{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T16:46:39Z","timestamp":1787071599094,"version":"build-2736575974"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","license":[{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>\n                    A\n                    <jats:sc>BSTRACT<\/jats:sc>\n                  <\/jats:title>\n                  <jats:p>Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive disorder characterized by congenital abnormalities such as ectodermal dysplasia, post-axial polydactyly, chondrodysplasia, and congenital heart defects. The syndrome presents unique challenges in pediatric dentistry due to its diverse oral manifestations and the need for multidisciplinary care. This case report presents a 12-year-old male with EVC syndrome presented with bilateral polydactyly, a syndromic facial appearance, and congenital heart defects. Clinical and radiographic evaluation revealed several missing and malformed teeth. A comprehensive treatment plan was formulated which included prosthetic rehabilitation with removable partial dentures. Regular follow-ups were conducted to assess overall oral health. Multidisciplinary management, including dental, cardiac, and psychological care, is crucial for optimizing patient outcomes. Early diagnosis and treatment are crucial for improving oral health, function, and quality of life, thereby enhancing the psychological well-being and self-esteem of affected children.<\/jats:p>","DOI":"10.4103\/jfmpc.jfmpc_184_25","type":"journal-article","created":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T15:04:46Z","timestamp":1758639886000},"page":"3578-3581","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Navigating complexities: A pediatric case of Ellis-van Creveld syndrome"],"prefix":"10.4103","volume":"14","author":[{"given":"Mridula","family":"Goswami","sequence":"first","affiliation":[{"name":"Department of Pediatric and Preventive Dentistry, Maulana Azad Institute of Dental Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riya Marie","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Pediatric and Preventive Dentistry, Maulana Azad Institute of Dental Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Rimshheanam","sequence":"additional","affiliation":[{"name":"Department of Pediatric and Preventive Dentistry, Maulana Azad Institute of Dental Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,9,24]]},"reference":[{"key":"R1-20250924","doi-asserted-by":"crossref","first-page":"2527","DOI":"10.18203\/2320-1770.ijrcog20222323","article-title":"Pregnancy in Ellis Van Creveld syndrome women:A rare case report","volume":"11","author":"Sunanda","year":"2022","journal-title":"Int J Reprod Contracept Obstet Gynecol"},{"key":"R2-20250924","doi-asserted-by":"crossref","first-page":"167","DOI":"10.5624\/isd.2011.41.4.167","article-title":"Ellis-van Creveld syndrome in an Indian child:A case report","volume":"41","author":"Veena","year":"2011","journal-title":"Imaging Sci Dent"},{"key":"R3-20250924","doi-asserted-by":"crossref","first-page":"538","DOI":"10.32677\/ijcr.v7i12.3230","article-title":"Ellis van Creveld syndrome:An unusual presentation at birth","volume":"7","author":"Singh","year":"2022","journal-title":"Indian J Case Rep"},{"key":"R4-20250924","doi-asserted-by":"crossref","first-page":"e2183","DOI":"10.1002\/mgg3.2183","article-title":"A homozygous EVC mutation in a prenatal fetus with Ellis-van Creveld syndrome","volume":"11","author":"Wang","year":"2023","journal-title":"Mol Genet Genomic Med"},{"key":"R5-20250924","doi-asserted-by":"crossref","first-page":"407","DOI":"10.5005\/jp-journals-10005-1785","article-title":"Prevalence of shape-related developmental dental anomalies in India:A retrospective study","volume":"13","author":"Goswami","year":"2020","journal-title":"Int J Clin Pediatr Dent"},{"key":"R6-20250924","doi-asserted-by":"crossref","first-page":"391","DOI":"10.47391\/JPMA.7049","article-title":"Ellis-van Creveld syndrome:A case report","volume":"74","author":"Hameed","year":"2024","journal-title":"J Pak Med Assoc"},{"key":"R7-20250924","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1161\/01.CIR.96.1.358","article-title":"Prevention of bacterial endocarditis. Recommendations by the American Heart Association","volume":"96","author":"Dajani","year":"1997","journal-title":"Circulation"},{"key":"R8-20250924","doi-asserted-by":"crossref","first-page":"S55","DOI":"10.4103\/0976-237X.95106","article-title":"Oral manifestations of Ellis-van Creveld syndrome","volume":"3","author":"Kalaskar","year":"2012","journal-title":"Contemp Clin Dent"},{"key":"R9-20250924","first-page":"e369","article-title":"Discordance for Ellis-van Creveld syndrome in twins","volume":"49","author":"Thapa","year":"2008","journal-title":"Singapore Med J"},{"key":"R10-20250924","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1136\/adc.15.82.65","article-title":"A Syndrome characterized by ectodermal dysplasia, polydactyly, chondro-dysplasia and congenital morbus cordis:Report of three cases","volume":"15","author":"Ellis","year":"1940","journal-title":"Arch Dis Child"},{"key":"R11-20250924","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/j.ihj.2012.06.011","article-title":"Late survival in Ellis-van Creveld syndrome-A case report","volume":"64","author":"Mishra","year":"2012","journal-title":"Indian Heart J"},{"key":"R12-20250924","doi-asserted-by":"crossref","first-page":"e885","DOI":"10.1002\/mgg3.885","article-title":"Identification of a novel EVC variant in a Han-Chinese family with Ellis-van Creveld syndrome","volume":"7","author":"Huang","year":"2019","journal-title":"Mol Genet Genomic Med"},{"key":"R13-20250924","doi-asserted-by":"crossref","first-page":"e239663","DOI":"10.1136\/bcr-2020-239663","article-title":"Late survival in Ellis-van Creveld syndrome with common single atrium","volume":"14","author":"Prajapati","year":"2021","journal-title":"BMJ Case Rep"},{"key":"R14-20250924","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1136\/jmg-2022-108435","article-title":"Ellis-van Creveld Syndrome:Clinical and molecular analysis of 50 individuals","volume":"60","author":"Aubert-Mucca","year":"2023","journal-title":"J Med Genet"},{"key":"R15-20250924","doi-asserted-by":"crossref","first-page":"100781","DOI":"10.1016\/j.gimo.2023.100781","article-title":"Establishing an objective clinical spectrum, genotype-phenotype correlations, and CRMP1 as a modifier in the Ellis-van Creveld syndrome:The first systematic review of EVC- and EVC2-associated conditions","volume":"1","author":"Da","year":"2023","journal-title":"Genet Med Open"}],"container-title":["Journal of Family Medicine and Primary Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/jfmpc.jfmpc_184_25","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T15:49:17Z","timestamp":1787068157000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/jfmpc.jfmpc_184_25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8]]},"references-count":15,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2025]]}},"URL":"https:\/\/doi.org\/10.4103\/jfmpc.jfmpc_184_25","relation":{},"ISSN":["2249-4863","2278-7135"],"issn-type":[{"value":"2249-4863","type":"print"},{"value":"2278-7135","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,8]]},"assertion":[{"value":"2025-01-28","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-25","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}