Optic Pathway Gliomas in Neurofibromatosis Type 1: An Update: Surveillance, Treatment Indications, and Biomarkers of Vision

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Title Optic Pathway Gliomas in Neurofibromatosis Type 1: An Update: Surveillance, Treatment Indications, and Biomarkers of Vision
Creator Peter M. K. de Blank, MD, MSCE, Michael J. Fisher, MD, Grant T. Liu, MD, David H. Gutmann, MD, PhD, Robert Listernick, MD, Rosalie E. Ferner, MD, Robert A. Avery, DO, MSCE
Affiliation Cancer and Blood Diseases Institute (PMKdB), Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics (PMKdB), University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Oncology (MJF), Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics (MJF), Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Neuro-Ophthalmology Service (GTL, RAA), Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Departments of Neurology and Ophthalmology (GTL, RAA), Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Neurofibromatosis Center and Department of Neurology (DHG), Washington University, St. Louis, Missouri; Department of Pediatrics (RL), Ann & Robert H. Lurie Children's Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; Neurofibromatosis Centre, Department of Neurology (REF), Guy's and St. Thomas' NHS Foundation Trust; and Department of Clinical Neuroscience (REF), King's College, London, United Kingdom
Abstract Optic pathway gliomas (OPGs) occur in 15%-20% of children with neurofibromatosis type 1 (NF1), leading to visual deficits in fewer than half of these individuals. The goal of chemotherapy is to preserve vision, but vision loss in NF1-associated OPG can be unpredictable. Determining which child would benefit from chemotherapy and, equally important, which child is better observed without treatment can be difficult. Unfortunately, despite frequent imaging and ophthalmologic evaluations, some children experience progressive vision loss before treatment. Indications for chemotherapy usually are based on a comprehensive, quantitative assessment of vision, but reliable vision evaluation can be challenging in young children with NF1-OPG. The ability to identify and predict impending vision loss could potentially improve management decisions and visual outcomes. To address this challenge, ophthalmologic, electrophysiologic, and imaging biomarkers of vision in NF1-OPG have been proposed. We review current recommendations for the surveillance of children at risk for NF1-OPG, outline guidelines for initiating therapy, and describe the utility of proposed biomarkers for vision.
Subject Child; Combined Modality Therapy; Humans; Magnetic Resonance Imaging; Neurofibromatosis 1; Optic Nerve Glioma; Optic Nerve Neoplasms; Visual Acuity
OCR Text Show
Date 2017-09
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, September 2017, Volume 37, Issue 3
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s67h5ssk
Setname ehsl_novel_jno
ID 1374474
Reference URL https://collections.lib.utah.edu/ark:/87278/s67h5ssk
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