Abstract
Cleidocraniadl ysostosis (CCD) a, form of congenitalb one dysplasia, w as first reported by Marie and Sainton in 1898. They stated that it had four characteristics ; defective clavicles, abnormal ossification of cranial bones, imperfect growth of teeth, and the hereditary nature.
In Japan, less than 100 cases of CCD have been reported since Haneda reported the first case in 1933. We recently observed 3 cases of CCD, one hereditary and two isolated cases.
Case 1. A 49-year-oldm ale came to our hospitalc omplainingo f a continuoush eadachea fter havings uffered a head injury. A depression was observed on the patient's forehead. The patient stated it had been there since birth. There were no abnormalities in his past history. X-rays revealed a defective frontal bone and hypoplasia of the acromial ends of the clavicles. No other neurological abnormalities were found. A study of his family history revealed his mother, grandmother, elder brother, younger sister and two of his three children had similar depressions in the same area. His headache disappeared after medication and he is now leading a normal life.
Case 2. A 16-year-old male admitted with epilepsy was observed to have a soft forehead. He stated that elementary schooling had been delayed until he was 9 years old due to delayed physical development, and that he had experienced convulsions since pre-school days. X-rays revealed defects of the frontal bone, parietal bone, and both clavicles. He is now attending school regularly while undergoing medication with an anticonvulsant.
Case 3. A 10-month-old male infant came to our hospital with complaint of mid-frontal soft depression. He also had hypoplasia of acromial ends of the clavicles.
None of the three cases showed chromosomal abnormalities.