쇄골두개 이형성증 환아의 증례보고
Cleidocranial dysplasia : Case report
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쇄골두개 이형성증은 골격과 치아 이상을 동반하는 선천성 질환으로서 다수의 조직과 장기에 직접적인 영향을 주는 중배엽성 기능장애이다. 쇄골두개 이형성증에서 일반적인 골격 결함은 두개 봉합과 천문의 지연 폐쇄, wormian bone, clavicle aplasia가 나타날 수 있다. 또한 장골, 지골, 척추, 골반 등의 전체 골격 뿐 아니라 근육, 중앙 신경계 등에 영향을 줄 수 있다. 구강내 특징으로 유치의 만기잔존, 다수의 과잉치, 미맹출된 영구치, 낭 형성 등이 나타난다. 그러나 쇄골두개 이형성증 환아의 경우 정신적 및 신체적으로 특별한 이상을 나타내지 않아 스스로 질환을 인식하지 못하는 경우가 많으며 이의 결과 유치탈락 후 영구치의 미맹출로 인하여 저작 및 심리적인 문제가 초래되는 경우가 많다. 따라서 쇄골두개 이형성증은 조기진단이 필요하며, 적절한 시기에 과잉치 제거 및 영구치 맹출을 유도하는 치료를 시행하여 저작기능의 회복 뿐 아니라 환자의 안모도 개선시킬 수 있어야 한다.
Abstract
Cleidocranial dysplasia(CCD) is a congenital disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs. Skeletal abnormalities in CCD are delayed closure of cranial suture and fontanelle, presence of wormian bone and clavicle aplasia. And CCD has an effect on the long bones, phalanges, spine, pelvis, muscles, and central nervous system. Dental manifestations include retention of deciduous teeth, multiple supernumerary teeth, delayed or absent eruption of permanent teeth and formation of cysts around nonerupted teeth. But as a result in common with a lack of medical and physical disability, patient may have no substantive complaint, there are many masticatory and psychological problem by absent eruption of permanent teeth after exfoliation of deciduous teeth. For this reason CCD is necessary fo early diagnosis and must be improvement of the patient's appearance as well as provision of a functioning masticatory mechanism by treatment of surgical removal of supernumerary teeth, followed orthodontically eruption of the natural permanent teeth at adequate time.
참고문헌 (20)
- "Cleidocranial dysostosis: report of a case", 22(1), pp.83~86 (1997)
- "Dental management in cleido-cranial dysostosis", 12, pp.46~55 (1974)
- "Development of the dentition in cleidocranial dysplasia", 19, pp.89~93 (1990)
- "Cleidocranial dysplasia:radiological appearances on dental panoramic radiography", 28, pp.89~97 (1999)
- "Functional analysis of RUNX2 mutations in japanese patients with cleidocranial dysplasia demonstrates novel genotype-phenotype correlations", 71, pp.724~738 (2002)
- "Mutations in the RUNX2 gene in patients with cleidocranial dysplasia", 19, pp.209~216 (2002)
- "Multiple supernumerary teeth in association with cleidocranial dysplasia", 21(1), pp.87~93 (1996)
- "Functrional analysis of RUNX2 mutations in cleidocranial dysplasia", 30(2), pp.184~193 (2003)
- "Progressive development of supernumerary teeth in cleidocranial dysplasia", 16, pp.103~106 (1989)
- "Management of a mandibular fracture in a patient with cleidocranial dysplasia:report of a case and review of the literature", 49, pp.405~409 (1991)
- "Dental development and molar root length in children with cleidocranial dysplasia", 17, pp.101~105 (1995)
- "Cleidocranial dysplasia under-diagnosed and misdiagnosed", 59, pp.633~642 (1992)
- "Atypical expression of cleidocranial dysplasia:clinical and molecular-genetic analysis", 5, pp.243~249 (2002)
- "Cleidocranial dysostosis prenatal ultrasound diagnosis of a late onset form", 16, pp.100~102 (2000)
- "The surgical and orthodontic management of unerupted teeth in cleidocranial dysostosis", 14, pp.43~47 (1987)
- "Combined surgical and orthodontic management of the oral abnormalities in children with cleidocranial dysplasia", 7, pp.267~273 (1978)
- "Dental treatment strategies in cleidocranial dysplasia", 172, pp.243~247 (1992)
- "Cleidocranial dysplasia:part 1-general principles of the orthodontic and surgical treatment modality", 111, pp.28~33 (1997)
- "Autotransplantation in cleidocranial dysplasia:Case report with 5 year follow-up", 53, pp.1472~1475 (1995)
- "Cleidocranial dysplasia:part 2-general protocol for the orthodontic and surgical treatment modality", 111, pp.173~183 (1997)