| Subject | Contents |
| Definition | An autosomal dominant inherited disorder of bony development characterized by absent or incompletely formed collar bones, characteristic facial appearance, short stature, and dental abnormalities. |
| Alternative Names | Cleidocranial dysplasia |
| Causes, incidence, and risk factors | Cleidocranial dysostosis is inherited as an autosomal dominant characteristic. It is equally common in males and females. If one parent is affected, the child has a 50% probability of having the disease. A heavy protruding brow, protruding jaw, wide nasal bridge, and abnormal misaligned teeth characterize the disorder. The incomplete development or absence of the collar bones allows the shoulders to be brought together in front of the body. Other bony abnormalities exist. Intelligence is normal. |
| Symptoms | incomplete or absent collar bone (10% complete absence) ability to touch shoulders together in front of body loose joints (laxity of joints) delayed closure of fontanels (soft spots) failure to lose the primary teeth at the expected time slow eruption of secondary teeth extra teeth (supernumerary teeth) peg teeth teeth - delayed or absent formation low nasal bridge frontal, occipital, and parietal bossing short forearms, and fingers |
| Signs and tests | There is often a family history of cleidocranial dysostosis. Skeletal X-rays show: undergrowth of the clavicle (collarbone) undergrowth of the scapula (shoulder blade) failure of the pubic symphysis to close (the bony structures at the front of the pelvis) |
| Treatment | There is no specific treatment for the bony abnormalities. An oral surgeon should monitor teeth regularly, with particular attention to both decay and cosmetic appearance. An otologist should check for hearing defects. |
| Support Groups | |
| Expectations (prognosis) | The bony abnormalities cause little problem. Appropriate dental care is vital. |
| 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, or already have a child with similar symptoms, and are planning to have a child. |
| Prevention | Genetic counseling is appropriate for prospective parents with a family history of cleidocranial dysostosis or where one or both parents are affected. |
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