Cleidocranial Dysostosis Characteristics:
hypertelorism and permanent teeth not erupting are both characteristics of Cleidocranial Dysostosis (CCD), a rare genetic disorder affecting bone development. Here's how:
Hypertelorism:
- This refers to wider-than-normal spacing between the eyes. In CCD, it's caused by delayed or incomplete ossification of the facial bones, particularly the bones forming the bridge of the nose.
- This characteristic is present in approximately 70-80% of individuals with CCD and can be quite noticeable.
Permanent Teeth Not Erupting:
- CCD affects bones formed through intramembranous ossification, including the jaw bones. This can lead to delayed or even complete failure of permanent teeth to erupt properly.
- In some cases, deciduous (baby) teeth may persist throughout life, while permanent teeth remain embedded within the jaw.
- Additional dental anomalies associated with CCD include supernumerary teeth (extra teeth), malocclusion, and misshapen teeth.
Other Characteristics of CCD:
- Hypoplastic or aplastic clavicles (collarbones): This often manifests as the ability to touch shoulders together due to the lack of collarbones or their limited development.
- Delayed closure of fontanelles (soft spots) on the skull.
- Short stature.
- Midface hypoplasia (underdevelopment of the middle part of the face).
- Hearing loss due to abnormalities in the middle ear bones.
Diagnosis and Management:
CCD is diagnosed based on clinical features, X-rays, and genetic testing. Management focuses on addressing specific symptoms and improving quality of life. This may involve:
- Surgical procedures to create functional collarbones or correct jaw bone issues for tooth eruption.
- Orthodontic treatment to address malocclusion.
- Hearing aids as needed.
- Regular dental care to manage retained baby teeth and monitor permanent tooth development.
Remember:
I am not a medical professional, and this information should not be used as a substitute for professional medical advice. If you have concerns about yourself or someone you know, please consult a doctor or healthcare provider.
