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Cleidocranial dysostosis

Cleidocranial dysostosis


Definition:

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).

Causes, incidence, and risk factors:

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.

Symptoms:
  • Incomplete or absent collar bone
  • Ability to touch shoulders together in front of body
  • Loose joints
  • Delayed closure of fontanelles
  • Tooth problems
    • Primary teeth do not fall out at the expected time
    • Adult teeth are slow to develop
    • Extra teeth (supernumerary teeth)
    • Peg teeth
    • Teeth may be missing
  • Low nasal bridge
  • Occipital, parietal, and frontal bossing
  • Short forearms
  • Short fingers
Signs and tests:

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

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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