Nonsurgical Consecutive Exotropia Following Childhood Esotropia: A Multicentered Study.

PURPOSE: Nonsurgical consecutive exotropia (NCX) occurs when an esotropia (ET) spontaneously converts to exotropia (XT) without surgical intervention. Although NCX is considered to occur in early-onset accommodative ET with high hyperopia, consensus on causation is lacking. We report the clinical characteristics of NCX and assess the response to conservative management. DESIGN: Retrospective, multicenter, observational case series. METHODS: Patients aged 6 months and older with an initial diagnosis of ET who converted to XT without surgical intervention. Sensory strabismus was excluded. Age, visual acuity, cycloplegic refraction, glasses prescriptions, deviation, and binocular vision were collected. RESULTS: Forty-nine children were included with a mean age of 3.5 ± 1.6 years and 8.4 ± 3.6 years at the time of ET and NCX, respectively. Mean refractive error was +4.40 ± 2.13 diopters (D) and +4.05 ± 2.74 D at the time of ET and NCX, respectively. Accommodative ET occurred in 60% of cases, and only 35.7% were high hyperopes. All but 1 patient presented with XT at distance. In response to the XT, a mean decrease in hyperopic prescription of 1.55 ± 0.48 D was given (N = 17); only 1 case reverted to ET. Eventually, 43% underwent XT surgery, with similar rates between those who had refractive management and those who did not. CONCLUSIONS: NCX occurs in both accommodative and nonaccommodative ET; high hyperopia is present in only one-third of cases. On average, drift to XT occurs within 5 years. Refractive management has a modest result. No predictive risk factors were identified. Our findings challenge hyperopia-linked theories of causation. Nonrefractive explanations, such as the role of the vergence system, deserve further study.

A Meta-Analysis of Neurodevelopmental Outcomes following Intravitreal Bevacizumab for the Treatment of Retinopathy of Prematurity.

BACKGROUND: Retinopathy of prematurity (ROP) is the most common cause of preventable blindness in preterm infants. First-line treatments include intravitreal bevacizumab (IVB) or laser photocoagulation (LPC). OBJECTIVES: The aim of the study was to evaluate neurodevelopmental safety of IVB compared to LPC for ROP. METHODS: MEDLINE, Embase, and Cochrane library were searched up to September 2022. Studies were included with at least 12-month follow-up of primary outcomes such as severe neurodevelopmental impairment (sNDI), cerebral palsy (CP), and hearing impairment (HI). Secondary outcomes were moderate-to-severe neurodevelopmental impairment (msNDI), Bayley Scores of Infant Development (BSID-III), and visual impairment. RESULTS: 1,231 patients from 11 comparative studies were included. Quality of evidence was rated low for all outcomes. IVB was associated with a higher risk for sNDI (risk ratio [RR] = 1.25, 95% confidence interval [CI]: [1.01, 1.53], p = 0.04); and CP (RR = 1.40, CI: [1.08, 1.81], p = 0.01) compared to LPC. There was no significant difference between IVB and LPC for msNDI (RR = 1.15, CI: [0.98, 1.35], p = 0.08) and HI (RR = 1.43, CI: [0.86, 2.39], p = 0.17). BSID-III percentile scores were similar between IVB and LPC, with weighted mean differences of 1.51 [CI = -1.25, 4.27], 2.43 [CI = -1.36, 6.22], and 1.97 [CI = -1.06, 5.01] for cognitive, language, and motor domains, respectively (p > 0.05). CONCLUSION: To our knowledge, this is the largest meta-analysis on neurodevelopmental outcomes and the first to rigorously examine IVB monotherapy in ROP treatment. Compared to LPC, there was a marginally increased risk for sNDI and CP with IVB but little or no difference in the risk of msNDI and HI. Further randomized studies are needed to strengthen these findings.

Rise in the incidence of severe pediatric blepharokeratoconjunctivitis during the COVID-19 pandemic.

Severe blepharokeratoconjunctivitis (BKC) is associated with vision loss and ocular morbidity; hence, early diagnosis and treatment are crucial. Retrospective data collection using electronic patient and billing database records of all patients <18 years of age with severe BKC between March 2010 and March 2022 was performed at the Eye Clinic at the Hospital for Sick Children in Toronto, Canada. Severe BKC was defined as including corneal inflammation, new vessel formation, scarring, thinning and lipid deposits. We excluded patients with mild or no corneal involvement, and those with other corneal pathologies. Over the study period, 257 patients were diagnosed with severe BKC (161 females), with an average age of 8 years. There was a statistically significant threefold increase in the diagnosis of severe BKC since the beginning of the COVID-19 pandemic in 2020 compared to the previous years. We speculate that the increased use of facial masks during the pandemic contributed to this significant increase.

Subdural hemorrhages and severe retinal hemorrhages in a short fall with a rotational component.

We present the case of a 5-month-old referred for child abuse investigation with subdural hemorrhages and extensive retinal hemorrhages following a short fall from a swivel chair seen on video footage. Subdural hemorrhages with extensive retinal hemorrhages are not typically seen as the result of short household falls. Reviewing the footage, contributing factors may have included increased rotational and deceleration forces.

Outcomes of cataract surgery in children who present with cataract at uveitis diagnosis.

PURPOSE: To describe the clinical and demographic characteristics of patients presenting with cataract at uveitis diagnosis treated at a single institution between 2005 and 2019 and to analyze postoperative outcomes following cataract surgery. METHODS: We retrospectively reviewed the medical records of children (<18 years of age) diagnosed with cataract at their initial uveitis presentation who subsequently underwent cataract extraction. Outcome measures were best-corrected visual acuity, number of uveitis flare-ups (cells ≥1+), and postoperative complications. RESULTS: A total of 14 children (17 eyes) were included. Mean patient age was 7.2 ± 3.9 years. Methotrexate was initiated preoperatively in 11 patients; adalimumab, in 3. Primary intraocular lens was implanted in 4 eyes. Best-corrected visual acuity improved from a mean of 0.90 ± 0.40 logMAR preoperatively to 0.50 ± 0.35 logMAR at 1 year and 0.57 ± 0.40 logMAR at mean of 6.3 ± 3.4 years postoperatively. Four eyes (24%) had a single episode of uveitis flare-up during the first postoperative year. Macular and/or disk edema was discovered in 6 eyes following cataract removal. Only 3 eyes (18%) had ocular hypertension in the first year, but glaucoma developed in subsequent years in 7 eyes (41%), 5 of which required surgery. CONCLUSIONS: In our study cohort, surgery for preexisting cataract at uveitis diagnosis resulted in improved visual acuity. Postoperative uveitis flare-ups were relatively uncommon, occurring in 4 of 17 eyes. Glaucoma was the main long-term complication.

Bilateral keratouveitis associated with COVID-19 multisystem inflammatory syndrome in children.

We report the case of a 16-year-old girl admitted to hospital with multisystem inflammatory syndrome in children (MIS-C) secondary to COVID-19. Conjunctivitis-like symptoms prompted ocular examination, which demonstrated peripheral confluent corneal opacities and anterior uveitis. Uveitis laboratory investigations were negative, and with topical steroid treatment her signs and symptoms resolved completely. These features may be overlooked in the setting of MIS-C, where patients are systemically unwell and are typically examined at the bedside.

Long-term outcomes of type 1 retinopathy of prematurity following monotherapy with bevacizumab: a Canadian experience.

OBJECTIVE: To report long-term structural, visual, and refractive outcomes after monotherapy with intravitreal bevacizumab injection. DESIGN: Cohort retrospective chart review. PARTICIPANTS: A total of 56 premature infants with type 1 retinopathy of prematurity. METHODS: This is a chart review at 2 Canadian institutions. Inclusion criteria were single injection of 0.625 mg  intravitreal bevacizumab and minimum age at last follow-up of 3 years. Primary outcome was retinal structure. Secondary outcomes were refractive error in spherical equivalent, monocular visual acuity, strabismus, and amblyopia. RESULTS: Fifty-six infants (101 eyes) met inclusion criteria. Mean birth weight was 707 ± 178 g (range, 420-1520 g). Mean gestational age was 25.0 ± 1.3 weeks (range, 22.9-29.7 weeks). Twenty-four eyes were in zone I (24%) and 77 in zone II (76%). Mean postmenstrual age at treatment was 36.9 ± 2.1 weeks (range, 32.8-42.0 weeks). At a mean age of 5.4 ± 1.6 years (range, 3.0-8.0 years), all eyes had a favourable structural outcome with no reactivation requiring treatment. Mean monocular visual acuity was 0.29 ± 0.27 logMAR (range, 0.0-1.3 logMAR; 89 of 101 eyes). Mean spherical equivalent was -1.98 ± 4.91 D (range, -16.63 to +5.38 D; 101 of 101 eyes). Prevalence of emmetropia (>-1.0 to ≤1 D) was 43.6%; low myopia (≥1.0 to <5 D) was 17.8%; high myopia (≥5 to <8 D) was 8.9 %; very high myopia (≥8.0 D) was 12.9%; and hyperopia (>1 D) was 16.8%. Twelve children (23%) had amblyopia, and 17 (32%) developed strabismus. CONCLUSIONS: All patients demonstrated a favourable structural outcome with a single bevacizumab injection without the need for additional laser. We suggest regular monitoring following regression of acute retinopathy of prematurity as an alternative to universal, preplanned delayed prophylactic laser treatment. Future studies to evaluate other aspects of visual function are needed.

A typical anterior retinoblastoma: diagnosis by aqueous humor cell-free DNA analysis.

BACKGROUND: Aqueous humor from eyes with active retinoblastoma contains tumor-derived cell-free DNA. MATERIALS AND METHODS: Single retrospective case report. RESULTS: A 13-year-old girl with acute right eye pain and redness was diagnosed with hypertensive anterior uveitis. Following initial management, she was referred to ocular oncology for an atypical clinical picture. Multiple seeds were noted 360 degrees in the anterior chamber, at the equator of the lens and canal of Petit, and ultrasound biomicroscopy identified a temporal pars plana lesion. While aqueous humor cytology was inconclusive for malignancy, targeted next-generation sequencing of aqueous cell-free DNA identified biallelic RB1 full gene deletion, confirming the diagnosis of retinoblastoma. Partial regression followed three cycles of systemic carboplatin, etoposide, and vincristine and three intracameral melphalan injections. Four months later, she had recurrence of the primary tumor and increase in seeding and received the investigational sustained release episcleral topotecan chemoplaque. Stable regression was achieved to 28-month follow-up, with no detectable aqueous cell-free DNA. CONCLUSIONS: RB1 sequencing analysis of tumor-derived cell-free DNA from aqueous humor can confirm the diagnosis of retinoblastoma in cases of diagnostic uncertainty.

Late surgery for unilateral persistent fetal vasculature: long-term visual and anatomic outcomes.

BACKGROUND: Persistent fetal vasculature (PFV) is a congenital anomaly caused by failure of the ocular vasculature to regress. We report the visual and anatomic outcomes in patients who underwent surgery for PFV between 7-48 months of age. METHODS: The medical records of patients who underwent lensectomy and anterior or core vitrectomy for unilateral PFV, with or without intraocular lens implantation, were reviewed retrospectively. Inclusion criteria were surgery between 7-48 months of age and at least 12 months of follow-up. Patients with severe posterior segment involvement were excluded. The primary outcome was the final visual acuity using age-appropriate tests converted to logMAR. Secondary outcomes included the rate of adverse events and number of subsequent intraocular procedures. RESULTS: A total of 20 patients with a mean age at surgery of 19.3 ± 10.5 months and mean follow-up of 73.7 ± 46.7 months were included. Sixteen patients had delayed surgery due to late presentation; the remaining 4 were managed initially with refractive correction and occlusion. Eight patients (40%) achieved a final visual acuity better than 1.0 logMAR. Four eyes had adverse events, including one retinal detachment. No patient developed glaucoma. Four eyes underwent subsequent procedures. CONCLUSIONS: In our study cohort, surgery for unilateral PFV between 7-48 months of age achieved functional visual acuity in over one-third of patients. This is comparable to results achieved with surgery before 7 months of age, but with fewer adverse events. Differences in disease severity could account for later PFV presentation and surgery as well as outcomes.

Unilateral cataract and congenital stationary night blindness in a child with novel variants in TRPM1.

Unilateral cataract can cause pediatric vision impairment. Although the majority of unilateral cataracts are idiopathic in nature, genetic causes have been reported. We present the case of a 4-week-old child of nonconsanguineous parents who was affected with unilateral cataract. Whole-genome sequencing using DNA extracted from blood and the lens epithelial cells following cataract surgery revealed two presumed pathogenic variants in the TRPM1 gene, the founding member of the melanoma-related transient receptor potential (TRPM) subfamily. TRPM1 is responsible for regulating cation influx to hyperpolarized retinal ON bipolar cells, and mutations in this gene are a major cause of autosomal recessive congenital stationary night blindness (CSNB). Electroretinography revealed findings consistent with CSNB, a phenotype that was not initially suspected, and which would likely have been missed without genome sequencing. It remains unclear whether the TRPM1 variants are associated with the cataract phenotype.

Evaluation of IOL power calculation with the Kane formula for pediatric cataract surgery.

PURPOSE: To assess the accuracy of the Kane formula for intraocular lens (IOL) power calculation in the pediatric population. METHODS: The charts of pediatric patients who underwent cataract surgery with in-the-bag IOL implantation with one of two IOL models (SA60AT or MA60AC) between 2012 and 2018 in The Hospital for Sick Children, Toronto, Ontario, CanFada, were retrospectively reviewed. The accuracy of IOL power calculation with the Kane formula was evaluated in comparison with the Barrett Universal II (BUII), Haigis, Hoffer Q, Holladay 1, and Sanders-Retzlaff-Kraff Theoretical (SRK/T) formulas. RESULTS: Sixty-two eyes of 62 patients aged 6.2 (IQR 3.2-9.2) years were included. The SD values of the prediction error obtained by Kane (1.38) were comparable with those by BUII (1.34), Hoffer Q (1.37), SRK/T (1.40), Holaday 1 (1.41), and Haigis (1.50), all p > 0.05. A significant difference was observed between the Hoffer Q and Haigis formulas (p = 0.039). No differences in the median and mean absolute errors were found between the Kane formula (0.54 D and 0.91 ± 1.04 D) and BUII (0.50 D and 0.88 ± 1.00 D), Hoffer Q (0.48 D and 0.88 ± 1.05 D), SRK/T (0.72 D and 0.97 ± 1.00 D), Holladay 1 (0.63 D and 0.94 ± 1.05 D), and Haigis (0.57 D and 0.98 ± 1.13 D), p = 0.099. CONCLUSION: This is the first study to investigate the Kane formula in pediatric cataract surgery. Our results place the Kane among the noteworthy IOL power calculation formulas in this age group, offering an additional means for improving IOL calculation in pediatric cataract surgery. The heteroscedastic statistical method was first implemented to evaluate formulas' predictability in children.

Ophthalmic involvement in PHACES syndrome: prevalence, spectrum of anomalies, and outcomes.

PURPOSE: To highlight prevalence, spectrum of anomalies, and outcome of ophthalmic involvement in PHACES syndrome (posterior fossa malformations, infantile hemangiomas, arterial, cardiac, eye, and sternal anomalies). METHODS: A retrospective, noncomparative, single-institution observational case series of children with PHACES was conducted from 2000 to 2019. Data on ocular presentations, interventions and visual outcomes were collected. Primary outcome measures were the frequency and spectrum of ocular involvement. Secondary outcomes were final visual acuity, long-term ocular sequelae, and frequency of surgical interventions. RESULTS: A total of 43 infants had PHACES, of whom 29 (67%) had periocular infantile facial hemangiomas (IFH) and 6 (14%) had primary ocular anomalies that were always ipsilateral to the IFH. Five patients (12%) met ocular PHACES-specific diagnostic criteria, including optic nerve (3), retinal vascular (1) and lenticular (2) anomalies. Non-PHACES-specific abnormalities were Peters anomaly (1), persistent pupillary membranes (2), dysmorphic optic nerves (1), and iris/choroidal hemangiomas (2). IFH-related periocular abnormalities were frequent: ptosis (29), proptosis (9), strabismus (6). Surgery was required in 8 of the 29 children: (strabismus [6], entropion [2], ptosis [2], and optical iridectomy [1]), all of whom had orbital/conjunctival hemangioma (P = 0.03). Final visual acuity (follow-up, 8.7 years) ranged between 20/20 and 20/80 in 26 of 29 patients. All patients with visual acuity worse than 20/200 (3/29 [10%]) had structural anomalies. CONCLUSIONS: Two-thirds of infants with PHACES have periocular IFH causing vision compromising complications of amblyopia and strabismus. Structural ocular anomalies exist in 1 of 7 patients and are always ipsilateral to the IFH. Long-term ophthalmic monitoring and management is required, and the majority of patients obtain good visual outcomes.

Minimally Invasive Corneal Neurotization Provides Sensory Function, Protects Against Recurrent Ulceration, and Improves Visual Acuity.

PURPOSE: To measure sensory recovery after minimally invasive corneal neurotization, and to identify and quantify the extent to which patient and technical factors influence sensory recovery, ulceration rate, and visual outcomes. DESIGN: Retrospective case series. METHODS: This study included 23 patients with neurotrophic keratopathy who underwent indirect corneal neurotization. The primary outcome measure was corneal sensitivity with Cochet-Bonnet aesthesiometry (CBA), and the secondary outcome measure was epithelial breakdown. RESULTS: Over a 7-year period, 28 eyes of 23 patients (mean age, 15.6 ± 13.6 years) were included in the study. The CBA measurements improved from 3.5 ± 9.1 mm at baseline to 44.1 ± 18.2 mm at 24 months after surgery (P < .001). Maximum CBA was reached after 11.1 ± 6.2 months (median, 9 months). Compared to eyes neurotized with a contralateral donor nerve, eyes with an ipsilateral donor nerve achieved a higher mean CBA (36.0 ± 10.9 vs 10.4 ± 14.0 mm, P = .001) at 3 months. Both the number of fascicles (Spearman correlation coefficient, rs -0.474, P = .11) and insertions (rs -0.458, P = .014) negatively correlated with the final CBA. Nine eyes (32.1%) experienced at least 1 episode of epithelial breakdown after surgery. Visual acuity improved in the neurotized corneas from logMAR 0.57 ± 0.79 at baseline to 0.39 ± 0.66 at 12 months (P = .043). CONCLUSIONS: Corneal sensation improves over time after corneal neurotization. There is resultant improvement in visual acuity and protection against epithelial breakdown. It is important to maximize sensory recovery to protect against recurrent ulceration.

Multiple giant retinal tears due to inflicted injury in a neonate.

Purpose: This case represents the longest follow-up period and youngest patient treated for multiple GRTs in the same eye associated with physical abuse. Observations: A 4-week-old otherwise healthy male presented with a constellation of unexplained injuries. Examination of the left eye revealed a mild lens opacity and a shallow retinal detachment with two giant retinal tears (GRTs) and no retinal hemorrhages. Examination of the right eye was unremarkable. Extensive investigations were negative for any underlying medical conditions. The constellation of injuries was felt to be due to physical abuse. The giant retinal tears were treated successfully with lens sparing pars plana vitrectomy. After long-term follow-up of 5 years, there was no cataract progression or development of glaucoma. Conclusions and importance: Clinicians should suspect child abuse in any pediatric patient with GRTs, with or without retinal hemorrhages, to ensure they are connected with the appropriate children's safeguarding society as soon as possible.

Demographic Risk Factors of Retinopathy of Prematurity: A Systematic Review of Population-Based Studies.

INTRODUCTION: Current national guidelines use gestational age (GA) and birth weight (BW) as their basis for retinopathy of prematurity (ROP) screening. The strength of association of these and other demographic risk factors is inconsistent across studies. This review aims to evaluate the strength of association of documented risk factors for ROP in large sample, population-based studies. METHODS: MEDLINE, EMBASE, and Cochrane Library were searched from January 2010 to May 2020. Original studies reporting the risk of ROP in a region and demographic risk factors were included. RESULTS: Eighteen studies comprising 342,005 infants were included. The overall risk of ROP in preterm infants was 18.8%. For every week decrease in GA, there was a median adjusted odds ratio (aOR) of 1.4 times (range 1.2-1.9) of developing ROP. For every 100-g decrease in BW, the median aOR was 1.8 times (range 1.2-2.7). Higher risk was found in infants with neonatal sepsis and bronchopulmonary dysplasia. The risk of any, severe, and treatment-requiring ROP was highest for 23 weeks GA, which was 66.5, 40.3, and 39.4%, respectively. Regions with higher neonatal mortality rates had the highest mean GA of infants with ROP. CONCLUSION: For every week decrease in GA and every 100-g decrease in BW, there was a median of 1.4 times and 1.8 times the odds of developing ROP, respectively. Further research is required to clarify the role of additional risk factors.