{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T02:04:05Z","timestamp":1788141845400,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,7,12]],"date-time":"2006-07-12T00:00:00Z","timestamp":1152662400000},"content-version":"vor","delay-in-days":11,"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":[[2006,7]]},"abstract":"<jats:title>A<jats:sc>bstract<\/jats:sc><\/jats:title><jats:p><jats:bold>Background:\u2002<\/jats:bold> To assess the prevalence and causes of visual impairment in patients with craniosynostotic syndromes of Apert, Crouzon, Pfeiffer, Saethre\u2013Chotzen and craniofrontonasal dysplasia.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold> The medical records of patients who attended the Craniofacial Clinic at two large paediatric hospitals in Sydney, Australia between 1983 and 2004 were retrospectively reviewed. Presenting visual acuity (VA) was assessed using tests appropriate to age and cognition: \u2018fix and follow\u2019 in infants (&lt;18\u2003months old), Teller card acuity in preverbal children (18\u2003months to less than 3\u2003years old), Kay picture test or Sheridan\u2013Gardiner test in children aged between 3 and less than 6\u2003years and Snellen chart in those aged 6\u2003years or older. Visual impairment was defined as the inability to fix and follow or presenting VA\u2003&lt;\u20036\/12 in the better eye. Amblyopia was defined as a two\u2010line difference in VA between both eyes in the absence of an organic eye disease.<\/jats:p><jats:p><jats:bold>Results:\u2002<\/jats:bold> Sixty\u2010three patients with craniosynostotic syndromes were identified, of whom 55 had VA assessed at the first visit. Of these 55, 19 (35.5%) had bilateral visual impairment and 5 (9.1%) had unilateral visual impairment. Causes of visual impairment include amblyopia (16.7%), ametropia (25%), optic atrophy (16.7%) and exposure keratopathy (4.2%). Risk factors for amblyopia include strabismus (43.3%), astigmatism (\u22651.5 dioptres) (39.5%), hypermetropia (18.4%) and anisometropia (\u22651.5 dioptre difference between both eyes) (15.8%). Six of the 63 patients (9.5%) had papilloedema; those who were followed up showed gradual resolution of papilloedema following timely decompressive surgery.<\/jats:p><jats:p><jats:bold>Conclusions:\u2002<\/jats:bold> A high prevalence of visual impairment in patients with craniosynostotic syndromes was found, almost half of them due to potentially correctable causes, including amblyopia and ametropia. Optic atrophy remains an important cause of visual impairment. Further studies are needed to assess the timing and efficacy of intervention for modifiable causes of visual loss in craniosynostotic syndromes.<\/jats:p>","DOI":"10.1111\/j.1442-9071.2006.01242.x","type":"journal-article","created":{"date-parts":[[2006,7,12]],"date-time":"2006-07-12T09:08:40Z","timestamp":1152695320000},"page":"434-440","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":93,"title":["Prevalence and causes of visual impairment in craniosynostotic syndromes"],"prefix":"10.1111","volume":"34","author":[{"given":"Tien","family":"Tay","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Martin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Rowe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim","family":"Johnson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Poole","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberley","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian","family":"Kennedy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Gianoutsos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,12]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320550422"},{"key":"e_1_2_6_3_2","volume-title":"Craniosynostosis: Diagnosis, Evaluation, and Management","author":"Cohen MM","year":"2000"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0039-6257(90)90067-6"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.77.9.563"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.87.8.999"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2004.12.037"},{"key":"e_1_2_6_8_2","doi-asserted-by":"crossref","first-page":"344","DOI":"10.3928\/0191-3913-19911101-14","article-title":"Visual loss in patients with craniofacial synostosis","volume":"28","author":"Hertle RW","year":"1991","journal-title":"J Pediatr Ophthalmol Strabismus"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1038\/eye.1990.124"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.70.1.12"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(00)00623-1"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.03-0945"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2004.03.013"},{"key":"e_1_2_6_14_2","first-page":"184","volume-title":"Craniosynostosis: Diagnosis, Evaluation, and Management","author":"Miller M","year":"2000"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)74862-7"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9394(14)74927-X"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1067\/mpa.2000.110337"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1097\/00006123-199602000-00009"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1159\/000121038"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1159\/000028746"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00947081"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0402819101"}],"container-title":["Clinical &amp; Experimental Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1442-9071.2006.01242.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1442-9071.2006.01242.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T05:22:13Z","timestamp":1696915333000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1442-9071.2006.01242.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,7]]},"references-count":21,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2006,7]]}},"alternative-id":["10.1111\/j.1442-9071.2006.01242.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1442-9071.2006.01242.x","archive":["Portico"],"relation":{},"ISSN":["1442-6404","1442-9071"],"issn-type":[{"value":"1442-6404","type":"print"},{"value":"1442-9071","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,7]]},"assertion":[{"value":"2006-07-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}