{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T07:10:04Z","timestamp":1756278604587,"version":"3.44.0"},"reference-count":7,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2018,7,14]]},"abstract":"<jats:p>Brittle cornea syndrome (BCS1 OMIM #229200, BCS2 #614170) is a rare autosomal recessive condition characterised by diffuse thinning and fragility of the cornea. Affected individuals are at risk of globe rupture and blindness after relatively minor eye trauma. We describe a 9-year-old girl with BCS1, already blind in one eye following trauma, who had a 14\u2009mm diameter corneoscleral onlay graft to her contralateral eye to reduce gross irregular corneal astigmatism and potentially to reduce further risk from accidental injury. Although there was a significant initial improvement in the unaided visual acuity, there was subsequent visual loss from secondary glaucoma. In addition, despite the onlay graft, an acute corneal hydrops developed approximately 2 years following surgery, suggesting that in BCS1, corneal tissue degeneration or resorption continues despite external support. Finally, because secondary glaucoma is not a feature of BCS1, we speculate that the onlay graft may have reduced aqueous outflow by compression of the thinned sclera.<\/jats:p>","DOI":"10.1136\/bcr-2017-223824","type":"journal-article","created":{"date-parts":[[2018,8,16]],"date-time":"2018-08-16T09:59:51Z","timestamp":1534413591000},"page":"bcr-2017-223824","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Use of an onlay corneal lamellar graft for brittle cornea syndrome"],"prefix":"10.1136","volume":"2018","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6009-0923","authenticated-orcid":false,"given":"Kirithika","family":"Muthusamy","sequence":"first","affiliation":[{"name":"Moorfields Eye Hospital NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen","family":"Tuft","sequence":"additional","affiliation":[{"name":"Moorfields Eye Hospital NHS Foundation Trust, London, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2018,8,16]]},"reference":[{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-68"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.2","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1097\/ICO.0b013e3182a9b1ac","article-title":"Limbal stem cell-sparing lamellar keratoplasty for the management of advanced keratoglobus","volume":"33","author":"Karimian","year":"2014","journal-title":"Cornea"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.3","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.14-15792"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2008.04.001"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.5","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.05.007"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.6","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1097\/01.ico.0000244878.38621.fc","article-title":"Outcomes of epikeratoplasty for advanced keratoglobus","volume":"26","author":"Javadi","year":"2007","journal-title":"Cornea"},{"key":"2025082623444739000_2018.aug16_1.bcr-2017-223824.7","doi-asserted-by":"publisher","DOI":"10.1097\/00003226-200001000-00020"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2017-223824","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T06:44:58Z","timestamp":1756277098000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2017-223824"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8,16]]},"references-count":7,"alternative-id":["10.1136\/bcr-2017-223824"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2017-223824","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2018,8,16]]}}}