Otitic hydrocephalus and papilloedema-re-evaluating a treatment paradigm.

Background: Otitic hydrocephalus is increased intracranial pressure without ventricular dilation secondary to mastoiditis and cerebral venous sinus thrombosis (CVST). It is associated with significant visual morbidity, though more detailed data on visual outcomes is lacking. We sought to better characterize the management of increased intracranial pressure and visual outcomes in this population.

Efficacy and Side Effects of Topiramate in Treatment of Children With Pseudotumor Cerebri Syndrome.

Background: Topiramate is often considered as a second-line medication for the treatment of pseudotumor cerebri syndrome (PTCS), but limited studies exist that evaluate its efficacy in children.

Methods: Retrospective study of patients aged <21 years with PTCS who were treated with topiramate alone or in combination with acetazolamide was performed. Data regarding clinical courses and visual outcomes were recorded.

Prevalence, time course, and visual impact of peripapillary hyperreflective ovoid mass-like structures (PHOMS) in pediatric patients with optic nerve pathologies.

Background: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a recently defined optical coherence tomography (OCT) finding. The purpose of this study was to characterize the presence of PHOMS and their visual significance in pediatric patients with and without optic nerve pathologies.

Methods: This retrospective study evaluated 400 patients (<18 years of age) including normal control subjects and patients with optic neuritis, papillitis, optic nerve head drusen (ONHD), and papilledema.

Long-term visual outcomes in spasmus nutans.

Background: The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid ophthalmic conditions in patients with spasmus nutans.

Methods: We retrospectively reviewed the charts of consecutive patients diagnosed with spasmus nutans between 2000 and 2020.

Aberrant regeneration of accommodation in pediatric inferior division oculomotor palsy.

Aberrant regeneration occurs in forms of oculomotor motor nerve palsy and frequently involves the pupil, but the incidence and functional impact of ciliary muscle involvement in pediatric patients is sparsely reported in the literature. A 4-year-old girl presented with inflammatory oculomotor motor nerve paresis affecting the inferior division. Initial treatment focused on her inability to accommodate through her physiologic +2.

The Relationship Between Choroidal Abnormalities and Visual Outcomes in Pediatric Patients With NF1-Associated Optic Pathway Gliomas.

Background: Choroidal abnormalities (CAs) visualized on near-infrared reflectance (NIR) imaging are a new diagnostic criterion for neurofibromatosis type 1 (NF1), but the association between the presence of CAs and visual function remains unknown. This study evaluated the relationship between visual acuity (VA) with the presence, number, or total area of CAs visualized by NIR in children with NF1-associated optic pathway gliomas (NF1-OPGs).

Methods: Patients (<18 years) enrolled in a prospective longitudinal study of children with NF1-associated OPGs from 3 institutions were eligible if they had optical coherence tomography (OCT) of the macula (Heidelberg Spectralis) with ≥1 year of follow-up.

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.

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.

The impact of the COVID-19 pandemic on the surgical volume of pediatric ophthalmology and strabismus fellows.

This study used data from the annual fellowship survey over 7 academic years (2014-15 to 2020-21) to describe the trends in surgical experience for pediatric ophthalmology and strabismus fellows and to quantify the impact of the COVID-19 pandemic on trainee surgical volume. The overall number of procedures performed by fellows in the primary surgeon role declined during the first academic year impacted by the pandemic but recovered in the second year. There was an increase in the number of intraocular cases performed per year during the 7-year study interval.

Aberrant regeneration in an international registry of patients with 3rd-nerve palsy.

Background/aims: To describe the patterns of pre-operative aberrant regeneration and motility outcomes reported in an international registry of patients with 3-nerve palsy treated with nasal transposition of the split lateral rectus muscle (NTSLR).

Methods: This cross-sectional study used data from an international, multicentre registry of patients with 3-nerve palsy treated with NTSLR. Patients with aberrant regeneration were identified, and patterns of innervation described.

Neuro-ophthalmic Complications in Pediatric Leukemia.

Background: Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood dyscrasias, or adverse effects of treatment. We aimed to characterize visual outcomes in pediatric patients with leukemia-associated neuro-ophthalmic manifestations.

Methods: We retrospectively identified patients with leukemia and optic nerve pathology over 13 years by diagnostic billing codes.

The visual morbidity of optic nerve head drusen: a longitudinal review.

Background: The ophthalmologic complications of optic nerve head drusen (ONHD) in adults have been documented, whereas data on the degree of visual morbidity from OHND in children are limited.

Methods: The medical records of all patients diagnosed with ONHD at a single, tertiary care ophthalmology department from January 1, 2010, until July 1, 2018, were reviewed retrospectively. Patients were identified using ICD-9 and ICD-10 codes.

The Impact of Strabismus on Psychosocial Equity.

Strabismus, the condition of misaligned eyes, can result in severe, long-lasting functional and psychosocial sequelae. This review examines existing literature that has described and quantified the psychosocial consequences of strabismus. In particular, the role of strabismus in creating social, psychological, and vocational disparities, and how these intersect with race, ethnicity, and gender, is described.

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.

A decline in the strabismus surgical experience of ophthalmology residents in the United States from 2010 to 2019.

Subspecialty exposure during residency can influence the future pursuit of fellowship training. In this study, we compared the trends in strabismus surgical experience reported by graduating ophthalmology residents in the United States with other categories of ophthalmic surgery. Over the 10-year period (2010-2019), there was a decline in the total number of strabismus procedures performed during residency by ophthalmology residents graduating in a given year (1.

An overview of peripapillary hyperreflective ovoid mass-like structures.

Purpose Of Review: The purpose of this review is to provide an overview of the ophthalmic findings associated with peripapillary hyperreflective ovoid mass-like structures (PHOMS) in both adult and pediatric patients.

Recent Findings: PHOMS have recently been identified in a number of different ophthalmic disease entities ranging from nonpathologic to pathologic, including but not limited to anatomic abnormalities (tilting in myopia), optic nerve head drusen, optic disc edema from inflammation (optic neuritis, white dot syndromes), vascular insults (ischemic optic neuropathy, retinal vascular occlusion), and papilledema. The mechanism underlying the formation of PHOMS has not been fully elucidated although it has been hypothesized that PHOMS occur secondary to axoplasmic stasis from crowding at the optic nerve head.

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.

Comparison of fellowship match opportunities and results across pediatric surgical subspecialities.

There is a nationwide shortage of pediatric ophthalmologists, with trends in fellowship applicants foreshadowing a continued reduction in the number of active pediatric ophthalmologists in the years ahead. In this study, we investigated whether similar fellowship applicant shortages exist in other pediatric surgical subspecialties. We compared the match statistics of six pediatric surgical fellowships from 2015 to 2020.

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.

Impact of Adding Augmented Superior Rectus Transpositions to Medial Rectus Muscle Recessions When Treating Esotropic Moebius Syndrome.

Purpose: To describe outcomes after treatment of Moebius syndrome (MBS) esotropia by adjustable bilateral medial rectus recession (BMR) with and without augmented superior rectus transposition (SRT).

Design: Retrospective case series.

Methods: Patients meeting 2014 diagnostic criteria for MBS and treated at Boston Children's Hospital between 2003 and 2019 were identified via billing records and chart review.

Evaluation of the Relationship Between Preferential Looking Testing and Visual Evoked Potentials as a Biomarker of Cerebral Visual Impairment.

Cerebral visual impairment (CVI) is a leading cause of visual impairment in children in developed countries, but diagnostic tools to detect CVI are limited. We sought to analyze the visual acuity of children with CVI as assessed by visual evoked potentials (VEPs) and preferential looking test (PLT) to determine whether the relationship between the visual outcomes on these two testing methods may serve as a biomarker of CVI. We performed a retrospective chart review of patients with a confirmed diagnosis of CVI and at least one ophthalmological assessment with visual acuity measured by VEP and PLT.

Adjustable Sutures in the Treatment of Strabismus: A Report by the American Academy of Ophthalmology.

Purpose: To review the scientific literature that evaluates the effectiveness of adjustable sutures in the management of strabismus for adult and pediatric patients.

Methods: Literature searches were performed in the PubMed database through April 2021 with no date limitations and were restricted to publications in English. The searches identified 551 relevant citations, of which 55 were reviewed in full text.

Content Validation of Clinician-Reported Items for a Severity Measure for CDKL5 Deficiency Disorder.

CDKL5 deficiency disorder (CDD) results in early-onset seizures and severe developmental impairments. A CDD clinical severity assessment (CCSA) was previously developed with clinician and parent-report items to capture information on a range of domains. Consistent with US Food and Drug Administration (FDA) guidelines, content validation is the first step in evaluating the psychometric properties of an outcome measure.