ORAL MENIFESTATION AND TREATMENT STRATEGIES IN CLEIDOCRANIAL DYSPLASIA

Suk, Park Hee; Kim, Young-Jin; Kim, Hyun-Jung; Nam, Soon-Hyeun

Journal of the Korean Academy of Pediatric Dentistry 2009;36(3):456-463.
Published online August 31, 2009.

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

쇄골두개 이형성증 환아의 구강내 증상과 치료 전략

박희숙, 김영진, 김현정, 남순현

경북대학교 치과대학 소아치과학교실

Correspondence: Soon-Hyeun Nam, Tel: 053-600-7201, Email: shnam@knu.ac.kr

Received: 17 March 2009 • Accepted: 27 June 2009

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.

Key Words: Cleidocranial dysplasia, Unerupted teeth, Eruption guidance