Abstract

 


Ellis-van Creveld Syndrome includes several types of ectodermal dysplasia involving skin, nails and teeth, polydactyly and congenital heart defects in addition to chondrodystrophy. A case is described with polydactyly and intermediate type atrioventricular septal defect without other major associated anomalies. This specific association is extremely rare. The patient underwent cardiac operation for atrioventricular septal defect. Complete improvement was seen postoperatively. It is suggested that there is a specific association between polydactyly and the atrioventricular septal defect found in this patient. This picture does not conform to, but bears some resemblance to the Ellis-van Creveld syndrome. [Journal of Turgut Ozal Medical Center 1997;4(2): 209-211]

Key Words: Ellis-van Creveld Syndrome, polydactyly, atrioventricular septal defect