Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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NEVOID BASAL CELL CARCINOMA SYNDROME : A REPORT OF CASE

기저세포모반 증후군 환아의 증례보고

  • Heo, Su-Kyung (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 2) ;
  • Choi, Nam-Ki (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 2) ;
  • Kim, Seon-Mi (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 2) ;
  • Yang, Kyu-Ho (Department of Pediatric Dentistry, School of Dentistry, Chonnam National University and Dental Research Institute and second stage of BK 2)
  • 허수경 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 최남기 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 김선미 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21) ;
  • 양규호 (전남대학교 치의학전문대학원 소아치과학교실 및 치의학연구소 및 2단계 BK21)
  • Published : 2008.11.30
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Abstract

Nevoid basal cell carcinoma syndrome is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. The syndrome is an autosomal dominant inherited, with a high penetration and visible expression. The syndrome is characterized by a series of associated anomalies such as cutaneous, dentofacial, skeletal, ophthalmologic, neurological, and genital anomalies. Generally, the jaw cysts are multiple odontogenic keratocysts, affecting any area of maxilla and mandible. Multiple odontogenic keratocysts of this syndrome are more recurrent than the keratocysts of non-syndrome, thus they are treated aggressively for complete removal. We report a case of multiple jaw cysts associated with nevoid basal cell carcinoma syndrome. In clinical and radiological examinations, frontal bossing, hypertelorism, mild mental retardation and two odontogenic keratocysts in both the maxilla and mandible were observed. Two cysts were treated by marsupialization. For the management of eruption of unerupted teeth, periodic recall check and orthodontic treatment are required.

기저세포모반증후군은 상염색체 우성으로 유전하며 외배엽성과 중배엽성 기관과 장기에 이환된다. 기저세포모반 증후군은 특징적으로 피부 이상, 치아와 골격 이상, 안과적 장애, 신경 장애, 생식계 이상 등이 나타난다. 악골의 병소는 보통 여러 부위를 포함하는 치성각화 낭종이며 상, 하악에 모두 나타난다. 다발성 악골 낭종은 워낙 높은 재발율을 가진 각화성 낭종이기 때문에 완전 적출을 위해 적극적인 치료를 해야 한다. 제거 후는 재발 여부를 확인하기 위해 신중한 주기적 재검진이 필요하다. 본 증례는 다발성 치성낭종의 치료를 위해 내원한 환자가 기저세포모반증후군으로 진단되어 양호한 결과를 얻었기에 이를 보고하는 바이다. 이 환자는 전두골 돌출, 양안격리증, 정신지체 및 상, 하악에 2개의 치성 각화낭종을 가지고 있었다. 낭종은 조대술로 치료되었고 현재 obturator와 공간유지장치로 유지하고 있으며 치아 맹출 여부를 관찰하고 있다.

Keywords

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