Abstract
The Ellis-van Creveld syndrome is characterised by chondrodysplasia, ectodermal dysplasia, postaxial polydactyly of bilateral hands, and congenital heart disease.
The patient was a 3-year 6-month old boy. Dwarfism due to shortening of the extremities and bilateral postaxial polydactyly of the hands were found at birth, and he was diagnosed as having the Ellis-van Creveld syndrome when he was 2-month old. He was born by incest between his mother and “her father”. He had no congenital heart disease.
In this paper, we report especially with respect to his oral findings.
1) A failure in fusion was found in the middle portion of the mandibular bone.
2) Because of the direct connection of the upper lip to the gingiva and numerous buccal frenums, the mucobuccal fold was narrow in the anterior region.
3) Congenitally missing teeth and a serrated alveolar ridge were found in the maxillary and mandibular anterior regions.
4) The shapes of crown, root, and pulp chamber of all the primary teeth were malformed. The primary canines were peg shaped, while the primary molars were small and malformed. The maxillary first and second primary molars and the mandibular first primary molars had single roots, and the mandibular second primary molars showed taurodont teeth.
