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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

Treatment

There is no specific treatment for the bone problems. An oral surgeon should monitor teeth regularly. An otologist should check for hearing problems.

Expectations (prognosis)

The bone symptoms usually cause few problems. Appropriate dental care is important.

Complications

The dental problems are the most significant complications.

Calling your health care provider

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.

Prevention

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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