Cleidocranial dysostosis is disorder involving the abnormal development of bones in the skull and clavicle (collar) area. The condition is passed down through families (inherited).
Cleidocranial dysostosis is inherited as an autosomal dominant characteristic. That means if one of your parents has the condition, you and your siblings each have a 50% chance of developing the cleidocranial dysostosis.
Cleidocranial dysostosis is congenital, which means it is present from before birth. The condition affects girls and boys equally. Patients with cleidocranial dysostosis have a jaw and brow area that sticks out (protrudes) and the middle of their nose (nasal bridge) is wide. The collar bones may be missing or abnormally developed. This pushes the shoulders together in front of the body. Other bone problems may exist.
Adult teeth may develop later than normal, and extra set of adult teeth grow in. This causes the normal teeth to become crooked.
The condition does not affect one's intelligence.
There is often a family history of cleidocranial dysostosis. X-rays are usually taken. They may show:
- Undergrowth of the clavicle (collarbone)
- Undergrowth of the scapula (shoulder blade)
- Failure of the pubic symphysis (the place in the front of the pelvis) to close
There is no specific treatment for the bone problems. An oral surgeon should monitor teeth regularly. An otologist should check for hearing problems.
The bone symptoms usually cause few problems. Appropriate dental care is important.
The dental problems are the most significant complications.
Call your health care provider if you have a family history of cleidocranial dysostosis and are planning to have a child. Also call if you have a child with similar symptoms.
Genetic counseling is appropriate if a person with a family or personal history of cleidocranial dysostosis is planning to have children.
Review Date:
10/16/2006
Reviewed By:
Thomas T. Jeneby, M.D., The Plastic and Cosmetic Center of South Texas, San Antonio, TX. Review provided by VeriMed Healthcare Network.
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