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J Korean Acad Pediatr Dent. 2009;36(3): 456-463.
ORAL MENIFESTATION AND TREATMENT STRATEGIES IN CLEIDOCRANIAL DYSPLASIA
Park Hee Suk, Young-Jin Kim, Hyun-Jung Kim, Soon-Hyeun Nam
Pediatric dentistry, School of Dentistry, Kyungpook National University
Corresponding Author: Soon-Hyeun Nam ,Tel: 053-600-7201, Email: shnam@knu.ac.kr
Received: March 17, 2009;  Accepted: June 27, 2009.
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ABSTRACT
Cleidocranial dysplasia(CCD) is a congenital disorder of skeletal and dental abnormality, which is a mesodermal dysfunction influencing many tisssues and organs. Skeletal abnormalities in CCD are delayed closure of cranial suture and fontanelle, presence of wormian bone and clavicle aplasia. CCD also has an effect on long bones, phalanges, spine, pelvis, muscles, and central nervous system. Dental manifestations include retention of deciduous teeth, multiple supernumerary teeth, delayed eruption or impaction of permanent teeth and formation of cysts around nonerupted teeth. However, due to lack of any substansive medical or physical disability, diagnosis is often late, thereby causing masticatory and psychological problems caused by delayed eruption of permanent teeth after exfoliation of deciduous teeth. For this reason, CCD requires early diagnosis, and the patient's appearance must be improved. Also, provision for a functional masticatiory mechanism by treatment of surgical removal of supernumerary teeth followed by orthodontic eruption of the natural permanent teeth at an adequate time is necessary.
Keywords: Cleidocranial dysplasia | Unerupted teeth | Eruption guidance
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