Imaging Science in Dentistry

Korean Academy of Oral and Maxillofacial Radiology (대한영상치의학회)
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Ellis-van Creveld syndrome in an Indian child: a case report

  • Veena, K.M. (Department of Oral Medicine and Radiology, Yenepoya Dental College, Yenepoya University) ;
  • Jagadishchandra, H. (Department of Oral Surgery, Yenepoya Dental College, Yenepoya University) ;
  • Rao, Prasanna Kumar (Department of Oral Medicine and Radiology, Yenepoya Dental College, Yenepoya University) ;
  • Chatra, Laxmikanth (Department of Oral Medicine and Radiology, Yenepoya Dental College, Yenepoya University)
  • Received : 2011.06.29
  • Accepted : 2011.10.25
  • Published : 2011.12.31
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Abstract

Ellis-van Creveld syndrome is a rare congenital genetic disorder having autosomal recessive inheritance. It is a syndrome affecting the Amish population of Pennsylvania in USA with prevalence rate of 1/5,000 live at birth. In non-Amish population, the birth prevalence is 7/1,000,000. The syndrome is characterized by bilateral postaxial polydactyly of the hands, chondrodysplasia of long bones resulting in acromesomelic dwarfism, ectodermal dysplasia affecting nails as well as teeth and congenital heart malformation. There were very rare reports of this syndrome in dentistry. The present case focuses on the striking and constant oral findings of these patients, which are the main diagnostic features of this syndrome. Since the oral manifestations affect the esthetic, speech, and jaw growth of the child, the dentists have an important role to play in proper management of such case.

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References

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