girl with the initial presentation of ocular symptoms who was confirmed to have OS through genetic testing. She was born full-term and found to have nystagmus since the age of 1 year. Her best-corrected visual acuity was 1.2/60 for both eyes. Exotropia of the left eye and bilateral small-amplitude pendular nystagmus were also noted. Color fundoscopy revealed a tessellated fundus and pale discs with cup-to-disc ratios of 0.5-0.6. Magnetic resonance imaging revealed bilateral optic canal stenosis and optic nerve atrophy. Whole-exome sequencing revealed a biallelic chloride voltage-gated channel 7 mutation, c.2297T > C (p.Leu766Pro) and c.1577G > A (p.Arg526Gln), and autosomal recessive OS was diagnosed. The patient is currently being evaluated for possible hematopoietic stem cell transplantation. We suggest that OS should be considered a differential diagnosis for unexplained nystagmus and optic nerve atrophy.

Impact of atropine use for myopia control on intraocular pressure in children: A comprehensive review including postpupil dilation intraocular pressure changes.

Topical atropine has been widely used for controlling myopia progression in children, yet its long-term efficacy and safety, including potential intraocular pressure (IOP) elevation, are still being studied. The mydriasis and cyclopegia induced by atropine may reduce traction on the trabecular meshwork, together with pigment released into anterior chamber due to the friction between the iris and lens during pupil dilation, may obstruct and reduce the trabecular outflow. This review first explores postdilation IOP changes across different groups - healthy individuals, glaucoma patients, and children. The response to pupil dilation varies widely, with IOP potentially increasing or decreasing. Glaucoma patients, whether with open or closed-angle glaucoma, may experience more significant IOP rises postdilation. The second section examines IOP effects in children using topical atropine for myopia, where most of the 25 reviewed studies showed nonsignificant IOP changes, although slight increases were observed in a few. In addition, no alterations in the retinal nerve fiber layer thickness were found. However, the research on children's IOP under topical atropine is constrained by small sample sizes, cross-sectional studies, brief follow-ups, and often lacks control groups or pretreatment IOP measurements. Given the extended atropine use for myopia and the significant individual variation in IOP response, we recommend routine IOP monitoring for children receiving topical atropine.

The impact of light properties on ocular growth and myopia development.

The objective of this article is to comprehensively review the effect of environmental lighting on ocular growth and refractive status in both animal and clinical studies, with an emphasis on the underlying mechanisms. This review was performed by searching research articles and reviews utilizing the terms "myopia," "light therapy," "axial length," "refractive error," and "emmetropization" in PubMed datasets. The review was finalized in December 2023. In the animal studies, high lighting brightness, illumination periods aligning with circadian rhythm, and color contrast signals including multiple wavelengths all help regulate ocular growth against myopia. Long wavelengths have been found to induce myopia in chicks, mice, fish, and guinea pigs, whereas shorter wavelengths lead to hyperopia. In contrast, red light has been observed to have a protective effect against myopia in tree shrews and rhesus monkeys. Apart from wavelength, flicker status also showed inconsistent effects on ocular growth, which could be attributed to differences in ocular refractive status, evolutionary disparities in retinal cone cells across species, and the selection of myopia induction models in experiments. In the clinical studies, current evidence suggests a control effect with red light therapy. Although the lighting conditions diverge from those in animal experiments, further reports are needed to assess the long-term effects. In conclusion, this review encompasses research related to the impact of light exposure on myopia and further explores the retinoscleral signaling pathway in refractive development. The aim is to establish a theoretical foundation for optimizing environmental factors in lighting design to address the epidemic of childhood myopia.

Efficacy of Multifocal Soft Contact Lenses in Reducing Myopia Progression Among Taiwanese Schoolchildren: A Randomized Paired-Eye Clinical Trial.

INTRODUCTION: To evaluate the efficacy and safety of myopia control using a multifocal soft contact lens designed with high peripheral add power in schoolchildren. METHODS: This 1-year multi-center, prospective, randomized, double-blind, controlled study enrolled myopic schoolchildren aged 6-15 years with refractive errors between - 1.0 D and - 10.0 D. Each participant was randomly allocated to wear a daily disposable multifocal soft contact lens as the treatment in one eye and a single-vision soft contact lens as the control in the other eye. The primary endpoints were changes in the cycloplegic spherical equivalent (SE) and axial length at 1 year. RESULTS: Fifty-two of the 59 participants (88.1%) completed the study protocol. The mean change in SE was - 0.73 ± 0.40 D in the treatment group. and - 0.85 ± 0.51 D in the control group (mean difference: - 0.12 ± 0.34 D, p = 0.012). The mean change in axial length was 0.25 ± 0.14 mm in the treatment group, and 0.33 ± 0.17 mm in the control group (mean difference: 0.08 ± 0.10 mm, p < 0.001). The treatment was well tolerated, and no serious adverse events were observed. CONCLUSIONS: Treatment with multifocal soft contact lenses with high peripheral add power was effective in controlling the progression of myopia and axial length elongation in myopic schoolchildren.

Relationship between genotype, phenotype, and refractive status in patients of inherited retinal degeneration.

BACKGROUND: To elucidate the relationship between inherited retinal disease, visual acuity and refractive error development in Asian patients. SUBJECTS: Five hundred phakic eyes with refractive data were analysed in this retrospective cohort. Diseases were categorized by clinical phenotypes, and the prevalent genotypes identified in the Taiwan Inherited Retinal Degeneration Project were analysed. Consecutive surveys in Taiwan have provided the rates of myopia in the general population. RESULTS: No differences were observed among the disease phenotypes with respect to myopia (P = 0.098) and high myopia rates (P = 0.037). The comparison of refractive error between retinitis pigmentosa and diseases mainly affecting the central retina showed no difference, and the refraction analyses in diseases of different onset ages yielded no significance. Moreover, there was no difference in the myopia rate between the diseases and general population. Among the genotypes, a higher spherical equivalent was seen in RPGR and PROM1-related patients and emmetropic trends were observed in patients with CRB1 and PRPF31 mutations. Furthermore, significantly poorer visual acuity was found in ABCA4, CRB1 and PROM1-related patients, and more preserved visual acuity was seen in patients with EYS, USH2A, and RDH12 mutations. CONCLUSIONS: No significant differences were observed in visual acuity, refractive state and myopia rate between patients with inherited retinal disease and the general population, and different subtypes of inherited retinal disease shared similar refractive state, except for higher cylindrical dioptres found in patients with Leber's congenital amaurosis. The heterogeneity of disease-causing genes in Asian patients may lead to variable refractive state.

Clinical characteristics and visual function tests with retinal tomographic correlation in patients with Stargardt's disease in Taiwan.

BACKGROUND/PURPOSE: Stargardt's disease is the most common form of juvenile macular dystrophy. The purpose of this study is to report the clinical characteristics, visual function, and retinal tomography of patients diagnosed with Stargardt's disease in Taiwan. METHODS: Retrospective case series; data collected include results of complete ophthalmic examinations: best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, indirect ophthalmoscopy, fundus photography, fluorescein angiography, color sense discrimination test, electroculography (EOG), electroretinography (ERG), dark adaptation, visual field test, and optical coherence tomography (OCT) examinations focused on the macular area. RESULTS: Twenty patients were enrolled in this study. The mean age of the patients was 32.1 years. The mean logMAR-converted visual acuity of all patients was 0.96. Maculopathy with or without flecks was evident in all patients. Fluorescein angiography revealed hyperfluorescence in the macula of all cases. Varying degrees of color sense discrimination dysfunction were noted. Electrophysiologic studies showed subnormal to abnormal responses in most patients. Decreased foveal thickness was evident on OCT and foveal thickness was inversely associated with logMAR visual acuity. Also, logMAR visual acuity was associated with total error scores on the color sense discrimination test and the Arden ratio of EOG. The Arden ratio of EOG correlated with the thickness of the outer ring of the macula. CONCLUSION: Typical clinical presentations of Stargardt's disease were demonstrated in our case series. OCT findings showed a correlation between foveal thickness and visual acuity, making OCT a useful predictor for other visual function tests. Our results indicate that OCT is helpful in the evaluation of visual acuity and the progression of maculopathy in patients with Stargardt's disease.

Ophthalmologic diagnoses in youths with autism spectrum disorder: Prevalence and clinical correlates.

Autism spectrum disorder (ASD) is associated with a high prevalence of visual dysfunction. This study aimed to investigate the rates of amblyopia, refractive errors, and strabismus, as well as their clinical correlates in ASD. This population-based matched-cohort study used data from the Taiwan National Health Insurance Research Database. A total of 3,551 youths with ASD and 35,510 non-autistic control participants matched by age and sex were included. All the participants were followed-up until they were 18 years old. The prevalence of amblyopia, refractive errors, and strabismus was compared between the ASD and control groups. Effect modifiers, including sex, ASD subgroup, and co-diagnosis of intelligence disability, were examined. Compared to the control group, youths with ASD had a significantly increased risk of amblyopia (adjusted odds ratio [aOR] = 1.75), anisometropia (aOR = 1.66), astigmatism (aOR = 1.51), hypermetropia (aOR = 2.08), exotropia (aOR = 2.86), and esotropia (aOR = 2.63), but a comparable likelihood of myopia according to age. Males with ASD had a significantly lower likelihood of exotropia, but a higher likelihood of myopia than females with ASD. The autism subgroup had a higher OR for hypermetropia, but a lower OR for myopia than the other ASD subgroups. ASD youths with intelligence disabilities demonstrated significantly higher ORs for amblyopia, hypermetropia, and all types of strabismus and lower OR for myopia than those without intelligence disabilities. In conclusion, the rates of amblyopia, refractive errors, and strabismus were higher in youths with ASD. Ocular abnormalities in youths with ASD require a comprehensive assessment and management.

Immediate Effects of Vergence Exercises Using Automatic Dual Rotational Risley Prisms on Accommodative Lag and Facility.

INTRODUCTION: This study evaluated novel automatic dual rotational Risley prisms (ADRRPs) as a vergence exercise tool for patients with myopia to improve accommodative lag and accommodative facility. METHODS: Participants with myopia aged 20-24 years were recruited. After vergence exercises with prisms (treatment group) or plano lenses (control group) using ADRRPs for 10 min, measurements were taken using an open-field autorefractor (Grand Seiko WAM-5500) at viewing distances of 0.4 m and 6.0 m. We measured accommodative facility using a ± 2.00 D accommodative flipper. RESULTS: A total of 56 participants (treatment group, 39; control group, 17) performed vergence exercises using ADRRPs. Participants in the treatment group showed improvements in accommodative lag at a 0.4 m viewing distance, with measurements of 0.57 D (right eye; OD) and 0.53 D (left eye; OS) and 0.21 D (OD) and 0.27 D (OS) before and after the exercises, respectively (p < 0.001). Over-refractions using an open-field autorefractor with spherical equivalent contact lenses at a 6.0 m viewing distance were - 0.01 ± 0.30 D (OD) and 0.03 ± 0.34 D (OS) and 0.15 ± 0.32 D (OD) and 0.19 ± 0.28 D (OS) before and after the exercises, respectively (difference + 0.16 D; p < 0.001). Accommodative facility values before and after exercises were 14.88 ± 3.36 and 15.59 ± 3.60 cpm, respectively (p < 0.01). No significant differences in accommodative lag, relaxation, and accommodative facility before and after exercise were observed in the control group. CONCLUSIONS: Using ADRRPs in vergence exercises can improve accommodative lag, accommodative facility, and accommodative relaxation in adults with myopia. Further research to evaluate persistent and long-term effects is needed.