Evaluation of 3D tablet-based stereoacuity test ASTEROID in children with normal and abnormal visual acuity.

PURPOSE: To assess the utility of 3D, tablet-based, glasses-free Accurate STEReotest (ASTEROID) in children compared with the Titmus test. METHODS: Children aged 5-13 years were enrolled in a single-center, nonrandomized, observational comparison study and analyzed by age (5-7 vs 8-13 years) and visual acuity (20/25 or better in both eyes vs abnormal). Each participant underwent both the ASTEROID and Titmus stereoacuity tests. Stereoacuity was defined as fine (≤60 arcsec), moderate (61-200 arcsec), coarse (201-1199 arcsec), or very coarse to nil (≥1200 arcsec). Agreement between the tests was assessed using a weighted kappa (κ) statistic based on all four categories. RESULTS: A total of 112 children were included: 28 aged 5-7 with normal visual acuity, 30 aged 5-7 with abnormal visual acuity, 34 aged 8-13 with normal visual acuity, and 20 aged 8-13 with abnormal visual acuity. Mean ASTEROID score was 688 ± 533 arcsec (range, 13-1200 arcsec). Agreement between ASTEROID and Titmus test scores for participants overall was moderate (κ = 0.52). By subgroup, agreement was fair for children 5-7 with abnormal visual acuity (κ = 0.31), moderate for children 5-7 with normal visual acuity (κ = 0.47) and children 8-13 with normal visual acuity (κ = 0.42), and substantial for children 8-13 with abnormal visual acuity (κ = 0.76). Where ASTEROID and Titmus score group varied, ASTEROID score was poorer in 94% (47/50) of cases. CONCLUSIONS: ASTEROID is a digital, tablet-based test that evaluates global stereopsis, does not require glasses, and provides a continuum of scores. Among children, ASTEROID has good agreement with the Titmus test; however, it may be more sensitive at detecting stereovision deficits. Further study is necessary to determine which test is more accurate.

Inferior Rectus Muscle Y-Split for Thyroid-Related Vertical Strabismus.

Incomitant hypotropia in thyroid eye disease can be difficult to manage, especially in the presence of orthotropia with fusion in down gaze and reading position. Recessing the affected ipsilateral inferior rectus muscle may result in an undesirable downgaze diplopia secondary to a hypertropia in downgaze. Various surgical techniques have been described to manage this potential complication including asymmetric recession of both inferior rectus muscles, posterior myoscleropexy operation, and the Scott recess/resect procedure of the contralateral inferior rectus. In 2004, Hoerantner et al. introduced the y-split recession of the medial rectus muscle for near esotropic deviations. The anterior portion of muscle is split and secured in a y-shaped configuration, which reduces the muscle lever arm and helps minimize incomitance and muscle slippage. Unlike the traditional Cüppers Faden, a y-split recession results in torque reduction in all gaze positions. In addition, a y-split recession does not involve scleral passes posteriorly reducing the risk of globe perforation. We report a patient with incomitant strabismus secondary to thyroid eye disease who underwent a combination of traditional recession and y-splitting recession of the contralateral inferior rectus muscle, resulting in good functional alignment in primary gaze and in the reading position.

Artificial Intelligence to Differentiate Pediatric Pseudopapilledema and True Papilledema on Fundus Photographs.

Purpose: To develop and test an artificial intelligence (AI) model to aid in differentiating pediatric pseudopapilledema from true papilledema on fundus photographs. Design: Multicenter retrospective study. Subjects: A total of 851 fundus photographs from 235 children (age < 18 years) with pseudopapilledema and true papilledema. Methods: Four pediatric neuro-ophthalmologists at 4 different institutions contributed fundus photographs of children with confirmed diagnoses of papilledema or pseudopapilledema. An AI model to classify fundus photographs as papilledema or pseudopapilledema was developed using a DenseNet backbone and a tribranch convolutional neural network. We performed 10-fold cross-validation and separately analyzed an external test set. The AI model's performance was compared with 2 masked human expert pediatric neuro-ophthalmologists, who performed the same classification task. Main Outcome Measures: Accuracy, sensitivity, and specificity of the AI model compared with human experts. Results: The area under receiver operating curve of the AI model was 0.77 for the cross-validation set and 0.81 for the external test set. The accuracy of the AI model was 70.0% for the cross-validation set and 73.9% for the external test set. The sensitivity of the AI model was 73.4% for the cross-validation set and 90.4% for the external test set. The AI model's accuracy was significantly higher than human experts on the cross validation set (P < 0.002), and the model's sensitivity was significantly higher on the external test set (P = 0.0002). The specificity of the AI model and human experts was similar (56.4%-67.3%). Moreover, the AI model was significantly more sensitive at detecting mild papilledema than human experts, whereas AI and humans performed similarly on photographs of moderate-to-severe papilledema. On review of the external test set, only 1 child (with nearly resolved pseudotumor cerebri) had both eyes with papilledema incorrectly classified as pseudopapilledema. Conclusions: When classifying fundus photographs of pediatric papilledema and pseudopapilledema, our AI model achieved > 90% sensitivity at detecting papilledema, superior to human experts. Due to the high sensitivity and low false negative rate, AI may be useful to triage children with suspected papilledema requiring work-up to evaluate for serious underlying neurologic conditions. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Pediatric Optic Pathway Gliomas Resource Utilization and Prevalence in the OptumLabs Data Warehouse.

BACKGROUND: Although significant progress has been made in improving the rate of survival for pediatric optic pathway gliomas (OPGs), data describing the methods of diagnosis and treatment for OPGs are limited in the modern era. This retrospective study aims to provide an epidemiological overview in the pediatric population and an update on eye care resource utilization in OPG patients using big data analysis. METHODS: Using the OptumLabs Data Warehouse, 9-11 million children from 2016 to 2021 assessed the presence of an OPG claim. This data set was analyzed for demographic distribution data and clinical data including average ages for computed tomography (CT), MRI, strabismus, and related treatment (surgery, chemotherapy, and radiation), as well as yearly rates for optical coherence tomography (OCT) and visual field (VF) examinations. RESULTS: Five hundred fifty-one unique patients ranging in age from 0 to 17 years had an OPG claim, with an estimated prevalence of 4.6-6.1 per 100k. Among the 476 OPG patients with at least 6 months of follow-up, 88.9% had at least one MRI and 15.3% had at least one CT. Annual rates for OCT and VF testing were similar (1.26 vs 1.35 per year), although OCT was ordered for younger patients (mean age = 9.2 vs 11.7 years, respectively). During the study period, 14.1% of OPG patients had chemotherapy, 6.1% had either surgery or radiation, and 81.7% had no treatment. CONCLUSIONS: This study updates OPG demographics for the modern era and characterizes the burden of the treatment course for pediatric OPG patients using big data analysis of a commercial claims database. OPGs had a prevalence of about 0.005% occurring equally in boys and girls. Most did not receive treatment, and the average child had at least one claim for OCT or VF per year for clinical monitoring. This study is limited to only commercially insured children, who represent approximately half of the general child population.

Genetic testing for infantile nystagmus syndrome with or without associated findings.

PURPOSE: To review the published literature assessing the clinical utility of genetic testing in individuals with infantile nystagmus syndrome (INS), defined as binocular conjugate nystagmus and onset prior to 6 months of age, with or without associated findings. METHODS: A literature search was last conducted in October 2022. The results were limited to articles published in English. The search yielded 517 abstracts, of which 72 papers were reviewed in full text. Of these papers, 4 met the criteria for inclusion and were graded by a study methodologist. RESULTS: The 4 studies that met inclusion criteria used next-generation sequencing with gene panels ranging from 31 to 336 genes. The overall molecular diagnostic rate ranged from 35% to 60% in the included studies, although the yield was higher when genetic testing was guided by clinical phenotyping (approximately 80%) and in the subsets of patients with a family history (up to 88%). As many as 30% of patients tested had a reclassification of the diagnosis based on the genetic testing results. CONCLUSIONS: Genetic testing has the potential to provide a definitive diagnosis and identify treatable conditions in patients presenting with INS, especially when considered in conjunction with clinical phenotyping and family history.

Levodopa/Carbidopa to Augment the Treatment of Amblyopia: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature on the use of levodopa/carbidopa to augment the treatment of amblyopia. METHODS: Literature searches for English language studies were last conducted in October 2022 in the PubMed database with no date restrictions. The combined searches yielded 55 articles, of which 23 were reviewed in full text. Twelve of these were considered appropriate for inclusion in this assessment and were assigned a level of evidence rating by the panel methodologist. Nine studies were rated level I, and 3 studies were rated level II; there were no level III studies. RESULTS: The duration of treatment was limited to 3 to 16 weeks because of concern about long-term adverse effects such as tardive dyskinesia. This complication was not reported in any of the study participants. The dose of levodopa ranged from 1.5 to 8.3 mg/kg/day, generally divided into 3 daily doses. The carbidopa dose was approximately 25% of the levodopa dose in all treatments. Evidence from these studies indicates that augmenting traditional patch occlusion therapy with the oral administration of levodopa/carbidopa can improve the vision of amblyopic children, but the effect was small (0.17-0.3 logarithm of the minimum angle of resolution [logMAR] units) and only statistically significant when compared with patching alone in 2 of the 12 studies cited. Regression of vision was reported in the majority of studies (9 of 12 reported; range, 0-0.17 logMAR unit regression) after discontinuation of therapy. Short-term side effects of the medications were not consistently reported but were most frequently mild and included headache and nausea. CONCLUSIONS: The best available evidence is currently insufficient to show that augmenting amblyopia therapy using up to 16 weeks of levodopa/carbidopa will result in meaningful improvement in visual acuity. Given the potential for significant side effects such as tardive dyskinesia with long-term therapy, levodopa/carbidopa does not appear to be a viable option for amblyopia therapy FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

An unusual presentation of bilateral optic pathway glioma in Crouzon Syndrome.

Crouzon Syndrome is a genetic craniosynostosis disorder associated with a high risk of ophthalmologic sequelae secondary to structural causes. However, ophthalmologic disorders due to intrinsic nerve aberrations in Crouzon Syndrome have not been described. Optic pathway gliomas (OPGs) are low grade gliomas that are intrinsic to the visual pathway, frequently associated with Neurofibromatosis type 1 (NF-1). OPGs involving both optic nerves without affecting the optic chiasm are rarely seen outside of NF-1. We report an unusual case of bilateral optic nerve glioma without chiasmatic involvement in a 17-month-old male patient with Crouzon Syndrome without any clinical or genetic findings of NF-1. This case suggests that close ophthalmologic follow up and orbital MRIs may benefit patients with Crouzon Syndrome.

Strabismus and Pediatric Psychiatric Illness: A Literature Review.

PURPOSE: This literature review aims to investigate the potential association between strabismus and mental illness among children. MATERIALS: The search was conducted in the PubMed and Google Scholar databases using a wide range of search terms related to strabismus, mental disorders, psychiatric illness, childhood, and adolescence. RESULTS: Eleven published studies were included in this review. The findings from this review suggest an association between strabismus and mental illness. Negative attitudes and social bias against children with strabismus were also noted. CONCLUSIONS: These findings should alert healthcare providers to counsel children and their caregivers regarding the risk for mood disorders in children with strabismus and to consider mental health screening and referral as needed.

A Potentially Adjustable Modification of the Nishida Procedure.

For patients with a complete, chronic abducens nerve palsy and resulting abduction deficit, a transposition procedure is often the procedure of choice. One such transposition procedure involves transposing the superior rectus (SR) and inferior rectus (IR) laterally without disinserting or splitting either muscle. While effective, this procedure - like many transposition procedures - carries with it the risk of induced torsional or vertical misalignment. Here, we describe an adjustable variation of the above transposition procedure, one which potentially would allow for post-operative correction of induced vertical or torsional deviations.

Surgical Treatments to Improve Visual Acuity in Infantile Nystagmus Syndrome: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the literature on the efficacy of surgical procedures to improve visual acuity (VA) in patients with infantile nystagmus syndrome (INS). METHODS: Literature searches were last conducted in January 2022 in the PubMed database for English-language studies with no date restrictions. The combined searches yielded 354 abstracts, of which 46 were reviewed in full text. Twenty-three of these were considered appropriate for inclusion in this assessment and were assigned a level of evidence rating by the panel methodologist. RESULTS: One included study was a randomized trial; the remaining 22 were case series. The 23 studies included children and adults with INS and a variable proportion with anomalous head position (AHP), strabismus, and sensory diagnoses. The surgical interventions evaluated included large recessions, tenotomy and reattachment (TAR), myectomy with or without pulley fixation, and anterior extirpation of the 4 horizontal rectus muscles, as well as various procedures to correct an AHP in which VA was reported as a secondary outcome. The data were mixed, with improvements in binocular best-corrected visual acuity (BCVA) ranging from no improvement to 0.3 logarithm of the minimum angle of resolution (logMAR), or 3 lines. (Most studies were in the range of 0.05-0.2 logMAR.) Statistically significant improvement in VA was noted in 12 of 16 studies (75%) that performed statistical analyses, with no clear advantage of any single procedure. Complications and reoperations were lowest in patients who underwent TAR and highest in those who underwent myectomy or anterior extirpation. CONCLUSIONS: The best available evidence suggests that eye muscle surgery in patients with INS results in a modest improvement in VA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Association of Pediatric Strabismus and Functional Impairment: A Cross-sectional Nationwide Analysis.

PURPOSE: To examine the association of childhood strabismus with functional limitation to identify particular domains of impairment. METHODS: The authors analyzed 201 children ages 5 to 17 years with strabismus enrolled in the 1996-2015 Medical Expenditure Panel Surveys, representative annual surveys of the U.S. POPULATION: Functional impairment was assessed using the Columbia Impairment Scale (CIS), a validated measure of behavioral and psychosocial functioning. A CIS score of 16 or greater defined clinically significant functional impairment. Multivariate regression models adjusted for age, sex, race, ethnicity, household income, geographic location, and insurance type were constructed to examine the association of strabismus diagnosis with overall impairment and individual domains of function. RESULTS: Children diagnosed as having strabismus had higher rates of clinically significant functional impairment compared to those without strabismus (15.1% vs 9.1%, adjusted odds ratio [95% CI]: 1.82 [1.11 to 2.97], P = .02). Moreover, strabismus diagnosis was associated with higher rates of problems with getting along with their mother (1.70 [1.21 to 2.40], P = .003) and father (1.66 [1.16 to 2.38], P = .006), getting along with other children (1.67 [1.16 to 2.40], P = .006), behavior at home (1.94 [1.37 to 2.74], P = .0002), staying out of trouble (1.52 [1.04 to 2.23], P = .03), nervousness (1.49 [1.05 to 2.11], P = .02), and getting involved with sports and hobbies (1.55 [1.03 to 2.34], P = .04). CONCLUSIONS: Childhood strabismus is associated with 1.8-fold greater odds of clinically significant functional impairment, with greater dysfunction in specific relationship and behavioral domains. Functional burden may be an important consideration in management decisions. [J Pediatr Ophthalmol Strabismus. 2023;60(4):268-276.].

Acquired Diplopia in Adults: Heavy Eye Syndrome.

Heavy eye syndrome is a condition that results in an acquired, progressive strabismus that is due to extreme myopia and long axial length. The underlying pathogenesis of the condition is due to prolapse of the supero-temporal aspect of the myopic globe causing displacement of the lateral and superior rectus muscles inferiorly and nasally, respectively. Treatments for heavy eye syndrome often target this anatomical defect and seek to re-place the globe within the extraocular muscle cone. This review will discuss current theories of pathogenesis and treatment of heavy eye syndrome.

Agreement of iCare IC200 tonometry with Perkins applanation tonometry in healthy children.

PURPOSE: To assess interdevice agreement between the iCare IC200 rebound tonometer and Perkins applanation tonometry (gold standard) in a healthy pediatric population. METHODS: A total of 42 eyes of 42 healthy children were assessed using both tonometers. Data was collected on subject's age, sex, best-corrected visual acuity, and central corneal thickness (CCT). Intraclass correlation coefficient (ICC) and Bland-Altman analyses were used to determine agreement between IC200 and Perkins applanation tonometers. Linear regression analyzed the effects of intraocular pressure (IOP) on device difference. RESULTS: The mean age and standard deviation of healthy pediatric subjects was 10.0 ± 3.3 years. The mean difference between IC200 and Perkins tonometers (IC200-Perkins) was 0.72 mm Hg, with a mean of 17.1 ± 3.0 mm Hg and 16.4 ± 2.5 mm Hg, respectively. The absolute agreement, or ICC, between tonometers was 0.63 (95% CI, 0.56-0.70). Bland-Altman analysis showed 95% limits of agreement ranging from -5.2 to +6.6 mm Hg. CCT was not correlated with IOP for either the IC200 (P = 0.35) or the Perkins tonometer (P = 0.052). CONCLUSIONS: Compared to applanation tonometry, IC200 overestimated IOP in healthy children, with a greater frequency of readings > +2 mm Hg than < -2 mm Hg compared to Perkins. There was moderate agreement between tonometers. CCT was not found to influence IOP measurement for either tonometer.

Effectiveness of Laser Refractive Surgery to Address Anisometropic Amblyogenic Refractive Error in Children: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature assessing the safety and effectiveness of laser refractive surgery to treat anisometropic amblyogenic refractive error in children aged ≤ 18 years. METHODS: A literature search of the PubMed database was conducted in October 2021 with no date limitations and restricted to publications in English. The search yielded 137 articles, 69 of which were reviewed in full text. Eleven articles met the criteria for inclusion and were assigned a level of evidence rating. RESULTS: The 11 included articles were all level III evidence and consisted of 1 case-control study and 10 case series. Six studies used laser-assisted in situ keratomileusis (LASIK), 1 used photorefractive keratectomy (PRK), 1 used refractive lenticule extraction/small incision lenticule extraction, and the rest used a combination of LASIK, PRK, laser epithelial keratomileusis (LASEK), or refractive lenticule extraction/small incision lenticule extraction. Five studies enrolled patients with anisometropic myopia, 2 studies enrolled patients with anisometropic hyperopia, and the remainder were mixed. Although all studies demonstrated an improvement in best-corrected visual acuity (BCVA), the magnitude of improvement varied widely. As study parameters varied, a successful outcome was defined as residual refractive error of 1 diopter (D) or less of the target refraction because this was the most commonly used metric. Successful outcomes ranged between 38% and 87%, with a mean follow-up ranging from 4 months to 7 years. Despite this wide range, all studies demonstrated an improvement in the magnitude of anisometropia. Regression in refractive error occurred more frequently and to a greater degree in myopic eyes and eyes with longer follow-up, and in younger patients. Although one study reported 2 free flaps, most studies reported no serious adverse events. The most common complications were corneal haze and striae. CONCLUSIONS: Findings from included studies suggest that laser refractive surgery may address amblyogenic refractive error in children and that it appears to decrease anisometropia. However, the evidence for improvement in amblyopia is unclear and long-term safety data are lacking. Long-term data and well-designed clinical studies that use newer refractive technologies in standardized patient populations would help address the role of refractive surgery in children and its potential impact on amblyopia.

The Pediatric Optic Neuritis Prospective Outcomes Study: Two-Year Results.

PURPOSE: Pediatric optic neuritis (ON) is a rare disease that has not been well characterized. The Pediatric ON Prospective Outcomes Study (PON1) was the first prospective study to our knowledge aiming to evaluate visual acuity (VA) outcomes, including VA, recurrence risk, and final diagnosis 2 years after enrollment. DESIGN: Nonrandomized observational study at 23 pediatric ophthalmology or neuro-ophthalmology clinics in the United States and Canada. PARTICIPANTS: A total of 28 (64%) of 44 children initially enrolled in PON1 (age 3-<16 years) who completed their 2-year study visit. METHODS: Participants were treated at the investigator's discretion. MAIN OUTCOMES MEASURES: Age-normal monocular high-contrast VA (HCVA). Secondary outcomes included low-contrast VA (LCVA), neuroimaging findings, and final diagnoses. RESULTS: A total of 28 participants completed the 2-year outcome with a median enrollment age of 10.3 years (range, 5-15); 46% were female, and 68% had unilateral ON at presentation. Final 2-year diagnoses included isolated ON (n = 11, 39%), myelin oligodendrocyte glycoprotein-associated demyelination (n = 8, 29%), multiple sclerosis (MS) (n = 4,14%), neuromyelitis optica spectrum disease (NMOSD) (n = 3, 11%), and acute disseminated encephalomyelitis (n = 2, 7%). Two participants (7%; 95% confidence interval [CI], 1-24) had subsequent recurrent ON (plus 1 participant who did not complete the 2-year visit); all had MS. Two other participants (7%) had a new episode in their unaffected eye. Mean presenting HCVA was 0.81 logarithm of the minimum angle of resolution (logMAR) (∼20/125), improving to 0.14 logMAR (∼20/25-2) at 6 months, 0.12 logMAR (∼20/25-2) at 1 year, and 0.11 logMAR (20/25-1) at 2 years (95% CI, -0.08 to 0.3 [20/20+1-20/40-1]). Twenty-four participants (79%) had age-normal VA at 2 years (95% CI, 60-90); 21 participants (66%) had 20/20 vision or better. The 6 participants without age-normal VA had 2-year diagnoses of NMOSD (n = 2 participants, 3 eyes), MS (n = 2 participants, 2 eyes), and isolated ON (n = 2 participants, 3 eyes). Mean presenting LCVA was 1.45 logMAR (∼20/500-2), improving to 0.78 logMAR (∼20/125+2) at 6 months, 0.69 logMAR (∼20/100+1) at 1 year, and 0.68 logMAR (∼20/100+2) at 2 years (95% CI, 0.48-0.88 [20/50+1-20/150-1]). CONCLUSIONS: Despite poor VA at presentation, most children had marked improvement in VA by 6 months that was maintained over 2 years. Associated neurologic autoimmune diagnoses were common. Additional episodes of ON occurred in 5 (18%) of the participants (3 relapses and 2 new episodes).

Association of Strabismus With Mood Disorders, Schizophrenia, and Anxiety Disorders Among Children.

Importance: Children with strabismus have poorer functional vision and decreased quality of life than those without strabismus. Objective: To evaluate the association between strabismus and mental illness among children. Design, Setting, and Participants: This cross-sectional study analyzed claims data from the OptumLabs Data Warehouse, a longitudinal deidentified commercial insurance claims database, from 12 005 189 patients enrolled in the health plan between January 1, 2007, and December 31, 2017. Eligibility criteria included age younger than 19 years at the time of strabismus diagnosis, enrollment in the health plan between 2007 and 2018, and having at least 1 strabismus claim based on International Classification of Diseases, Ninth Revision, Clinical Modification and International Statistical Classification of Diseases and Related Health Problems, Tenth Revision, Clinical Modification codes. Controls were children in the same database with no eye disease codes other than refractive error reported. Demographic characteristics and mental illness claims were compared. Statistical analysis was conducted from December 1, 2018, to July 31, 2021. Main Outcomes and Measures: Presence of mental illness claims. Results: Among the 12 005 189 patients (6 095 523 boys [50.8%]; mean [SD] age, 8.0 [5.9] years) in the study, adjusted odds ratios for the association of mental illnesses with strabismus were 2.01 (95% CI, 1.99-2.04) for anxiety disorder, 1.83 (95% CI, 1.76-1.90) for schizophrenia, 1.64 (95% CI, 1.59-1.70) for bipolar disorder, 1.61 (95% CI, 1.59-1.63) for depressive disorder, and 0.99 (95% CI, 0.97-1.02) for substance use disorder. There was a moderate association between each strabismus type (esotropia, exotropia, and hypertropia) and anxiety disorder, schizophrenia, bipolar disorder, and depressive disorder; odds ratios ranged from 1.23 (95% CI, 1.17-1.29) for the association between esotropia and bipolar disorder to 2.70 (95% CI, 2.66-2.74) for the association between exotropia and anxiety disorder. Conclusions and Relevance: This cross-sectional study suggests that there was a moderate association between strabismus and anxiety disorder, schizophrenia, bipolar disorder, and depressive disorder but not substance use disorder. Recognizing that these associations exist should encourage mental illness screening and treatment for patients with strabismus.

Association of Mood Disorders, Substance Abuse, and Anxiety Disorders in Children and Teens With Serious Structural Eye Diseases.

PURPOSE: We sought to evaluate the association between 5 eye diseases (including glaucoma, cataract, congenital optic nerve disease, congenital retinal disease, and blindness/low vision) and mental illness in a pediatric population. DESIGN: Cross-sectional study. METHODS: A de-identified commercial insurance claims database, OptumLabs Data Warehouse, between January 1, 2007, and December 31, 2018, was used. Children and teens less than 19 years of age at the time of eye diagnosis were included. Demographics and mental illness claims were compared, looking at the association of mental illness and eye disease claims. RESULTS: A total of 11,832,850 children and teens were included in this study with mean age of 8.04 ± 5.94 years at the first claim. Of the patients with at least 1 of the 5 eye diseases (n = 180,297), 30.5% had glaucoma (n = 54,954), 9.5% had cataract (n = 17,214), 21.4% had congenital optic nerve disease (n = 38,555), 26.9% had congenital retinal disease (n = 48,562), and 25.9% had blindness or low vision (n = 46,778). There was a statistically significant association, after adjusting for confounding variables, between at least 1 of the 5 eye diseases and schizophrenia disorder (OR = 1.54, 95% CI = 1.48-1.61, P < .001), anxiety disorder (OR = 1.45, 95% CI = 1.43-1.48, P < .001), depressive disorder (OR = 1.27, 95% CI = 1.25-1.29, P < .001), and bipolar disorder (OR = 1.27, 95% CI = 1.21-1.31, P < .001), but a reversed association with substance use disorder (OR = 0.88, 95% CI = 0.86-0.90, P < .001). CONCLUSIONS: We found associations between eye disease in children and teens and mental illness. Understanding these relationships may improve mental illness screening and treatment in the pediatric population.

Eyelid nystagmus in a child with cardiofaciocutaneous syndrome associated with BRAF mutation.

Eyelid nystagmus, thought to reflect midbrain or hindbrain disease, is a rare condition that typically occurs in association with neurologic and neuroophthalmic abnormalities. Cardiofaciocutaneous (CFC) syndrome associated with B-raf protooncogene serine/threonine kinase (BRAF) mutation is a complex syndrome that is associated with neurologic and neuroophthalmologic abnormalities. We report the case of a 4-year-old girl with CFC syndrome with global developmental delay who was referred for evaluation of alternating exotropia, right-sided dissociated vertical deviation, bilateral upgaze nystagmus, and bilateral eyelid fluttering. Neurological work-up revealed bilateral frontocentral sharp waves without seizure activity on electroencephalogram and mild left-sided cerebral volume loss on magnetic resonance imaging. To our knowledge, this is the first reported case of eyelid nystagmus in CFC syndrome. Eyelid nystagmus warrants neurological evaluation, because it is commonly associated with significant neurological abnormalities.