Oral manifestations of Ellis-van Creveld syndrome

Ellis-van Creveld syndrome is a rare autosomal-recessive disorder characterized by short limbs, post-axial polydactyly, ectodermal dysplasia, edentulous mandibular incisor region, absence of mucobuccal fold, congenitally missing teeth, slight serrations of the alveolar ridge and multiple small alveo...

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Main Authors: Ritesh Kalaskar, Ashita R Kalaskar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2012-01-01
Series:Contemporary Clinical Dentistry
Subjects:
Online Access:http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=5;spage=55;epage=59;aulast=Kalaskar
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author Ritesh Kalaskar
Ashita R Kalaskar
author_facet Ritesh Kalaskar
Ashita R Kalaskar
author_sort Ritesh Kalaskar
collection DOAJ
description Ellis-van Creveld syndrome is a rare autosomal-recessive disorder characterized by short limbs, post-axial polydactyly, ectodermal dysplasia, edentulous mandibular incisor region, absence of mucobuccal fold, congenitally missing teeth, slight serrations of the alveolar ridge and multiple small alveolar notches. The clinical report not only describes the classical oral and dental manifestations of Ellis-van Creveld syndrome but also presents unusual findings such as single-rooted and funnel-shaped primary first molars, single conical roots of primary second molars and taurodontisum, which must be considered in the differential diagnostic criteria to avoid misdiagnosis of syndromes. The article also discusses the differential diagnosis and preventive and therapeutic oral health care for these patients. The management of Ellis-van Creveld syndrome is multidisciplinary and, therefore, the oral health care provider should get updated with latest knowledge for timely referral to prevent the patient from further complications of heart defect and bony deformity.
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spelling doaj.art-3e8dc70fc487459198c59dd55981b1a52022-12-21T21:43:54ZengWolters Kluwer Medknow PublicationsContemporary Clinical Dentistry0976-237X0976-23612012-01-0135555910.4103/0976-237X.95106Oral manifestations of Ellis-van Creveld syndromeRitesh KalaskarAshita R KalaskarEllis-van Creveld syndrome is a rare autosomal-recessive disorder characterized by short limbs, post-axial polydactyly, ectodermal dysplasia, edentulous mandibular incisor region, absence of mucobuccal fold, congenitally missing teeth, slight serrations of the alveolar ridge and multiple small alveolar notches. The clinical report not only describes the classical oral and dental manifestations of Ellis-van Creveld syndrome but also presents unusual findings such as single-rooted and funnel-shaped primary first molars, single conical roots of primary second molars and taurodontisum, which must be considered in the differential diagnostic criteria to avoid misdiagnosis of syndromes. The article also discusses the differential diagnosis and preventive and therapeutic oral health care for these patients. The management of Ellis-van Creveld syndrome is multidisciplinary and, therefore, the oral health care provider should get updated with latest knowledge for timely referral to prevent the patient from further complications of heart defect and bony deformity.http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=5;spage=55;epage=59;aulast=KalaskarAbsence of mucobuccal foldalveolar notchesautosomal recessivegenu valgumpartial harelip
spellingShingle Ritesh Kalaskar
Ashita R Kalaskar
Oral manifestations of Ellis-van Creveld syndrome
Contemporary Clinical Dentistry
Absence of mucobuccal fold
alveolar notches
autosomal recessive
genu valgum
partial harelip
title Oral manifestations of Ellis-van Creveld syndrome
title_full Oral manifestations of Ellis-van Creveld syndrome
title_fullStr Oral manifestations of Ellis-van Creveld syndrome
title_full_unstemmed Oral manifestations of Ellis-van Creveld syndrome
title_short Oral manifestations of Ellis-van Creveld syndrome
title_sort oral manifestations of ellis van creveld syndrome
topic Absence of mucobuccal fold
alveolar notches
autosomal recessive
genu valgum
partial harelip
url http://www.contempclindent.org/article.asp?issn=0976-237X;year=2012;volume=3;issue=5;spage=55;epage=59;aulast=Kalaskar
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