The Japanese Journal of Pediatric Dentistry
Online ISSN : 2186-5078
Print ISSN : 0583-1199
ISSN-L : 0583-1199
A Case of Monozygotic Twin Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)
Haruhisa OguchiHiroshi SasakiHiroshi Ono
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1987 Volume 25 Issue 4 Pages 851-862

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Abstract
In 1940 Ellis and van Creveld described three infants with this disorder. The Ellis-van Creveld syndrome is characterized by polydactly, ectodermal dysplasia and congenital cardiac defects.
The patients were monozygotic twin sisters,3 years of age. As far as their family history is concerned, there were no congenital malformations in the case of the parents and relatives. No contributory event was noted during the course of pregnancy. Dental examination showed that the superior right deciduous central incisor was missing only in the case of the elder sister. There were many severe caries present. Calcification of the deciduous teeth seemed to be complete. The developing buds of the permanent teeth were present and development would seem to be normal for a child of this age. The upper and lower lips were not connected to the gingiva with frenula except in the case of the labium frenum of the younger sister. Further studies based on accumulated cases will be necessary.
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© The Japanese Society of Pediatric Dentistry
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