A case of cleidocranial dysostosis with numerous unerupted teeth.

Abstract

Cleiodocranial dysostosis is a rather rare bone development disease and is characterized by abnormalities of the skull, clavicles and teeth.We experienced a case of cleidocranial dysostosis in a 48-year-old man.The patient was of short stature and could bring his shoulders medially. The maxilla appeared underdeveloped, so that the mandible was relatively prognathous. In the oral cavity, the palate was high with a groove along the median palatine suture.Roentgenographic examinations revealed the following: The fontanels remained open and the sutures were unossified. The maxilla and mandible contained at least eighteen unerupted teech. A cyst involving the retained teeth was present in the mandibular premolar region on the right side. The lateral portions of the clavicles were absent. Spina bifida occult and coxa vara were present.