Miho Sato

Purpose: To investigate changes in strabismus angles in children and young adult patients with recent onset of constant acquired comitant esotropia (ACE) following instructions on hand-held digital devices (DD). Study design: Prospective multicenter non-randomized interventional study. Methods: This study included subjects aged 5–35 years who developed ACE within 1 year. Subjects were divided into two groups: (i) subjects who used DD for an average of ≥120 minutes/day for junior high-school students and older and ≥60 minutes/day for primary-school students and younger (DD+); and (ii) subjects who used DD for less than that (DD-) based on the questionnaire at the study’s start. During the initial visit, glasses were prescribed when necessary; verbal instructions on DD use including time reduction and viewing-distance elongation were provided. For each group, strabismus angles at the initial visit and at 3 months were compared. Cure was defined as esophoria within 8 prism diopters without symptoms. Results: In total, 181 cases were investigated. At baseline, strabismus angles in DD+ and DD- groups were 23±14 PD and 25±15 PD at near and 25±13 PD and 27±14 PD at distance, respectively. At 3 months, they were 22±16 PD and 25±15 PD at near and 23±14 PD and 27±13 PD at distance, respectively. Only in the DD+ group, reduction in strabismus angle was observed, but this was not clinically significant. Ten and 1 subjects in DD+ and DD- groups were cured. Conclusion: Although changes in strabismus angles were not large enough, DD use instructions were beneficial for some ACE subjects.

Objective: The objective of this study is to investigate factors associated with outcomes after 3 months of instructed usage of hand-held digital devices (DD) in patients with acquired comitant esotropia (ACE). Methods and analysis: This prospective multicentre observational study included patients with ACE, aged 5–35 years, who used DD within 1 year of onset and were followed up for clinical findings and instructed use of DD. The outcomes were classified into four groups: cured, improved, unchanged and worsened. After the analysis of group differences in the clinical and DD use-related factors by univariate analysis, we used ordinal logistic regression models to identify factors associated with favourable outcomes. Results: Of 156 patients (mean age (SD): 16.4 (7.4) years), 10 (6%), 58 (37%), 67 (43%) and 21 (14%) were classified into the cured, improved, unchanged, and worsened, respectively. In the univariate analysis, consultation within 3 months of onset, small-angle strabismus at distance and good stereoacuity were associated with good outcomes. Ordinal logistic regression analysis on adjusting for age with stereoacuity or successful DD-use time halving showed that small-angle strabismus at distance (OR: 1.02, 95% CI 1.00 to 1.03, p=0.023), good stereoacuity (OR: 1.31; 95% CI 1.10 to 1.56; p=0.003) and successful halving of DD-use time (OR: 0.63; 95% CI 0.43 to 0.92; p=0.016) influenced favourable outcomes. Conclusion: Patients with small-angle esotropia, good stereoacuity on consultation and success in halving DD-use time had a higher chance of recovery through instructional DD usage. Further studies using objectively measurable systems are needed to ensure the accuracy of DD-use time.

Acute acquired comitant esotropia (AACE) is characterized by acute onset of esotropia without limitation of eye movements in children and adults. AACE has been considered relatively rare, and most previous reports were small case series. However, recently, there has been great interest in this disease because of the rapid increase in the number of cases speculated to be related to the use of digital devices. In a growing number of studies, disputes remain regarding its etiology, clinical features, management, and classification. Herein, we review the literature on the history, clinical features, possible etiologic classifications, and treatment strategies for AACE to provide a comprehensive and updated understanding of the disease.

Purpose To evaluate changes in intraocular pressure (IOP) at different gaze positions before and after superior rectus muscle-lateral rectus muscle (SR-LR) loop myopexy in highly myopic strabismus (HMS). Study design Nonrandomized clinical, prospective, interventional trial. Methods Fourteen patients with HMS (18 eyes) who underwent SR-LR loop myopexy were divided into 3 groups: < 100 prism diopters (PD) (mild esotropia [ET] group), > 100 PD (large ET group), and > 100 PD, and simultaneous recession of the medial rectus (MR) muscle was performed (large ET + MR group). Intraocular pressure was measured preoperatively and postoperatively at the primary, abduction, and adduction positions in each group. Results Intraocular pressure did not change after surgery in the mild ET group. Intraocular pressure significantly decreased in the abduction position (from 20.0 ± 2.1 to 16.0 ± 1.9 mmHg, P = 0.043) in the large ET group and in the abduction (from 22.2 ± 5.9 to 15.6 ± 4.3 mmHg, P = 0.048) and primary positions (from 15.8 ± 5.0 to 10.2 ± 2.8 mmHg, P = 0.043) in the large ET + MR group. The preoperative significant differences in IOP between the abduction and adduction positions in the large ET group (7.4 ± 3.4 mmHg) and the large ET + MR group (10.0 ± 5.5 mmHg) disappeared postoperatively (3.2 ± 2.8 mmHg and 3.6 ± 1.7 mmHg, respectively). The differences in IOP between abduction and adduction were similar in all the groups. Conclusion SR-LR loop myopexy decreased IOP in patients with HMS in the abduction and primary positions.

Purpose To describe clinical presentations of acquired comitant esotropia and digital device use in children, adolescents, and young adults without neurological problems. Study design Multicenter prospective observational study. Methods Patients with acquired comitant esotropia, without intracranial diseases aged 5–35 years at the time of visit, who were seen at pre-registered facilities within 1 year of onset were enrolled. The duration from the onset of symptoms and the time of digital device usage approximately 1 month before onset and their lifestyles were surveyed. Visual acuity, cycloplegic refraction, and strabismus angles were measured. Data were analyzed in three age groups (Child: 5–12 years, Adolescent: 13–18 years, and Young adult: 19–35 years). Results Between November 2019 and December 2021, 218 patients were enrolled from 55 facilities, and 194 patients (including 62 children, 69 adolescents, and 63 young adults) were analyzed. The child group spent the least amount of time using digital devices (children: 159; adolescents: 210; young adults: 267 min/work day, p < 0.05; (mean time in the same order below) 229, 338, 314 min/holiday, p < 0.05) and had the largest strabismus angle (mean strabismus angle at near: 30, 22, 18 PD, p < 0.01; at far: 28, 26, 21 PD, p<0.05). Conclusion The clinical features of acquired comitant esotropia and hand-held digital device usage differed between children aged ≤ 12 years and older patients. This report gives the current clinical characteristics of young patients with acquired esotropia and digital device usage.

Leber congenital amaurosis (LCA) is the most severe form of inherited retinal dystrophy. RPGRIP1-related LCA accounts for 5–6% of LCA. We performed whole-exome sequencing and whole-genome sequencing (WGS) on 29 patients with clinically suspected LCA and examined ophthalmic findings in patients with biallelic pathogenic variants of RPGRIP1. In addition to five previously reported cases, we identified five cases from four families with compound heterozygous RPGRIP1 variants using WGS. Five patients had null variants comprising frameshift variants, an Alu insertion, and microdeletions. A previously reported 1339 bp deletion involving exon 18 was found in four cases, and the deletion was relatively prevalent in the Japanese population (allele frequency: 0.002). Microdeletions involving exon 1 were detected in four cases. In patients with RPGRIP1 variants, visual acuity remained low, ranging from light perception to 0.2, and showed no correlation with age. In optical coherence tomography images, the ellipsoid zone (EZ) length decreased with age in all but one case of unimpaired EZ. The retinal structure was relatively preserved in all cases; however, there were cases with great differences in visual function compared to their siblings and a 56-year-old patient who still had a faint EZ line. Structural abnormalities may be important genetic causes of RPGRIP1-related retinal dystrophy in Japanese patients, and WGS was useful for detecting them.

Purpose To report the characteristics of the steroid-induced ocular hypertensive response in pediatric patients with acute lymphoblastic leukemia (ALL) treated with prednisolone (PSL) during induction therapy and with dexamethasone (DEX) during reinduction therapy. Study design Retrospective. Patients and methods This study included pediatric patients diagnosed with B-cell precursor ALL and treated with systemic corticosteroids sometime during the period from 2016 to 2018 at Shizuoka Children's Hospital. Data were extracted from the hematology/oncology records related to the type, dose, and duration of systemic corticosteroids as well as to the ophthalmologic examination findings, intraocular pressure (IOP) data, symptoms of high IOP, and antiglaucoma medications obtained during corticosteroid administration. The maximal IOPs of the PSL and DEX groups were compared. Results Twenty-eight patients (18 boys and 10 girls; mean age 5.5 years) were treated with systemic corticosteroids. Twelve of the 22 courses of PSL and 33 of the 44 courses of DEX were found to be associated with high IOP. The maximal IOP was higher with the use of DEX than with the use of PSL, including in those who received prophylactic therapy (PSL 25.2 mmHg, DEX 33.6 mmHg; P = 0.02). Antiglaucoma medication was given to 21 patients; 6 patients had symptoms of ocular hypertension. The maximal IOPs were 52.8 mmHg and 70.8 mmHg in the PSL and DEX groups, respectively. Both groups of patients reported severe headache. Conclusion Increased IOP was frequently observed during systemic corticosteroid therapy in pediatric patients with ALL. Although most patients were asymptomatic, they occasionally presented with severe systemic symptoms. Regular ophthalmologic examinations should be included in the treatment guidelines for ALL.