Cleidocranial Dysplasia Teeth

Cleidocranial dysplasia which is also known as cleidocranial dysostosis is the birth defect related to bones and teeth. Along with small or abnormal collar bones, the new born have abnormal teeth arrangement. As it is a birth defect and develops during the development of fetus within the womb of mother, so the teeth are abnormal when they 1st grow up. A specific Gene is involved in the mutation, which is responsible for this abnormality. This gene is RUNX2 gene. The mandible jaw is not aligned and is prognathic. Prognathism is the positional placement of mandible according to maxilla.

The mandibular jaw line may position far ahead from maxillary jaw line with abnormal placement and growth of teeth. The teeth are not properly grown. The jaw line protrudes ahead from the original jaw line in case of cleidocranial dysplasia. There is a specific difference between the teeth. The teeth are not aligned and are broken and deshaped. In cleidocranial dysplasia the new born may suffer from mandibular hypoplasia. In this condition the mandibular jaw line is not properly developed. The teeth looks distorted and rough.

The overall appearance of face is not attractive. Due to mandibular hypoplasia, infant and his mother may find problem during feeding. In case of emergency or any injury, the intubation becomes difficult in both the cases i.e. in infants and in adults. There is a problem regarding development of permanent teeth. Children with cleidocranial dysplasia may does not develop permanent teeth. Children most probably develop supernumerary teeth. Supernumerary teeth are the additional teeth which grows above the original jaw line.

This condition is also termed as hyperdontia. There is another condition named hypodontia which is right opposite to the hyperdontia. In hypodontia, there is a lack of teeth (original teeth). So supernumerary teeth are removed to make space for the adult teeth to grow. The no. Of supernumerary teeth observed is 13. 13 is the average count of supernumerary teeth it may vary. In cleidocranial dysplasia, the supernumerary teeth may be replaced by adult teeth, but still there may be deficient cementum formation. Sometimes even after removal of supernumerary teeth, the child does not develop permanent teeth.

The abnormality is all about the positioning of teeth And overall appearance of the jaws but he does not find any problem regarding language, speaking and talking. Same in the case of brain, they may have underdeveloped or abnormally developed head, but their thinking power and understanding power is good. They can think and talk normally. As it is a genetic disease, so it cannot be cured pemanently. There are various surgeries to correct the alignment of jaws and teeth. The surgical correction protects the patient from worsening of the deformity.

During surgery, the supernumerary teeth are removed and made a space for the development of adult teeth. All the surgeries regarding cleidocranial dysplasia are performed at the age of 5 years. Not before 5 years because the child is not strong enough for the surgeries.

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