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|Title:||Long-term visual outcomes in patients with orbitotemporal neurofibromatosis|
|Citation:||Clinical and Experimental Ophthalmology, 2014; 42(3):266-270|
|Publisher:||Blackwell Publishing Asia|
|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|
|Appears in Collections:||Opthalmology & Visual Sciences publications|
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