Capillary Hemangioma Presenting as a Scleral Vascular Lesion in a Child.

A 7-year-old healthy girl presented for an evaluation of a left vascular scleral mass. The lesion appeared spontaneously with no history of trauma, coagulopathy, or topical medication use. It was nontender, enlarging, and did not extend intraocularly. Her OS vision was 20/20, and the remainder of her eye examination was normal. Evaluation of the ocular mass included B-scan ultrasound, ultrasound biomicroscopy, anterior segment optical coherence tomography (OCT), and orbital MRI. The anterior segment OCT demonstrated vessels within the mass with no defined capsule. The orbital MRI confirmed a lesion isolated to the scleral layers of the globe, with low blood flow. The patient had a partial response to oral propranolol. Because the lesion vessels began to extend into her corneal endothelium, there was a concern for malignancy. A biopsy confirmed a benign intrascleral capillary hemangioma. Discontinuation of the propranolol demonstrated stability of the lesion 6 months later.

Cataract Formation in a Child Receiving Dabrafenib and Trametinib Therapy.

The authors report a case of bilateral cataract in a 9-year-old girl after being treated with a combination of the targeted therapy drugs dabrafenib and trametinib. Although adverse effects have been reported with this treatment, this report is the first documented case of cataract as a complication. [J Pediatr Ophthalmol Strabismus. 2024;61(5):e47-e49.].

Improved Clinic Flow and Satisfaction After Lean Implementation in a Pediatric Ophthalmology Clinic.

PURPOSE: To report outcomes using Lean concepts (FlowOne Lean Consulting, LLC) and implemented changes in a single academic pediatric ophthalmology practice. METHODS: Lean principles such as patient flow, wasted movement, and non-value added visit time were taught to clinic staff. Spaghetti maps to document patient and staff movement during examinations, patient simulation, and clinic flow exercises were performed prior to implementing clinic changes. Clinic changes included maximizing easily reachable equipment and standardizing equipment in each examination room, physician-controlled doorbells to notify staff for help, and implementing a visual real-time electronic board of patients throughout the entirety of their examination. Patient surveys before and after Lean and staff feedback were obtained. RESULTS: Four years of data were collected of patient examination visits after Lean principles were introduced. Prior to Lean implementation, mean pediatric ophthalmology clinic visit times were 120 minutes. Within 3 months of Lean clinic changes, mean visit times reduced to 75 minutes. Four years later, this was sustained at 69 minutes. Clinic templates were modified and increased by 17%. Patient satisfaction surveys before and after demonstrated improved scores from 6.5 to 9.0. Patient survey comments included satisfaction with shorter patient examination visits, less shuffling between examination rooms, less non-value added time, and perception of happier clinic staff. Staff survey comments included receiving fewer parent complaints, feeling less disorganized, being able to troubleshoot in real time, and leaving at an earlier time at the end of the clinic day. CONCLUSIONS: Lean improved clinic efficiency, reduced patient visit times, and improved patient and staff satisfaction. These improvements were sustained 4 years after Lean principles were implemented. [J Pediatr Ophthalmol Strabismus. 2024;61(1):14-19.].

Anterior segment optical coherence tomography (AS-OCT) in strabismus following trauma.

We describe 3 cases of ocular trauma that presented with strabismus. Anterior segment optical coherence tomography was used to delineate the morphology of extraocular muscle insertions and proved helpful in diagnosis and management.

Identification and treatment of amblyopia.

Amblyopia is the leading cause of vision loss in children. It is treatable if diagnosed early, making identification of affected children critical. The American Association for Pediatric Ophthalmology and Strabismus and the American Academy of Pediatrics recommend that clinicians routinely perform age-appropriate vision chart testing, red reflex testing, and examination for signs of strabismus. The U.S. Preventive Services Task Force recommends vision screening for all children at least once between three and five years of age to detect the presence of amblyopia or its risk factors. Photoscreening may be a useful adjunct to traditional vision screening, but there is limited evidence that it improves visual outcomes. Treatments for amblyopia include patching, atropine eye drops, and optical penalization of the nonamblyopic eye. In children with moderate amblyopia, patching for two hours daily is as effective as patching for six hours daily, and daily atropine is as effective as daily patching. Children older than seven years may still benefit from patching or atropine, particularly if they have not previously received amblyopia treatment. Amblyopia recurs in 25 percent of children after patching is discontinued. Tapering the amount of time a patch is worn each day at the end of treatment reduces the risk of recurrence.

Coats' Disease in a Patient With Cornelia de Lange Syndrome: Management With Laser and Bevacizumab.

Cornelia de Lange syndrome is a congenital disorder with multisystem abnormalities including multiple ocular findings. The authors report a case of Coats' disease in a patient with Cornelia de Lange syndrome who was successfully treated with laser and intravitreal bevacizumab. This case demonstrates the importance of fluorescein angiography in making the diagnosis and directing treatment and the efficacy of combined laser with intravitreal anti-vascular endothelial growth factor therapy for persistent vascular leakage associated with Coats' disease in Cornelia de Lange syndrome. [J Pediatr Ophthalmol Strabismus. 2023;60(4):e45-e48.].

Use of sweep visual evoked potential in preverbal children with optic nerve hypoplasia.

PURPOSE: To evaluate sweep VEP (sVEP) in preverbal children with optic nerve hypoplasia (ONH) and to assess associations between sVEP results, patient clinical characteristics and future recognition visual acuity. METHODS: The medical records of children with ONH who had sVEP testing and documented recognition visual acuity at the University of Wisconsin from 2005 to 2013 were reviewed retrospectively. Optic nerve size, amblyopia treatment, and neurologic diagnoses were collected. RESULTS: A total of 57 patients were included: 41 (71%) with bilateral ONH and 27 (47%) with neurologic abnormality. Mean age at initial sVEP was 13.3 months (range, 1-32). Mean duration between initial sVEP and final recognition acuity was 5.5 years (range, 3.5-7). Sweep VEP was associated with ONH severity (P < 0.05). Sweep VEP, and the combination of ONH severity and neurologic status, were significant predictors (P < 0.05) of logMAR optotype acuity, together accounting for 54%-61% of the variance in final recognition acuity. CONCLUSIONS: Sweep VEP in preverbal children with ONH depends on ONH severity and correlates with final recognition visual acuity. Children with milder degrees of ONH without neurologic abnormalities had better final vision, and patients with severe ONH and neurologic diagnoses had worse vision outcomes.

Opioid prescribing to preteen children undergoing ambulatory surgery in the United States.

BACKGROUND: Overuse and misuse of opioids is a continuing crisis. The most common reason for children to receive opioids is postoperative pain, and they are often prescribed more than needed. The amount of opioids prescribed varies widely, even for minor ambulatory procedures. This study uses a large national sample to describe filled opioid prescriptions to preteen patients after all ambulatory surgical procedures and common standard procedures. METHODS: We analyzed Truven Health MarketScan data for July 2012 through December 2016 to perform descriptive analyses of opioid fills by age and geographic area, change over time, second opioid fills in opioid-naïve patients, and variation in the types and amount of medication prescribed for 18 common and standard procedures in otolaryngology, urology, general surgery, ophthalmology, and orthopedics. RESULTS: Over 10% of preteen children filled perioperative opioid prescriptions for ambulatory surgery in the period 2012 to 2016. The amount prescribed varied widely (median 5 days' supply, IQR 3-8, range 1-90), even for the most minor procedures, for example, frenotomy (median 4 days' supply, IQR 2-5, range 1-60). Codeine fills were common despite safety concerns. Second opioid prescriptions were filled by opioid-naïve patients after almost all procedures studied. The rate of prescribing declined significantly over time and varied substantially by age and across census regions. CONCLUSIONS: We identified opioid prescribing outside of the norms of standard practice in all of the specialties studied. Standardizing perioperative opioid prescribing and developing guidelines on appropriate prescribing for children may reduce the opioids available for misuse and diversion.

SVEP1 as a Genetic Modifier of TEK-Related Primary Congenital Glaucoma.

Purpose: Affecting children by age 3, primary congenital glaucoma (PCG) can cause debilitating vision loss by the developmental impairment of aqueous drainage resulting in high intraocular pressure (IOP), globe enlargement, and optic neuropathy. TEK haploinsufficiency accounts for 5% of PCG in diverse populations, with low penetrance explained by variable dysgenesis of Schlemm's canal (SC) in mice. We report eight families with TEK-related PCG, and provide evidence for SVEP1 as a disease modifier in family 8 with a higher penetrance and severity. Methods: Exome sequencing identified coding/splice site variants with an allele frequency less than 0.0001 (gnomAD). TEK variant effects were assayed in construct-transfected HEK293 cells via detection of autophosphorylated (active) TEK protein. An enucleated eye from an affected member of family 8 was examined via histology. SVEP1 expression in developing outflow tissues was detected by immunofluorescent staining of 7-day mouse anterior segments. SVEP1 stimulation of TEK expression in human umbilical vascular endothelial cells (HUVECs) was measured by TaqMan quantitative PCR. Results: Heterozygous TEK loss-of-function alleles were identified in eight PCG families, with parent-child disease transmission observed in two pedigrees. Family 8 exhibited greater disease penetrance and severity, histology revealed absence of SC in one eye, and SVEP1:p.R997C was identified in four of the five affected individuals. During SC development, SVEP1 is secreted by surrounding tissues. SVEP1:p.R997C abrogates stimulation of TEK expression by HUVECs. Conclusions: We provide further evidence for PCG caused by TEK haploinsufficiency, affirm autosomal dominant inheritance in two pedigrees, and propose SVEP1 as a modifier of TEK expression during SC development, affecting disease penetrance and severity.

Acquired Pediatric Brown Syndrome Secondary to Sinusitis.

Acquired pediatric Brown syndrome is rarely associated with sinusitis. Children may present with diplopia, elevation deficiency in adduction, and a head tilt or chin-up position. Magnetic resonance imaging can confirm the diagnosis. Antibiotic treatment is often insufficient for resolution. Surgical correction can effectively prevent amblyopia and restore binocular vision. [J Pediatr Ophthalmol Strabismus. 2019;56:e17-e19.].

Comparative Intraoperative Anterior Segment OCT Findings in Pediatric Patients with and without Glaucoma.

PURPOSE: To compare the angle structures during surgery using anterior segment (AS) OCT in children with and without glaucoma. DESIGN: Prospective comparative study, with single masked grader evaluating AS OCT images. PARTICIPANTS: This study included a total of 44 eyes of 8 children with glaucoma and 14 children without glaucoma undergoing ophthalmic surgery with general anesthesia. The median age for the glaucoma group was 6 months (range, 0.5-108 months), and that for the nonglaucoma group was 42 months (range, 14-95 months). METHODS: An Envisu C2300 handheld OCT with a high-resolution AS lens was used to capture videos of temporal and nasal quadrant anterior chamber angles from each eye. Still images of the temporal and nasal angles were selected and then graded by a masked ophthalmologist examiner to determine whether Schlemm's canal (SC) was visible, if abnormal tissue was present in the angle, and whether intrascleral lumens were present. Clinical correlation with severity of glaucoma was performed after AS OCT images were graded. MAIN OUTCOME MEASURES: Presence or absence of SC on AS OCT in glaucomatous and nonglaucomatous eyes. RESULTS: Schlemm's canal was present in 7 of 13 glaucomatous eyes compared with 27 of 27 nonglaucomatous eyes. In patients with unilateral glaucoma, absence of SC was noted only in the affected eye. Eight eyes with glaucoma showed abnormal tissue over the angle and SC. Intrascleral lumen and collector channels were found in both groups. Two patients with severe stenosis of SC during glaucoma angle surgery showed absent SC on AS OCT. CONCLUSIONS: Intraoperative AS OCT identified differences in the presence and characteristics of SC and the anterior chamber angle in pediatric patients with and without glaucoma. Further studies are needed to determine clinical correlation.

Validation of Peek Acuity application in pediatric screening programs in Paraguay.

AIM: To validate the Peek Acuity mobile phone application in pediatric populations and compare its utility, both economic and diagnostic, against conventional screening methods using a pediatric ophthalmologist examination as the gold standard. METHODS: A cohort of 393 subjects from Fernando de la Mora, Paraguay (ages 6-16y) were enrolled in the study. Subjects were randomly assigned a starting screening modality among: Peek Acuity, a single line of tumbling E optotypes set at 20/40, and Spot Vision Screener. Once completing the first screening modality, the subjects completed the two remaining techniques. Referral criteria were established based on the most current American Association of Pediatric Ophthalmology and Strabismus (AAPOS) recommendations: 20/40 for Peek Acuity and the tumbling E, and refractive error detection for the Spot Vision Screener. Subjects that failed to achieve the cut-off for any of the three screening techniques or subjects that passed the screening but were randomly selected to perform a comprehensive eye exam to determine the false negative rate, were evaluated by a pediatric ophthalmologist. This evaluation was considered the gold standard, and included vision assessment by a Snellen chart, strabismus evaluation, and cycloplegic refraction with dilated fundoscopy. RESULTS: We obtained 48% sensitivity, 83% specificity, 43% positive predictive value, and 86% negative predictive value for Peek Acuity's ability to refer compared to evaluation by a pediatric ophthalmologist, failing to achieve a desired sensitivity for implementation. Peek Acuity trended to overestimate the subject's visual acuity, providing a higher visual acuity that would not indicate referral for a comprehensive eye examination. However, its high specificity accurately predicted a significant number of children who did not need further evaluation. When comparing the three screening methods, no single screening modality outperformed the others. Peek Acuity represented a technology that was economically feasible compared to other screening modalities in low income settings, due to the prevalence of cell phone use. CONCLUSION: Peek Acuity represents an efficient tool that has potential for implementation in school screenings with different strategies aimed at pediatric populations due to its low cost and high specificity. An increase in sensitivity would improve detection of children with refractive errors.

Pseudotumor cerebri in children with Down syndrome.

PURPOSE: To report 4 pediatric Down syndrome patients with optic nerve elevation and pseudotumor cerebri. DESIGN: Retrospective observational case series. PARTICIPANTS: Four pediatric Down syndrome patients found to have optic nerve elevation on ophthalmologic examination at a single institution. METHODS: Medical record review of 117 pediatric Down syndrome patients aged 0 to 18 years was performed. All patients were seen at the University of Wisconsin pediatric ophthalmology clinic between 1995 and 2005. MAIN OUTCOME MEASURES: Visual acuity, optic nerve appearance, and neurologic work-up. RESULTS: Four (3.4%) of 117 Down syndrome patients seen at the university's pediatric ophthalmology clinic were found to have optic nerve elevation. This included 2 boys and 2 girls, aged 2 to 10 years. None of the patients had a history of headache or transient visual obscuration. One of the patients had high hyperopia at the time of presentation, and all were referred for neurologic evaluation of suspected increased intracranial pressure. Three patients were diagnosed with pseudotumor cerebri (PTC) based on a normal brain magnetic resonance imaging (MRI) scan, an elevated opening cerebrospinal fluid (CSF) pressure on lumbar puncture, and normal CSF analysis results. The fourth patient's elevated optic nerve appearance was likely secondary to PTC after obtaining normal brain MRI results; however, this patient did not undergo a lumbar puncture. One child with PTC demonstrated improvement in visual acuity and optic nerve appearance after acetazolamide and weight loss therapy. The 2 additional patients with PTC were treated with either low-dose acetazolamide or weight loss therapy and experienced optic atrophy. The final patient, who did not undergo lumbar puncture, experienced spontaneous resolution of optic nerve elevation. Mean follow-up duration was 49 months. CONCLUSIONS: Only 3.4% (4/117) of Down syndrome children were found to have optic nerve elevation in a single institution. Pseudotumor cerebri should be considered in asymptomatic Down syndrome patients with elevated optic nerves. Medical therapy in PTC with acetazolamide can lead to improvement in visual acuity and optic nerve appearance, although optic atrophy also was seen.

Factors associated with recurrence of amblyopia on cessation of patching.

PURPOSE: In a prospective observational study, we previously reported that weaning (tapering or gradually reducing) treatment in children treated with 6 to 8 hours of daily patching for amblyopia resulted in a 4-fold reduction in odds of recurrence. We now report the association of additional factors with recurrence or regression of amblyopia in this same cohort. DESIGN: Prospective, nonrandomized, observational study. PARTICIPANTS: Sixty-nine children aged <8 years with successfully treated anisometropic or strabismic amblyopia (improved > or =3 logarithm of the minimum angle of resolution [logMAR] lines). METHODS: Patients were enrolled at the time they stopped patching for amblyopia. Patients were classified according to whether patching was stopped abruptly or weaned before cessation. They were followed off treatment for 52 weeks to assess recurrence of amblyopia. MAIN OUTCOME MEASURE: Recurrence of amblyopia defined as a > or =2-logMAR level reduction of visual acuity from enrollment (cessation of patching) confirmed by a second examination. Recurrence was also considered to have occurred if treatment was restarted with a > or =2-logMAR level reduction of visual acuity, even if it was not confirmed by a second examination. RESULTS: The risk of recurrence was higher with better visual acuity at the time of cessation of treatment (adjusted risk ratio [RR], 0.68 per line of worse visual acuity; 95% confidence interval [CI], 0.51-0.90), a greater number of lines improved during the previous treatment (adjusted RR, 1.5 per line increase; 95% CI, 1.1-2.0), and a history of recurrence (adjusted RR, 2.7; 95% CI, 1.5-4.9). Orthotropia or excellent stereoacuity at the time of patching cessation did not appear to have a protective effect on the risk of recurrence. CONCLUSIONS: The higher risk of recurrence in the most successfully treated children with amblyopia and absence of protection from orthotropia and excellent random dot stereoacuity suggests that careful and prolonged follow-up is needed for all children who have been previously treated for amblyopia.

Sweep visual evoked potential testing as a predictor of recognition acuity in albinism.

OBJECTIVE: To determine if sweep visual evoked potential (VEP) acuity is predictive of recognition acuity in children with albinism. METHODS: A retrospective review was performed in children with albinism who underwent sweep VEP testing from 1992 to 2003. All patients had a complete ophthalmologic examination with either binocular or monocular sweep VEP testing and at least 5 years of follow-up. Positive predictability of sweep VEP acuity was defined as final recognition acuity within 1 Snellen line of initial sweep VEP acuity. RESULTS: Of the 13 patients included in the study, 11 had nystagmus, iris transillumination defects, and foveal hypoplasia at initial examination. The mean age at initial sweep VEP testing was 3.1 years (range, 0.1-10.0 years). Five of 13 patients had initial sweep VEP acuity that was predictive of final recognition acuity. Five additional patients had final recognition acuity, which surpassed initial sweep VEP acuity by 2 to 3 lines. Of these 10 patients, the mean duration for recognition acuity to reach VEP acuity was 5.4 years. There was no correlation between predictive VEP acuity and foveal pigmentation, refractive error, strabismus, nystagmus, or longer follow-up. CONCLUSIONS: Sweep VEP testing can be used as a predictive tool for recognition acuity in children with albinism. Predictability was found in a clinical spectrum of albinism.

Chorioretinopathy and microcephaly with normal development.

PURPOSE: To report a pediatric patient with bilateral chorioretinopathy and microcephaly who from birth to 2 years of age is reaching appropriate developmental milestones. DESIGN: Retrospective case report with clinical findings and literature review. MAIN OUTCOME MEASURES: Clinical findings and visual acuity estimated by sweep visual evoked potentials (VEP), electroretinogram (ERG) and fundoscopic exam. RESULTS: A microcephalic child with normal motor and cognitive development had improving sweep VEP despite atypical fundoscopic findings of bilateral chorioretinopathy, attenuated retinal vessels, and anomalous optic nerves. The etiology for these collective findings despite extensive workup, including prenatal TORCH titers and neuro-imaging, has remained unidentified. CONCLUSIONS: Most published cases of microcephaly with chorioretinopathy have described patients with mild to severe mental retardation. Patients with chorioretinopathy and microcephaly may, however, reach all developmental milestones with improvement in visual development as was seen in this case. The long-term cognitive and visual prognosis may be better than previously reported.

A 3-month safety and efficacy study of travoprost 0.004% ophthalmic solution compared with timolol in pediatric patients with glaucoma or ocular hypertension.

PURPOSE: To evaluate efficacy and safety of travoprost in pediatric patients with ocular hypertension or glaucoma and demonstrate its noninferiority to timolol. METHODS: Patients aged 2 months to <18 years with glaucoma or ocular hypertension were randomized to receive travoprost (0.004%) or timolol eye drops (0.25% for patients aged 2 months to <3 years and 0.5% for patients ≥3 years old) for 3 months in this double-masked, parallel-group study. Intraocular pressure (IOP) was measured and patients were evaluated at 2 weeks, 6 weeks, and 3 months after treatment. Change in IOP from baseline to 3 months was the primary endpoint, and the test of noninferiority was based on a margin of +3.0 mm Hg using the 95% 2-sided confidence interval of the mean change. RESULTS: Of 157 patients included (mean age, 9.6 years), 77 received travoprost and 75 timolol. All patients experienced a significant reduction in IOP in the study eye at 3 months: the mean IOP change from baseline was -5.4 mm Hg for travoprost; -5.3 mm Hg, for timolol. The mean difference between travoprost and timolol at month 3 was -0.1 mm Hg (95% CI, -1.5 to 1.4 mm Hg). The most common treatment-related adverse events for the travoprost group were ocular hyperemia and eyelash growth. No serious adverse events were reported. CONCLUSIONS: This study found travoprost to be noninferior to timolol in lowering IOP in patients with pediatric glaucoma or ocular hypertension. Travoprost was well-tolerated, and no treatment-related systemic adverse events were reported.

Angiopoietin-1 is required for Schlemm's canal development in mice and humans.

Primary congenital glaucoma (PCG) is a leading cause of blindness in children worldwide and is caused by developmental defects in 2 aqueous humor outflow structures, Schlemm's canal (SC) and the trabecular meshwork. We previously identified loss-of-function mutations in the angiopoietin (ANGPT) receptor TEK in families with PCG and showed that ANGPT/TEK signaling is essential for SC development. Here, we describe roles for the major ANGPT ligands in the development of the aqueous outflow pathway. We determined that ANGPT1 is essential for SC development, and that Angpt1-knockout mice form a severely hypomorphic canal with elevated intraocular pressure. By contrast, ANGPT2 was dispensable, although mice deficient in both Angpt1 and Angpt2 completely lacked SC, indicating that ANGPT2 compensates for the loss of ANGPT1. In addition, we identified 3 human subjects with rare ANGPT1 variants within an international cohort of 284 PCG patients. Loss of function in 2 of the 3 patient alleles was observed by functional analysis of ANGPT1 variants in a combined in silico, in vitro, and in vivo approach, supporting a causative role for ANGPT1 in disease. By linking ANGPT1 with PCG, these results highlight the importance of ANGPT/TEK signaling in glaucoma pathogenesis and identify a candidate target for therapeutic development.

Successful treatment of anisometropic amblyopia with spectacles alone.

BACKGROUND: The efficacy of treating anisometropic amblyopia with occlusion therapy is well known. However, this form of treatment can be associated with risks. Spectacle correction alone may be a successful and underutilized form of treatment. METHODS: The records of 28 patients treated successfully for anisometropic amblyopia with glasses alone were reviewed. Age, initial visual acuity and stereoacuity, and nature of anisometropia were analyzed to assess associations with time required for resolution, final visual acuity, and stereoacuity. Incidence of amblyopia recurrence and results of subsequent treatment, including patching, were also studied. RESULTS: Mean time to amblyopia resolution (interocular acuity difference