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Medical Encyclopedia |
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Definition Return to top
Cleidocranial dysostosis is disorder involving the abnormal development of bones in the skull and collar (clavicle) area. The condition is passed down through families (inherited).
Causes Return to top
Cleidocranial dysostosis is caused by an abnormal gene. It is passed down through families as an autosomal dominant trait. That means you only need to get the abnormal gene from one parent in order for you to inherit the disease.
Cleidocranial dysostosis is a congenital condition, which means it is present from before birth. The condition affects girls and boys equally.
Symptoms Return to top
Patients with cleidocranial dysostosis have a jaw and brow area that sticks out. 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.
Primary teeth do not fall out at the expected time. 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.
Other symptoms can include
Exams and Tests Return to top
There is often a family history of cleidocranial dysostosis. X-rays are usually taken and may show:
Treatment Return to top
There is no specific treatment for the bone problems. An oral surgeon should monitor teeth regularly. An otologist should check for hearing problems.
Outlook (Prognosis) Return to top
The bone symptoms usually cause few problems. Appropriate dental care is important.
Possible Complications Return to top
Complications include dental problems and shoulder dislocations.
When to Contact a Medical Professional Return to top
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 Return to top
Genetic counseling is appropriate if a person with a family or personal history of cleidocranial dysostosis is planning to have children.
References Return to top
Horton WA, Hecht JT. Disorders involving transcription factors. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 696.
Update Date: 12/1/2008 Updated by: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
