{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T18:23:23Z","timestamp":1781029403462,"version":"3.54.1"},"reference-count":28,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2024,7,5]],"date-time":"2024-07-05T00:00:00Z","timestamp":1720137600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background\/Objectives: To evaluate how the surgical technique and type of implanted intraocular lens influence the postoperative visual acuity and complications in ectopia lentis associated to Marfan syndrome patients. Materials and Methods: The medical records and videos of ectopia lentis surgeries in patients (children and adults) with Marfan syndrome, were retrospectively reviewed and compared. The study included 33 eyes that underwent four different intraocular lens implantation (IOL) techniques: IOL in conjunction with a simple capsular tension ring, IOL in conjunction with a Cionni modified capsular tension ring (m-CTR), two-point scleral IOL fixation and IOL with one haptic in the bag and one haptic sutured to the sclera. Results: Vision significantly improved from a mean preoperative visual acuity of 0.1122 to a mean postoperative visual acuity of 0.4539 in both age groups (p &lt; 0.0001), with no difference in the primary outcome between children and adults. The most common surgical technique used in both age groups was IOL in conjunction with an m-CTR. There was only one major postoperative complication requiring additional surgery. Conclusions: Zonular weakness mainly influenced by age was the most important selection criterion for the surgical approach. Regardless of the technique employed, the postoperative visual acuity was improved in both adults and children.<\/jats:p>","DOI":"10.3390\/medicina60071098","type":"journal-article","created":{"date-parts":[[2024,7,5]],"date-time":"2024-07-05T03:48:56Z","timestamp":1720151336000},"page":"1098","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Visual Outcomes in Ectopia Lentis in Marfan Syndrome: A Study of Four Surgical Techniques in Children and Adults"],"prefix":"10.3390","volume":"60","author":[{"given":"Anca Cristina","family":"Dogaroiu","sequence":"first","affiliation":[{"name":"Alcor Clinic, 030829 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5071-9756","authenticated-orcid":false,"given":"Maria","family":"Dudau","sequence":"additional","affiliation":[{"name":"Laboratory of Biochemistry, \u201cVictor Babes\u201d National Institute of Pathology, 050096 Bucharest, Romania"},{"name":"Department of Cell Biology and Histology, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 020021 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catalin","family":"Dogaroiu","sequence":"additional","affiliation":[{"name":"Department of Morphological Sciences, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 020021 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Calin Petru","family":"Tataru","sequence":"additional","affiliation":[{"name":"Alcor Clinic, 030829 Bucharest, Romania"},{"name":"Department of Ophthalmology, \u201cCarol Davila\u201d University of Medicine and Pharmacy, 020021 Bucharest, Romania"},{"name":"Clinical Hospital for Ophthalmological Emergencies, 010464 Bucharest, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,7,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1172\/JCI115589","article-title":"Marfan syndrome: Defective synthesis, secretion, and extracellular matrix formation of fibrillin by cultured dermal fibroblasts","volume":"89","author":"Pyeritz","year":"1992","journal-title":"J. Clin. Investig."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"7321","DOI":"10.3892\/mmr.2017.7544","article-title":"A novel FBN1 mutation causes autosomal dominant Marfan syndrome","volume":"16","author":"Xiao","year":"2017","journal-title":"Mol. Med. Rep."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2499","DOI":"10.1083\/jcb.103.6.2499","article-title":"Fibrillin, a new 350-kD glycoprotein, is a component ofextracellular microfibrils","volume":"103","author":"Sakai","year":"1986","journal-title":"J. Cell Biol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J. Med. Gen."},{"key":"ref_5","first-page":"684","article-title":"The eye in the Marfan syndrome","volume":"79","author":"Maumenee","year":"1981","journal-title":"Trans. Am. Ophthalmol. Soc."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"135","DOI":"10.3109\/13816818809031488","article-title":"Unilateral lens dislocation and axial elongation in Marfan syndrome","volume":"9","author":"Rasooly","year":"1988","journal-title":"Ophthalmic Paediatr. Genet."},{"key":"ref_7","unstructured":"Colenbrander, A. (2002, January 17\u201318). Visual standards: Aspects and ranges of vision loss with emphasis on population surveys. Proceedings of the 29th International Congress of Ophthalmology (ICO), Sydney, Australia."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.2147\/OPTH.S256851","article-title":"Effects on IOL Power Calculation and Expected Clinical Outcomes of Axial Length Measurements Based on Multiple vs. Single Refractive Indices","volume":"14","author":"Shammas","year":"2020","journal-title":"Clin. Ophthalmol."},{"key":"ref_9","first-page":"149","article-title":"Retropupillary iris-claw intraocular lens in ectopia lentis in Marfan syndrome","volume":"9","author":"Faria","year":"2016","journal-title":"Int. Med. Case Rep. J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1111\/j.1442-9071.2011.02612.x","article-title":"Comparison of clinical outcomes between iris-fixated anterior chamber intraocular lenses and scleral-fixated posterior chamber intraocular lenses in Marfan syndrome with lens subluxation","volume":"40","author":"Zheng","year":"2012","journal-title":"Clin. Exp. Ophthalmol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"3285","DOI":"10.1093\/eurheartj\/ehv739","article-title":"Genotype impacts survival in Marfan syndrome","volume":"37","author":"Franken","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1161\/CIRCGENETICS.116.001647","article-title":"Associations of Age and Sex with Marfan Phenotype: The National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry","volume":"10","author":"Roman","year":"2017","journal-title":"Circ. Cardiovasc. Genet."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2994","DOI":"10.1038\/s41598-021-82586-6","article-title":"Visual outcomes of lens subluxation surgery with Cionni modified capsular tension rings in Marfan syndrome","volume":"11","author":"Chen","year":"2021","journal-title":"Sci. Rep."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1186\/s40064-015-1239-5","article-title":"How far is observation allowed in patients with ectopia lentis?","volume":"4","author":"Matsuo","year":"2015","journal-title":"SpringerPlus"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1016\/j.jcrs.2004.11.043","article-title":"Intralenticular bimanual irrigation: Aspiration for subluxated lens in Marfan\u2019s syndrome","volume":"31","author":"Sinha","year":"2005","journal-title":"J. Cataract. Refract. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1016\/j.ajo.2017.02.022","article-title":"Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago","volume":"177","author":"Kinori","year":"2017","journal-title":"Am. J. Ophthalmol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"71","DOI":"10.4103\/jovr.jovr_29_18","article-title":"Management strategies of ocular abnormalities in patients with Marfan syndrome: Current perspective","volume":"14","author":"Esfandiari","year":"2019","journal-title":"J. Ophthalmic Vis. Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1177\/1120672121992950","article-title":"Outcomes of three surgical approaches for managing ectopia lentis in Marfan syndrome","volume":"32","author":"Erdogan","year":"2022","journal-title":"Eur. J. Ophthalmol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Gehle, P., Goergen, B., Pilger, D., Ruokonen, P., Robinson, P.N., and Salchow, D.J. (2017). Biometric and structural ocular manifestations of Marfan syndrome. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0183370"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1111\/j.1755-3768.2010.01996.x","article-title":"Corneal curvature, pachymetry, and endothelial cell density in Marfan syndrome","volume":"90","author":"Konradsen","year":"2012","journal-title":"Acta Ophthalmol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"4079","DOI":"10.1038\/s41598-018-22358-x","article-title":"Corneal Curvature, Astigmatism, and Aberrations in Marfan Syndrome with Lens Subluxation: Evaluation by Pentacam HR System","volume":"8","author":"Chen","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1436834","DOI":"10.1155\/2018\/1436834","article-title":"Age Differences in Axial Length, Corneal Curvature, and Corneal Astigmatism in Marfan Syndrome with Ectopia Lentis","volume":"2018","author":"Chen","year":"2018","journal-title":"J. Ophthalmol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/S0161-6420(96)30633-7","article-title":"Surgery of the hereditary subluxated lens in children","volume":"103","author":"Halpert","year":"1996","journal-title":"Ophthalmology"},{"key":"ref_24","first-page":"71","article-title":"Optical correction of aphakia in children","volume":"9","author":"Shirzadeh","year":"2014","journal-title":"J. Ophthalmic Vis. Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1111\/ceo.13596","article-title":"Visual outcome and surgical results in children with Marfan syndrome","volume":"47","author":"Stifter","year":"2019","journal-title":"Clin. Exp. Ophthalmol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1531","DOI":"10.1007\/s10792-020-01322-7","article-title":"Outcome of sutured scleral fixated intraocular lens in Marfan syndrome in pediatric eyes","volume":"40","author":"Sen","year":"2020","journal-title":"Int. Ophthalmol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.ajo.2015.09.025","article-title":"Long-Term Outcomes of Iris-sutured Posterior Chamber Intraocular Lenses in Children","volume":"161","author":"Shah","year":"2016","journal-title":"Am. J. Ophthalmol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"401","DOI":"10.3928\/01913913-20210428-02","article-title":"Sutureless Scleral-Fixated Intraocular Lens Implantation in the Treatment of Congenital Lens Subluxation Related to Marfan Syndrome","volume":"58","author":"Bulut","year":"2021","journal-title":"J. Pediatr. Ophthalmol. Strabismus"}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/7\/1098\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:10:26Z","timestamp":1760109026000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/7\/1098"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,5]]},"references-count":28,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2024,7]]}},"alternative-id":["medicina60071098"],"URL":"https:\/\/doi.org\/10.3390\/medicina60071098","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,5]]}}}