Cleidocranial dysostosis

Definition of Cleidocranial dysostosis

Cleidocranial dysostosis is a disorder involving the abnormal development of bones in the skull and collar (clavicle) area. The condition is passed down through families (inherited).

Causes, incidence, and risk factors

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.

Symptoms

Patients with cleidocranial dysostosis have a jaw and brow area that sticks out. The middle of their nose (nasal bridge) is wide.

Signs and tests

There is often a family history of cleidocranial dysostosis. X-rays are usually taken and may show:

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.

Review

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. – 1/29/2010

ADAM Medical Encyclopedia

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