Oral manifestations of Ellis-van Creveld syndrome
Identifieur interne : 001D57 ( Pmc/Checkpoint ); précédent : 001D56; suivant : 001D58Oral manifestations of Ellis-van Creveld syndrome
Auteurs : Ritesh Kalaskar ; Ashita R. KalaskarSource :
- Contemporary Clinical Dentistry [ 0976-237X ] ; 2012.
Abstract
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.
Url:
DOI: 10.4103/0976-237X.95106
PubMed: 22629068
PubMed Central: 3354796
Affiliations:
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<front><div type="abstract" xml:lang="en"><p>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.</p>
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<pmc article-type="case-report"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Contemp Clin Dent</journal-id>
<journal-id journal-id-type="iso-abbrev">Contemp Clin Dent</journal-id>
<journal-id journal-id-type="publisher-id">CCD</journal-id>
<journal-title-group><journal-title>Contemporary Clinical Dentistry</journal-title>
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<issn pub-type="ppub">0976-237X</issn>
<issn pub-type="epub">0976-2361</issn>
<publisher><publisher-name>Medknow Publications & Media Pvt Ltd</publisher-name>
<publisher-loc>India</publisher-loc>
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<article-id pub-id-type="pmc">3354796</article-id>
<article-id pub-id-type="publisher-id">CCD-3-55</article-id>
<article-id pub-id-type="doi">10.4103/0976-237X.95106</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group><article-title>Oral manifestations of Ellis-van Creveld syndrome</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Kalaskar</surname>
<given-names>Ritesh</given-names>
</name>
<xref ref-type="aff" rid="aff1"></xref>
<xref ref-type="corresp" rid="cor1"></xref>
</contrib>
<contrib contrib-type="author"><name><surname>Kalaskar</surname>
<given-names>Ashita R.</given-names>
</name>
<xref ref-type="aff" rid="aff2">1</xref>
</contrib>
</contrib-group>
<aff id="aff1"><italic>Department of Pedodontics, Government Dental College and Hospital, VSPM Dental College and Research Center, Nagpur, Maharashtra, India</italic>
</aff>
<aff id="aff2"><label>1</label>
<italic>Department of Oral Diagnosis Medicine and Radiology, VSPM Dental College and Research Center, Nagpur, Maharashtra, India</italic>
</aff>
<author-notes><corresp id="cor1"><bold>Correspondence:</bold>
Dr. Ritesh Kalaskar, Department of Pedodontics, Government Dental College and Hospital, Nagpur, Maharashtra, India. E-mail: <email xlink:href="riteshpedo@gmail.com">riteshpedo@gmail.com</email>
</corresp>
</author-notes>
<pub-date pub-type="ppub"><month>4</month>
<year>2012</year>
</pub-date>
<volume>3</volume>
<issue>Suppl1</issue>
<fpage>S55</fpage>
<lpage>S59</lpage>
<permissions><copyright-statement>Copyright: © Contemporary Clinical Dentistry</copyright-statement>
<copyright-year>2012</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc-sa/3.0"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
</license>
</permissions>
<abstract><p>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.</p>
</abstract>
<kwd-group><kwd>Absence of mucobuccal fold</kwd>
<kwd>alveolar notches</kwd>
<kwd>autosomal recessive</kwd>
<kwd>genu valgum</kwd>
<kwd>partial harelip</kwd>
</kwd-group>
</article-meta>
</front>
</pmc>
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