Publications by authors named "Ken Nischal"

Objective: To assess the educational impact of a worldwide webinar approach to myopia progression management in children <8 years and 8-12 years old.

Design: Cross-sectional study.

Methods: A self-administered survey was conducted for attendees of a 3 h worldwide webinar held in two parts on consecutive days on the management of myopia progression in children.

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Purpose: To study the utility of integrated intraoperative OCT (i2OCT) in pediatric patients with cataracts in the real world.

Methods: It was a retrospective case series. We included patients aged 0-12 years with unilateral or bilateral cataracts who underwent cataract surgery or membranectomy for visual axis opacification between July 2022 and December 2023, where intraoperative OCT was used.

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Objective: To assess clinical factors leading to recurrent retinal detachment (RD) and characteristics of recurrence in patients with Stickler Syndrome.

Methods: Retrospective case series study of patients with clinical diagnosis of Stickler Syndrome who underwent rhegmatogenous RD repair. Recurrent RD after initial surgery was categorized as "early" if the recurrence was within 1 year or "late" if greater than 1 year.

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Background: Eye care organizations and professionals worldwide are increasingly focusing on bridging the gap between population health and medical practice. Recent advances in genomics and anthropology have revealed that most Indian groups trace their ancestry to a blend of 2 genetically distinct populations: Ancestral North Indians, who share genetic affinities with Central Asians, Middle Easterners, Caucasians, and Europeans; and Ancestral South Indians, genetically distinct from groups outside the Indian subcontinent. Studies conducted among North Indian populations can therefore offer insights that are potentially applicable to these diverse global populations, underscoring significant implications for global health.

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Article Synopsis
  • Corneal cross-linking (CXL) is effective for treating keratoconus in children and those with developmental delays, requiring tailored surgical techniques and careful decision-making.
  • A study at UPMC Children's Hospital analyzed data from 34 patients, revealing that all underwent standard epithelium-off CXL under general anesthesia, with minimal complications and a noted trend towards improved visual acuity post-surgery.
  • The approach included temporary tarsorrhaphy to manage pain effectively, highlighting a safe and timely diagnostic and treatment strategy for these patient groups.
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Article Synopsis
  • * Myopia management aims to reduce severe forms of myopia and the sight-threatening complications associated with it, alongside tackling the dangers of ultraviolet radiation on eye health.
  • * There is a pressing need for public health policies that not only promote high lux light exposure but also ensure effective UV protection, particularly with advancements in protective eyewear design that balances light exposure and UV defense.
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Vernal keratoconjunctivitis (VKC) is a chronic, progressive, allergic ocular surface disorder that can lead to sight-threatening complications. VKC occurs primarily in children and generally resolves about the time of puberty; however, case series and retrospective analyses indicate that approximately 10% of patients with VKC are adults, and that a subset of adult cases develop after puberty. Consequently, two age-related variants of VKC have recently been described: early-onset VKC-which manifests during childhood and persists into adult life-and late-onset disease, which emerges after puberty.

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Objective: To assess clinical factors leading to recurrent retinal detachment (RD) and characteristics of recurrence in patients with Stickler Syndrome.

Methods: Retrospective case series study of patients with clinical diagnosis of Stickler Syndrome who underwent rhegmatogenous RD repair. Recurrent RD after initial surgery was categorized as "early" if the recurrence was within 1 year or "late" if greater than 1 year.

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Long chain 3-hydroxyacyl-CoA dehydrogenase (LCHADD) is the only fatty acid oxidation disorder to develop a progressive chorioretinopathy resulting in vision loss; newborn screening (NBS) for this disorder began in the United States around 2004. We compared visual outcomes among 40 participants with LCHADD or trifunctional protein deficiency diagnosed symptomatically to those who were diagnosed via NBS or a family history. Participants completed ophthalmologic testing including measures of visual acuity, electroretinograms (ERG), fundal imaging, contrast sensitivity, and visual fields.

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Purpose: The International Committee for the Classification of Corneal Dystrophies (IC3D) was created in 2005 to develop a new classification system integrating current information on phenotype, histopathology, and genetic analysis. This update is the third edition of the IC3D nomenclature.

Methods: Peer-reviewed publications from 2014 to 2023 were evaluated.

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Article Synopsis
  • - The study aimed to analyze the imaging and clinical aspects of rare calcified lesions in the eyes of patients with mosaic RASopathy, involving 10 eyes across 7 patients.
  • - Various imaging techniques, including fundus photography and B-scan ultrasonography, revealed calcified lesions near the optic nerve, with most affecting both the choroid and sclera.
  • - Results showed that these lesions were associated with different RASopathy syndromes, and complications such as the development of a choroidal neovascular membrane were noted, requiring treatment.
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Objective: To develop an updated staging system for long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency (LCHADD) chorioretinopathy based on contemporary multimodal imaging and electrophysiology.

Methods: We evaluated forty cases of patients with genetically confirmed LCHADD or trifunctional protein deficiency (TFPD) enrolled in a prospective natural history study. Wide-field fundus photographs, fundus autofluorescence (FAF), optical coherence tomography (OCT), and full-field electroretinogram (ffERG) were reviewed and graded for severity.

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Purpose: To report the clinical utility of optical coherence tomography angiography (OCTA) for demonstrating choroidal neovascularization (CNV) associated with Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHADD) retinopathy.

Methods: Thirty-three participants with LCHADD (age 7-36 years; median 17) were imaged with OCTA and the Center for Ophthalmic Optics & Lasers Angiography Reading Toolkit (COOL-ART) software was implemented to process OCTA scans.

Results: Seven participants (21 %; age 17-36 years; median 25) with LCHADD retinopathy demonstrated evidence of CNV by retinal examination or presence of CNV within outer retinal tissue on OCTA scans covering 3 × 3 and/or 6 × 6-mm.

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Purpose: The aim of this study is to describe the variable phenotype of congenital corneal opacities occurring in patients with biallelic CYP1B1 pathogenic variants.

Methods: A retrospective chart review was conducted to identify patients with congenital corneal opacities and CYP1B1 pathogenic variants seen at UPMC Children's Hospital of Pittsburgh. Ophthalmic examination, high-frequency ultrasound, anterior segment optical coherence tomography, histopathologic images, and details of genetic testing were reviewed.

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Purpose: To describe our experience with locally developed evidence-based guidelines for oral fluorescein angiography (FA) for retinal imaging in children.

Methods: The medical records of consecutive pediatric patients (≤18 years of age) at University of Pittsburgh Medical Center Children's Hospital Eye Center who underwent oral FA between November 1, 2018, and April 1, 2022, were reviewed retrospectively. Adherence to or deviation from the guidelines was noted at the time of testing.

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Purpose: The purpose of this study was to evaluate the epidemiology, etiology, clinical assessment, investigation, management, and visual consequences of high myopia (≤-6 diopters [D]) in infants and young children.

Findings: High myopia is rare in pre-school children with a prevalence less than 1%. The etiology of myopia in such children is different than in older children, with a high rate of secondary myopia associated with prematurity or genetic causes.

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Objective: To describe the first paediatric case series of Thygesons' superficial punctate keratitis (TSPK) with management outcomes.

Methods: A retrospective chart review was done for all children either diagnosed at initial presentation or referred with TSPK from 01/2012 to 08/2021 at a tertiary children's hospital. Records were assessed for signs, symptoms, diagnosis, steroid and cyclosporine 0.

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Article Synopsis
  • Relentless placoid chorioretinitis (RPC) is a rare eye condition affecting the retinal pigment epithelium, characterized by a prolonged and recurring clinical course, with no standard treatments available.
  • A study reviewed existing literature and analyzed four young patients diagnosed with RPC to compare their treatment responses, noting the atypical characteristics of their lesions.
  • All patients received steroid treatments, with varying results; some achieved remission with steroid-sparing therapies, while others experienced complications or treatment failures, highlighting the complexity of effectively managing RPC.
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Background: Optical coherence tomography angiography (OCTA) is a noninvasive imaging modality used to analyze the retinochoroidal vasculature and detect vascular flow. The resulting images can be segmented to view each vascular plexus individually. While fluorescein angiography is still the gold standard for the diagnosis of posterior uveitis, it has limitations, and can be replaced by OCTA in some cases.

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Digital eye strain (DES) or computer vision syndrome (CVS) is a phenomenon linked to ever increasing digital screen use globally, affecting a large number of individuals. Recognizing causative and alleviating factors of DES may help establish appropriate policies. We aimed to review factors that aggravate or alleviate DES symptoms in young, i.

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Article Synopsis
  • The study focuses on a staged surgical technique for treating large ocular surface lipodermoids, which may affect vision and eyelid closure in children.
  • The procedure involves debulking the conjunctival part of the lipodermoid, followed by excising the corneal section and performing autologous limbal epithelial transplantation to aid healing.
  • Results from three pediatric cases showed significant improvement in visual axis clearance and eyelid function, with minimal complications, suggesting this method as a viable alternative to more invasive options.
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Purpose: The aim of this study was to report a case of corneal plana-like phenotype with bilateral peripheral scleralization associated with a PITX2 pathogenic variant.

Methods: Clinical findings were obtained by ophthalmologic examination. Molecular diagnosis was performed by whole-exome sequencing in the patient and his parents.

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