Document Detail


Chondroectodermal dysplasia (Ellis van Creveld syndrome): a report of three cases with review of literature.
MedLine Citation:
PMID:  17347543     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Chondroectodermal dysplasia is a rare mesenchymal - ectodermal dysplasia first described in 1940 by Richard W.B. Ellis and Simon van Creveld now known as Ellis van Creveld syndrome. It is also known as Mesvectodermal dysplasia. Majority of cases were characteristically seen in one particular inbred population from the Amish community of Lancaster County, Pennsylvania, U.S.A. The syndrome manifests with several skeletal anomalies, oral mucosal and dental anomalies, congenital cardiac defects and nail dysplasia. Ellis van Creveld syndrome may be differentiated from other chondrodystrophies like achondroplasia, chondroplasia punctata, asphyxiating thorasic dystrophy and Morquio's syndrome. The presence of oral mucosal and dental alterations like notching of the lower alveolar process, fusion of the upper lip with gingival mucosal margin, occasional presence of neonatal teeth, oligodontia and conical shape of anterior teeth will confirm the diagnosis of Ellis van Creveld syndrome and hence its importance to dentists.
Authors:
K Kurian; S Shanmugam; T Harsh Vardah; Siddharth Gupta
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Indian journal of dental research : official publication of Indian Society for Dental Research     Volume:  18     ISSN:  0970-9290     ISO Abbreviation:  Indian J Dent Res     Publication Date:    2007 Jan-Mar
Date Detail:
Created Date:  2007-03-09     Completed Date:  2007-06-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9202990     Medline TA:  Indian J Dent Res     Country:  India    
Other Details:
Languages:  eng     Pagination:  31-4     Citation Subset:  D    
Affiliation:
Department of Oral Medicine and Radiology, Ragas Dental College and Hospital, Chennai, India. bonkurian@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
Child
Diagnosis, Differential
Ellis-Van Creveld Syndrome / complications,  pathology*
Female
Humans
Infant
Male
Mouth Abnormalities / etiology*
Tooth Abnormalities / etiology*

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