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Oral manifestations in Ellis-van Creveld syndrome: report of five cases.
Pediatr Dent. 2004 May-Jun; 26(3):277-82.PD

Abstract

Ellis-van Creveld syndrome, or chondroectodermal dysplasia, is an autosomal recessive disorder with characteristic clinical manifestations. Its incidence in the general population is low. The oral manifestations of Ellis-van Creveld are found in soft tissues and teeth, but the dental literature on the subject is scarce. In the last 20 years, 5 cases of Ellis-van Creveld syndrome have been followed at the Pediatric Dentistry Service of the Hospital Sant Joan de Déu, Barcelona. The present study describes the constant and variable oral findings in these patients, which play an important role in the diagnosis criteria for the syndrome. The presence of a great variety of oral manifestations such as fusion of the upper lip to the gingival margin, presence of multiple frenula, abnormally shaped and microdontic teeth, and congenitally missing teeth requires multidisciplinary dental treatment, with consideration for the high incidence of cardiac defects in these patients.

Authors+Show Affiliations

Pediatric Dentistry and Orthodontics Service, University of Barcelona, Barcelona, Spain. acahuana@hsjdbcn.orgNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15185812

Citation

Cahuana, Abel, et al. "Oral Manifestations in Ellis-van Creveld Syndrome: Report of Five Cases." Pediatric Dentistry, vol. 26, no. 3, 2004, pp. 277-82.
Cahuana A, Palma C, Gonzáles W, et al. Oral manifestations in Ellis-van Creveld syndrome: report of five cases. Pediatr Dent. 2004;26(3):277-82.
Cahuana, A., Palma, C., Gonzáles, W., & Geán, E. (2004). Oral manifestations in Ellis-van Creveld syndrome: report of five cases. Pediatric Dentistry, 26(3), 277-82.
Cahuana A, et al. Oral Manifestations in Ellis-van Creveld Syndrome: Report of Five Cases. Pediatr Dent. 2004 May-Jun;26(3):277-82. PubMed PMID: 15185812.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Oral manifestations in Ellis-van Creveld syndrome: report of five cases. AU - Cahuana,Abel, AU - Palma,Camila, AU - Gonzáles,Wilber, AU - Geán,Esther, PY - 2004/6/10/pubmed PY - 2004/8/18/medline PY - 2004/6/10/entrez SP - 277 EP - 82 JF - Pediatric dentistry JO - Pediatr Dent VL - 26 IS - 3 N2 - Ellis-van Creveld syndrome, or chondroectodermal dysplasia, is an autosomal recessive disorder with characteristic clinical manifestations. Its incidence in the general population is low. The oral manifestations of Ellis-van Creveld are found in soft tissues and teeth, but the dental literature on the subject is scarce. In the last 20 years, 5 cases of Ellis-van Creveld syndrome have been followed at the Pediatric Dentistry Service of the Hospital Sant Joan de Déu, Barcelona. The present study describes the constant and variable oral findings in these patients, which play an important role in the diagnosis criteria for the syndrome. The presence of a great variety of oral manifestations such as fusion of the upper lip to the gingival margin, presence of multiple frenula, abnormally shaped and microdontic teeth, and congenitally missing teeth requires multidisciplinary dental treatment, with consideration for the high incidence of cardiac defects in these patients. SN - 0164-1263 UR - https://www.unboundmedicine.com/medline/citation/15185812/Oral_manifestations_in_Ellis_van_Creveld_syndrome:_report_of_five_cases_ L2 - https://www.ingentaconnect.com/openurl?genre=article&issn=0164-1263&volume=26&issue=3&spage=277&aulast=Cahuana DB - PRIME DP - Unbound Medicine ER -