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Type: Journal article
Title: Long-term visual outcomes in patients with orbitotemporal neurofibromatosis
Author: Greenwell, T.
Anderson, P.
Madge, S.
Selva-Nayagam, D.
David, D.
Citation: Clinical and Experimental Ophthalmology, 2014; 42(3):266-270
Publisher: Blackwell Publishing Asia
Issue Date: 2014
ISSN: 1442-6404
Statement of
Timothy H Greenwell, Peter J Anderson, Simon K Madge, Dinesh Selva and David J David
Abstract: Background: The study aimed to review the presentation and long-term visual outcomes of patients with orbitotemporal neurofibromatosis. Design: Retrospective case series. Participants: Patients with orbitotemporal neurofibromatosis presenting from 1981 to 2009. Methods: Demographic data, examination findings, causes of vision impairment and interventions performed were recorded for each patient from presentation through subsequent follow-up encounters. Visual impairment was defined as an ipsilateral Snellen acuity of <6/12. Main Outcome Measures: The proportion of patients with visual impairment or enucleation, the rate of new vision loss during follow up; and causes for vision loss or enucleation. Results: Thirty-seven patients (17 female) were included. Median presenting age was 15 years (range 2–45) with an average follow up of 7.4 years (range 0.5–20.3). Visual impairment occurred in 54% of patients at presentation. Causes were amblyopia (13 of 37), optic atrophy (4 of 37), previous enucleation/evisceration (2 of 37), and optic nerve glioma (1 of 37). At presentation, 76% of patients had ptosis, and 51% had strabismus. Thirty-one patients had surgery, with an average of two procedures per patient. At final follow up, 62% had visual impairment. The rate of visual decline was 2% per patient-years. Causes of visual decline were two patients with optic nerve atrophy, one with exposure keratitis and one whose cause was unknown. Five blind patients had enucleation. Conclusions: The first series of orbitotemporal neurofibromatosis to focus on visual outcomes was presented. Vision loss is common, with a high prevalence of amblyopia. Close monitoring from an early age is needed to prevent visual impairment.
Keywords: craniofacial surgery; neurofibromatosis type 1; orbitofacial tumours
Description: Article first published online: 13 SEP 2013
Rights: © 2013 Royal Australian and New Zealand College of Ophthalmologists
RMID: 0020135436
DOI: 10.1111/ceo.12179
Appears in Collections:Opthalmology & Visual Sciences publications

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