Cleidocranial Dysostosis Characteristics: Hypertelorism and Permanent Teeth Not Erupting

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.