Amblyopia risk factors among pediatric patients in a hospital-based setting using photoscreening.

PURPOSE: The aim of our study was to determine the prevalence of amblyopia risk factors in children visiting the American University of Beirut Medical Center (AUBMC) using automated vision screening. METHODS: This was a hospital-based screening of 1102 children aged between 2 and 6 years. Vision screening was performed using PlusoptiX S12 over 2 years (2018-2020). The need for referral to a pediatric ophthalmologist was based on the amblyopia risk factors set forth by the American Association for Pediatric Ophthalmology and Strabismus. Referred patients underwent a comprehensive eye examination. RESULTS: A total of 1102 children were screened, 63 were referred for amblyopia risk factors (5.7%); 37/63 (59%) underwent comprehensive eye examination and 73% were prescribed glasses. Of the non-referred group of children, 6.35% had astigmatism, 6.25% were hyperopic and 3.27% were myopic. The refractive errors observed among the examined patients were distributed as follows: 41% astigmatism, 51% hyperopia, and 8% myopia; amblyopia was not detected. Refractive amblyopia risk factors were associated with the presence of systemic disorders. Bland-Altman plots showed most of the differences to be within limits of agreement. CONCLUSION: Using an automated vision screener in a hospital-based cohort of children aged 2 to 6 years, the rate of refractive amblyopia risk factors was 5.7%. Hyperopia was the most commonly encountered refractive error and children with systemic disorders were at higher risk.

KIF21A pathogenic variants cause congenital fibrosis of extraocular muscles type 3.

Background: Congenital fibrosis of the extraocular muscles (CFEOM) is characterized by ptosis and non-progressive restrictive ophthalmoplegia. CFEOM1 is a stereotypical phenotype with isolated bilateral ptosis, bilateral ophthalmoplegia, absent upgaze, and globe infraduction. CFEOM3 is a more variable phenotype that can include unilateral disease, absent ptosis, residual upgaze, and/or orthotropia. Most cases of CFEOM1 result from recurrent heterozygous KIF21A missense mutations and less commonly from recurrent heterozygous TUBB3 missense mutations. While most cases of CFEOM3 result from recurrent heterozygous TUBB3 missense mutations, several pedigrees harbored pathogenic variants in KIF21A. Here, we asked if Lebanese pedigrees with CFEOM3 harbor pathogenic variants in TUBB3 or KIF21A.Materials and Methods: Families affected with congenital cranial dysinnervation disorders were prospectively recruited from the American University of Beirut pediatric ophthalmology clinic and included two probands with CFEOM. KIF21A hotspot exons and TUBB3 coding sequence were sequenced. Available family members were sequenced for co-segregation analysis.Results: Both families were found to have CFEOM3 and to harbor pathogenic variants in KIF21A(OMIM 608283). A simplex proband with CFEOM3 from a consanguineous Iraqi family harbored a de novo heterozygous KIF21A c.2860 C > T variant (p.R954W); this variant accounts for the majority of reported KIF21A mutations but is typically implicated in CFEOM1. A Lebanese father with CFEOM3 and his son with CFEOM1 segregated a heterozygous KIF21A c.2830 G > C variant (p.E944Q), previously reported in an individual with CFEOM1.Conclusions: These results support prior reports of KIF21A mutations as a rare cause of CFEOM3. These families are Middle Eastern or Chinese, supporting a genetic modifier in these populations.

Recurrence of Intermittent Exotropia after Bilateral Lateral Rectus Recession.

PURPOSE: The purpose of this study was to evaluate predictive factors for intermittent exotropia (XT) recurrence after bilateral lateral rectus (BLR) recession. METHODS: This is a retrospective chart review of patients with XT who underwent BLR recession surgery between January 2007 and March 2017 with at least one postsurgical follow-up. Forty-one medical records were reviewed. Information collected included age, gender, systemic diseases, history of prematurity, family history of eye diseases, visual acuity, refraction, ocular alignment and control, stereoacuity, slit-lamp examination, fundoscopy, and amount of BLR recession. Successful alignment was defined as ≤8 prism diopters of esotropia or exotropia postoperatively. RESULTS: The mean age of patients at the time of surgery and follow-up time was 9.2 ± 12.3 years (y) and 23.6 ± 36.5 months (m), respectively. The mean amount of BLR recession was 6.5 ± 1.0 mm. Recurrence rate was 43.9% on the last follow-up. Age at surgery and at the time of last follow-up were significantly higher in the recurring group (P = 0.04 and P = 0.05, respectively). Postoperative angle of misalignment during the first 3 months was correlated with exotropia recurrence. No statistical significance was found among the remaining factors studied. CONCLUSIONS: The recurrence rate of XT in our study was 43.9%; it was increased in patients operated at older age and amid those with significant exotropia detected in the early postoperative period (within 3 months of surgery).

Multifocal electroretinography in amblyopia.

PURPOSE: To identify whether there are functional abnormalities in the retina of amblyopic eyes using multifocal electroretinography (mfERG). METHODS: This is a prospective study of patients ≥ 7 years of age identified with unilateral amblyopia (strabismic or anisometropic). Multifocal ERG and flash ERG were performed to compare parameters between the amblyopic and non-amblyopic fellow eyes. A complete analysis of the five ring averages was done including the central ring. RESULTS: Thirty-eight patients were included: mean age was 14.3 ± 7.3 years; 18 patients were strabismic and 20 were anisometropic. Amblyopic eye responses across the rings in multifocal ERG were diminished compared with fellow non-amblyopic eyes with significant differences detected in the central rings (p = 0.001). On the other hand, flash ERG did not show any consistently significant differences. When divided by severity, amplitudes of central rings were significantly lower in severely amblyopic eyes compared with non-amblyopic eyes (p = 0.001), while in mild amblyopia, no significant differences were observed. No significant difference was observed between anisometropic and strabismic amblyopic eyes. CONCLUSIONS: Using multifocal ERG, significantly decreased amplitudes were observed in amblyopic eyes compared with normal fellow eyes in the central ring. This correlated with the severity of amblyopia. No difference was observed when comparing the two groups of amblyopia (strabismic and anisometropic). Those findings may help clarify the pathophysiology of amblyopia better and open the door for new objective ways to monitor the response to amblyopia treatment but this needs to be further studied.

Clinical Profile and Treatment Outcomes of Amblyopia Across Age Groups.

PURPOSE: The purpose is to study the clinical profile of amblyopia by age at diagnosis. SUBJECTS AND METHODS: A retrospective chart review of 327 amblyopic patients over 7 years (September 2009-December 2016) was performed, divided by age at diagnosis into four groups: <3, 3-7, 8-15, and >15 years. Demographics, eye conditions and eye examination parameters including visual acuity (VA), refractive errors, and motility measurements were collected. RESULTS: Mean age at diagnosis and follow-up time was 6.2 ± 6.1 years standard deviation (SD) and 12.4 months ± 20.6 SD, respectively. The most common overall cause of amblyopia was strabismus (37%) followed by anisometropia (36%). The main causes by age at diagnosis were: strabismus at <3 years, anisometropia at 3-7 years, anisometropia at 8-15 years, and mixed at >15 years. Significant improvement in VA with treatment was noted with age between 3 and 15 years (3-7 years, P = 0.001 and 8-15 years, P = 0.03). CONCLUSIONS: Strabismus was the main cause of amblyopia at <3 years of age; anisometropia was more prevalent in older children (3-15 years). The fact that more than a quarter of our amblyopic patients were detected late (after the age of 8 years) underscores the need for expanded vision screening measures in young children.

High-Definition Optical Coherence Tomography of the Macula in Deprivational Amblyopia.

BACKGROUND AND OBJECTIVE: This study evaluated macular thickness in deprivational amblyopia and compared it to matched controls. PATIENTS AND METHODS: The authors present a cross-sectional study of patients with amblyopia from pediatric cataract and matched controls. Macular high-definition optical coherence tomography scans of each eye were acquired. Central macular thickness (CMT) and parafoveal thicknesses at 500 µm, 1,000 µm, and 1,500 µm in the nasal, temporal, inferior, and superior locations were measured. RESULTS: Thirty-four eyes were included: 14 eyes with deprivational amblyopia and 20 controls. In amblyopes, the mean age was 10.06 years ± 3.89 years and logMAR visual acuity (VA) was 0.41 ± 0.53, whereas in the control group, mean age was 8.96 years ± 1.89 years and mean logMAR VA was 0.03 ± 0.05. The macula in deprivational amblyopia was significantly thicker centrally compared to controls (P = .0013), but only tended to be thicker at 500 µm and thinner at 1,000 µm and 1,500 µm. Male gender, poorer VA, and hyperopic refraction positively correlated with CMT (P = .011, P = .018, and P = .038, respectively). CONCLUSION: Eyes with deprivational amblyopia had increased CMT compared to controls, and this correlated with the severity of amblyopia. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:198-204.].

Long-term outcomes of Ahmed tube implantation in pediatric glaucoma after multiple surgeries.

PURPOSE: To report success of Ahmed glaucoma valve (AGV) implantation in eyes with pediatric glaucoma and subconjunctival scarring from previous surgeries. METHODS: A retrospective review of pediatric glaucoma patients who underwent AGV implantation after ≥ 2 previous surgeries with conjunctival scarring. Preoperative and postoperative parameters were recorded to study success, defined as intraocular pressure (IOP) < 22 mmHg with or without medications and without serious complications. RESULTS: Eleven eyes of 10 patients were included, and the mean age was 12.7 years. Diagnoses included primary childhood glaucoma, aphakic glaucoma, and anterior segment anomalies. Mean number of prior ocular surgeries was 3.3 (± 1.3). Final mean IOP was 17.5 (± 4.3) mmHg, significantly different from the preoperative mean of 24.8 (± 6.8) mmHg, p = 0.01. Mean number of anti-glaucoma medications also decreased significantly from 2.8 (± 1.5) to 1.4 (± 1.5), p = 0.02. The success rate was 82% at a mean follow-up of 51.6 (± 10.8) months. Complications were minor and transient. CONCLUSION: Subconjunctival scarring did not increase the risk of failure of tube shunt surgery.

Fornix-Based Versus Limbal-Based Conjunctival Trabeculectomy Flaps for Glaucoma: Findings From a Cochrane Systematic Review.

PURPOSE: To compare effectiveness of fornix- and limbal-based conjunctival flaps in trabeculectomy surgery. DESIGN: Systematic review. METHODS: Setting: CENTRAL, MEDLINE, LILACS, ISRCTN registry, ClinicalTrials.gov, WHO, and ICTRP were searched to identify eligible randomized controlled trials (RCTs). STUDY POPULATION: RCTs in which benefits and complications of fornix- vs limbal-based trabeculectomy for glaucoma were compared in adult glaucoma patients. OBSERVATION PROCEDURE: We followed Cochrane methodology for data extraction. MAIN OUTCOME MEASURES: Proportion of failed trabeculectomies at 24 months, defined as the need for repeat surgery or uncontrolled intraocular pressure (IOP) >22 mm Hg, despite topical/systemic medications. RESULTS: The review included 6 trials with a total of 361 participants, showing no difference in effectiveness between fornix-based vs limbal-based trabeculectomy surgery, although with a high level of uncertainty owing to low event rates. In the fornix-based and limbal-based surgery, mean IOP at 12 months was similar, with ranges of 12.5-15.5 mm Hg and 11.7-15.1 mm Hg, respectively. Mean difference was 0.44 mm Hg (95% CI -0.45 to 1.33) and 0.86 mm Hg (95% CI -0.52 to 2.24) at 12 and 24 months of follow-up, respectively. Mean number of postoperative glaucoma medications was similar between the 2 groups. Mean difference was 0.02 (95% CI -0.15 to 0.19) at 12 months. As far as postoperative complications, an increased risk of shallow anterior chamber was observed in the limbal-based group. CONCLUSION: Similar efficacy of trabeculectomy surgery with respect to bleb failure or IOP control was observed in both types of conjunctival flap incisions. A significant difference was detected in the risk of postoperative shallow anterior chamber, which was increased in the limbal-based group.

Choroidal Changes in Anisometropic and Strabismic Children With Unilateral Amblyopia.

BACKGROUND AND OBJECTIVE: To detect changes in the choroidal layer at the macular area in amblyopic eyes. PATIENTS AND METHODS: A cross-sectional study of 50 amblyopic patients (20 strabismic and 30 anisometropic) and 50 controls was done. Cross-sectional images using enhanced depth optical coherence tomography (OCT) were taken. Thicknesses were measured subfoveally and at 1,500 µm nasally, temporally, inferiorly, and superiorly. Submacular corresponding choroidal areas were also computed. Parameters were compared between amblyopic eyes, fellow eyes, and controls. RESULTS: Significantly thicker choroid was detected in the subfoveal, temporal, and nasal locations (P = .007, .009, and .01, respectively) in amblyopic compared to fellow eyes; areas were also significantly greater temporally, nasally, and inferiorly. Significant differences in all choroidal measurements were found between amblyopic eyes and controls; these persisted only in the anisometropic subgroup. CONCLUSION: Using enhanced depth OCT, the choroid of amblyopic eyes was observed to be thicker compared to normal fellow eyes and controls. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:900-907.].