Abstract
In 1940 Ellis and van Creveld first described a type of chondrodysplasia accompanied by ectodermal dysplasia, polydactyly and congenital morbus cordis, which is now generally accepted as the Ellis-van Creveld syndrome.
The patient, a female of 6 years, diagnosed as having the Ellis-van Creveld syndrome at the Department of Cardiovascular Surgery Toyama Red-cross Hospital were referred to the Pedodontic Clinic, Gifu College of Dentistry. Her family histry did not reveal any ectodermal and bone defects and her parents were not consanguinious and were apparently normal.
The patient had a supernumeraly digit on the ulnar side of both hands and these were amputated when she was 1 month old, and after she was operated for congenital heart disease when she was 5 years old.
Dental examination showed that bilateral primary lateral incisors of both jaws were congenitally missing. The crown of all deciduous central incisors were of a cylindric form, and the upper and lower lips were closely connected to the gingiva with numerous small frenula.
Radiographic examination of the jaws showed the congenital missing of upper lateral incisors, lower central and lateral incisors, and malformed upper central incisors.
The analysis of cephalometric radiograph revealed a relatively flattened gonial angle.
