| CASE REPORT |
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| Year : 2009 | Volume
: 27
| Issue : 4 | Page : 256-259 |
Clinical manifestations of Ellis-van Creveld syndrome
C Vinay1, R Sudhakara Reddy2, KS Uloopi1, R Chandra Sekhar1
1 Department of Pedodontics and Preventive Dentistry, Vishnu Dental College and Hospital, Bhimavaram - 534 202, Andhra Pradesh, India
2 Department of Oral Medicine and Radiology, Vishnu Dental College and Hospital, Bhimavaram - 534 202, Andhra Pradesh, India
Correspondence Address:
C Vinay
Department of Pedodontics and Preventive Dentistry, Vishnu Dental College, Bhimavaram - 534 202, Andhra Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-4388.57663
Ellis-van Creveld syndrome (EVC) is a chondro-ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation and ectodermal and heart defects. It is a rare disease complex and very few cases have been reported in dental literature. This condition is inherited as an autosomal recessive trait with variable expression. The present case report describes EVC in a 7-year-old girl, with all the tetrad of cardinal features. We found a rare dental aberration in form; appearance of single conical roots in primary molars. The management of children with EVC is multidisciplinary, with consideration for the high incidence of cardiac defects in these patients.
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