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Cleidocranial Dysostosis
Definition Cleidocranial dysostosis is an inherited disorder of bone development, characterized by absent or incompletely formed collar bones, abnormal shape of the skull with depression of the sagital suture, characteristic facial appearance, short stature, and dental abnormalities.
Overview, Causes, & Risk Factors
Cleidocranial dysostosis is inherited as an autosomal dominant characteristic, which means that if one parent is affected, the children have a 50% chance of having the disease and any child who inherits the gene for the condition from the affected parent (regardless of the other parent's normal gene) will develop it.
The disorder is present from before birth (congenital) and equally common in males and females. People with cleidocranial dysostosis have a heavy protruding brow, a protruding jaw, and a wide nasal bridge. As these children mature, their adult teeth may come in late, but then they often develop an extra set of adult teeth, which causes their normal teeth to become misaligned.
The incomplete development or absence of the collar bones allows the shoulders to be brought together in front of the body. Other bone abnormalities exist. Intelligence is normal.
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• Definition
• Overview, Causes, & Risk Factors
• Symptoms & Signs
• Prevention
• Diagnosis & Tests
• Treatment
• Prognosis
• Complications
• Calling Your Health Care Provider
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Review Date : 12/27/2002
Reviewed By : Hebe Molmenti, M.D., Ph.D., Private Practice specializing in Plastic and Reconstructive Surgery, Baltimore, MD. Review provided by VeriMed Healthcare Network.
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