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
