{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T22:53:40Z","timestamp":1773356020400,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,9,13]],"date-time":"2013-09-13T00:00:00Z","timestamp":1379030400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Exper Ophthalmology"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The study aimed to review the presentation and long\u2010term visual outcomes of patients with orbitotemporal neurofibromatosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>Retrospective case series.<\/jats:p><\/jats:sec><jats:sec><jats:title>Participants<\/jats:title><jats:p>Patients with orbitotemporal neurofibromatosis presenting from 1981 to 2009.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Demographic data, examination findings, causes of vision impairment and interventions performed were recorded for each patient from presentation through subsequent follow\u2010up encounters. Visual impairment was defined as an ipsilateral <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>nellen acuity of &lt;6\/12.<\/jats:p><\/jats:sec><jats:sec><jats:title>Main Outcome Measures<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010seven patients (17 female) were included. Median presenting age was 15 years (range 2\u201345) with an average follow up of 7.4 years (range 0.5\u201320.3). 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\u2010one 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\u2010years. 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ceo.12179","type":"journal-article","created":{"date-parts":[[2013,8,8]],"date-time":"2013-08-08T15:38:30Z","timestamp":1375976310000},"page":"266-270","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Long\u2010term visual outcomes in patients with orbitotemporal neurofibromatosis"],"prefix":"10.1111","volume":"42","author":[{"given":"Timothy H","family":"Greenwell","sequence":"first","affiliation":[{"name":"South Australian Institute of Ophthalmology  Adelaide South Australia Australia"},{"name":"Discipline of Ophthalmology and Visual Sciences University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter J","family":"Anderson","sequence":"additional","affiliation":[{"name":"Australian Craniofacial Unit  Adelaide South Australia Australia"},{"name":"Department of Surgery University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon K","family":"Madge","sequence":"additional","affiliation":[{"name":"South Australian Institute of Ophthalmology  Adelaide South Australia Australia"},{"name":"Discipline of Ophthalmology and Visual Sciences University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dinesh","family":"Selva","sequence":"additional","affiliation":[{"name":"South Australian Institute of Ophthalmology  Adelaide South Australia Australia"},{"name":"Discipline of Ophthalmology and Visual Sciences University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David J","family":"David","sequence":"additional","affiliation":[{"name":"Australian Craniofacial Unit  Adelaide South Australia Australia"},{"name":"Department of Surgery University of Adelaide  Adelaide South Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,9,13]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-198010000-00003"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-199307000-00001"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1988.00520290115023"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1986.tb48060.x"},{"key":"e_1_2_5_6_1","first-page":"424","article-title":"Deletions spanning the neurofibromatosis 1 gene: identification and phenotype of five patients","volume":"54","author":"Kayes LM","year":"1994","journal-title":"Am J Hum Genet"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-198306000-00001"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2003.05.029"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2005.10.011"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2004.12.037"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/eye.2009.6"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2003.06.008"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2009.06.019"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(98)91862-0"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1076\/0928-6586(200012)741-YFT249"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000637-198203000-00008"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0007-1226(03)00172-3"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-198404000-00003"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/00002341-198804040-00002"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006534-199809010-00005"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(83)34371-2"}],"container-title":["Clinical &amp; 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