{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:10:57Z","timestamp":1753891857875,"version":"3.41.2"},"reference-count":27,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T00:00:00Z","timestamp":1634688000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Ophthalmol."],"abstract":"<jats:sec><jats:title>Purpose<\/jats:title><jats:p>To assess the choroidal structure using the Choroidal Vascularity Index (CVI) and analyse choroidal changes in choroideremia (CHM) carriers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and Methods<\/jats:title><jats:p>Female CHM carriers, genetically characterized, and a control group were recruited at the Eye Clinic of Careggi Teaching Hospital, Florence. The patients underwent a complete ophthalmic evaluation and retinal imaging. In particular, the Stromal Area (SA), Luminal Area (LA), Total Choroidal Area (TCA), CVI, and Subfoveal Choroidal Thickness (SFCT) were calculated for each eye using Optical Coherence Tomography (OCT) examinations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twelve eyes of 6 CHM carriers and 14 eyes of 7 age-matched controls were analysed. The mean SFCT was 270.9 \u00b1 54.3\u03bcm in carriers and 281.4 \u00b1 36.8\u03bcm in controls (p = 0.564); LA was 0.99 \u00b1 0.25mm<jats:sup>2<\/jats:sup> and 1.01 \u00b1 0.13mm<jats:sup>2<\/jats:sup> (p = 0.172); SA was 0.53 \u00b1 0.09mm<jats:sup>2<\/jats:sup> and 0.59 \u00b1 0.07mm<jats:sup>2<\/jats:sup> (p = 0.075), and TCA was 1.53 \u00b1 0.34mm<jats:sup>2<\/jats:sup> and 1.69 \u00b1 0.19mm<jats:sup>2<\/jats:sup> respectively (p = 0.146). Mean CVI measured 64.03 \u00b1 3.98% in the CHM carriers and 65.25 \u00b1 2.55% in the controls (p = 0.360).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The CVI and CVI-related parameters (SA, LA, and TCA) do not differ between CHM female carriers and controls. These findings reveal a preserved choroidal vasculature in eyes with RPE impairment and support the primary role of RPE in the pathogenesis of CHM disease.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fopht.2021.755058","type":"journal-article","created":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T23:06:52Z","timestamp":1634771212000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Choroidal Vascularity Index in CHM Carriers"],"prefix":"10.3389","volume":"1","author":[{"given":"Dario Pasquale","family":"Mucciolo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dario","family":"Giorgio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Myrta","family":"Lippera","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Passerini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabetta","family":"Pelo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Cipollini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Sodi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianni","family":"Virgili","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabrizio","family":"Giansanti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vittoria","family":"Murro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,10,20]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(1997)9:2<110::AID-HUMU2>3.0.CO;2-D","article-title":"Molecular Basis of Choroideremia (CHM): Mutations Involving the Rab Escort Protein-1 (REP-1) Gene","volume":"9","author":"Van den Hurk","year":"1997","journal-title":"Hum Mutat"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.15-18421","article-title":"Visual Function and Central Retinal Structure in Choroideremia","volume":"57","author":"Heon","year":"2016","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-017-3751-5","article-title":"Retinal Dystrophy and Subretinal Drusenoid Deposits in Female Choroideremia Carriers","volume":"255","author":"Murro","year":"2017","journal-title":"Graefes Arch Clin Exp Ophthalmol Albrecht Von Graefes Arch Klin Exp Ophthalmol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.preteyeres.2020.100829","article-title":"Exploring Choroidal Angioarchitecture in Health and Disease Using Choroidal Vascularity Index","volume":"77","author":"Agrawal","year":"2020","journal-title":"Prog Retin Eye Res"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.19-27300","article-title":"Evaluation of the Choroid in Eyes With Retinitis Pigmentosa and Cystoid Macular Edema","volume":"60","author":"Iovino","year":"2019","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B6","doi-asserted-by":"publisher","first-page":"595","DOI":"10.3390\/jcm9020595","article-title":"Choroidal Vascularity Index: An In-Depth Analysis of This Novel Optical Coherence Tomography Parameter","volume":"9","author":"Iovino","year":"2020","journal-title":"J Clin Med"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1080\/13816810.2020.1747086","article-title":"Optical Coherence Tomography Angiography (OCT-A) in Choroideremia (CHM) Carriers","volume":"41","author":"Murro","year":"2020","journal-title":"Ophthalmic Genet"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0146344","article-title":"Choroidal Vascularity Index (CVI)\u2013A Novel Optical Coherence Tomography Parameter for Monitoring Patients With Panuveitis","volume":"11","author":"Agrawal","year":"2016","journal-title":"PloS One"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1038\/srep21090","article-title":"Choroidal Vascularity Index as a Measure of Vascular Status of the Choroid: Measurements in Healthy Eyes From a Population-Based Study","volume":"6","author":"Agrawal","year":"2016","journal-title":"Sci Rep"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2006.05.045","article-title":"Choroideremia: Variability of Clinical and Electrophysiological Characteristics and First Report of a Negative Electroretinogram","volume":"113","author":"Renner","year":"2006","journal-title":"Ophthalmology"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2009.01.016","article-title":"Fundus Autofluorescence in Carriers of Choroideremia and Correlation With Electrophysiologic and Psychophysical Data","volume":"116","author":"Preising","year":"2009","journal-title":"Ophthalmology"},{"key":"B12","article-title":"A Histopathologic Study of a Choroideremia Carrier","volume":"31","author":"Flannery","year":"1990","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.13-13454","article-title":"High-Resolution Adaptive Optics Retinal Imaging of Cellular Structure in Choroideremia","volume":"55","author":"Morgan","year":"2014","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1016\/s0161-6420(00)00643-6","article-title":"Evaluation of Retinal Photoreceptors and Pigment Epithelium in a Female Carrier of Choroideremia","volume":"108","author":"Syed","year":"2001","journal-title":"Ophthalmology"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.06-0424","article-title":"Remodeling of the Human Retina in Choroideremia: Rab Escort Protein 1 (REP-1) Mutations","volume":"47","author":"Jacobson","year":"2006","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B16","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1080\/13816810802206499","article-title":"Choroideremia: Analysis of the Retina From a Female Symptomatic Carrier","volume":"29","author":"Bonilha","year":"2008","journal-title":"Ophthalmic Genet"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.12-10707","article-title":"High-Resolution Images of Retinal Structure in Patients With Choroideremia","volume":"54","author":"Syed","year":"2013","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.survophthal.2009.02.008","article-title":"Choroideremia: New Findings From Ocular Pathology and Review of Recent Literature","volume":"54","author":"MacDonald","year":"2009","journal-title":"Surv Ophthalmol"},{"key":"B19","article-title":"Histopathology of the Retinal Pigment Epithelium of a Female Carrier of Choroideremia","volume":"32","author":"MacDonald","year":"1997","journal-title":"Can J Ophthalmol J Can Ophtalmol"},{"key":"B20","doi-asserted-by":"publisher","first-page":"76","DOI":"10.3928\/23258160-20190129-03","article-title":"High-Resolution Retinal Imaging Reveals Preserved Cone Photoreceptor Density and Choroidal Thickness in Female Carriers of Choroideremia","volume":"50","author":"Suzuki","year":"2019","journal-title":"Ophthalmic Surg Lasers Imaging Retina"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.15-18364","article-title":"Correlation of Optical Coherence Tomography and Autofluorescence in the Outer Retina and Choroid of Patients With Choroideremia","volume":"57","author":"Xue","year":"2016","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.ophtha.2016.06.051","article-title":"Clinical and Genetic Features of Choroideremia in Childhood","volume":"123","author":"Khan","year":"2016","journal-title":"Ophthalmology"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1038\/eye.2017.242","article-title":"Identifying Characteristic Features of the Retinal and Choroidal Vasculature in Choroideremia Using Optical Coherence Tomography Angiography","volume":"32","author":"Abbouda","year":"2018","journal-title":"Eye Lond Engl"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1080\/13816810.2019.1611880","article-title":"Optical Coherence Tomography Angiography (OCT-A) in Young Choroideremia (CHM) Patients","volume":"40","author":"Murro","year":"2019","journal-title":"Ophthalmic Genet"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1097\/IAE.0000000000002960","article-title":"Choroidal Vascularity Index in Young Choroideremia Patients","author":"Murro","year":"2020","journal-title":"Retina Phila Pa"},{"key":"B26","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.ajo.2019.06.002","article-title":"Spectrum of Disease Severity and Phenotype in Choroideremia Carriers","volume":"207","author":"Jauregui","year":"2019","journal-title":"Am J Ophthalmol"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2020.12.015","article-title":"Correlations Between Choriocapillaris and Choroidal Measurements and the Growth of Geographic Atrophy Using Swept Source OCT Imaging","volume":"224","author":"Shi","year":"2020","journal-title":"Am J Ophthalmol"}],"container-title":["Frontiers in Ophthalmology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fopht.2021.755058\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T23:06:55Z","timestamp":1634771215000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fopht.2021.755058\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,20]]},"references-count":27,"alternative-id":["10.3389\/fopht.2021.755058"],"URL":"https:\/\/doi.org\/10.3389\/fopht.2021.755058","relation":{},"ISSN":["2674-0826"],"issn-type":[{"type":"electronic","value":"2674-0826"}],"subject":[],"published":{"date-parts":[[2021,10,20]]},"article-number":"755058"}}