| Optic pathway gliomas in patients with neurofibromatosis type 1: follow-up of 44 patients. | |
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MedLine Citation:
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PMID: 20451859 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Children born with neurofibromatosis type 1 (NF1) have an increased risk of developing optic pathway gliomas (OPGs) during childhood. The aim of this study is to evaluate the clinical course of NF1 patients with OPGs at our institution with respect to visual and endocrinologic morbidity. METHODS: Retrospective case series of patients with OPGs and NF1 seen at the Montreal Children's Hospital, where screening imaging is performed on all NF1 patients. Details on patient demographics, tumor location, and progression of disease were recorded. RESULTS: Of 331 NF1 patient charts reviewed, 44 had confirmed OPG (13%). Average follow-up was 7 years. Mean age at presentation was 6 years, with 16 patients (36%) presenting past age 6. A total of 8 patients were symptomatic secondary to the OPG (defined as decreased vision or precocious puberty), with 5 of the 8 patients receiving treatment. These 8 patients all demonstrated chiasmal and/or retrochiasmal tumor in addition to nerve involvement. Final visual acuity was 20/40 or better in both eyes in 34 patients (77%); central, steady, and maintained in 3 preverbal children; and decreased vision secondary to OPG in 4 children (9%). CONCLUSIONS: OPGs can present and progress beyond the preschool years, and children should be screened with clinical ophthalmological examinations accordingly. The location of OPG as demonstrated on magnetic resonance imaging (MRI) cannot be used as a prognostic indicator because visual outcomes were similar between optic nerve/chiasmal and retrochiasmal tumors. |
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Authors:
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Laura Segal; Mahshad Darvish-Zargar; Marie-Emmanuelle Dilenge; June Ortenberg; Robert C Polomeno |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus Volume: 14 ISSN: 1528-3933 ISO Abbreviation: J AAPOS Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-05-10 Completed Date: 2010-08-04 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9710011 Medline TA: J AAPOS Country: United States |
Other Details:
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Languages: eng Pagination: 155-8 Citation Subset: IM |
Copyright Information:
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Copyright 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved. |
Affiliation:
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Department of Ophthalmology, McGill University, Montreal, Quebec, Canada. lsegal2@gmail.com |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Antineoplastic Agents / therapeutic use Child Child, Preschool Female Follow-Up Studies Humans Magnetic Resonance Imaging Male Neurofibromatosis 1 / complications*, diagnosis, therapy Optic Chiasm / pathology* Optic Nerve Glioma / complications*, diagnosis, therapy Optic Nerve Neoplasms / complications*, diagnosis, therapy Prevalence Prognosis Radiotherapy Retrospective Studies Visual Acuity / physiology Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Antineoplastic Agents |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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