Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Basal Cell Nevus Syndrome : A Case Report

기저세포 모반 증후군 : 증례보고

  • Kim, Jeeyoun (Department of Pediatric Dentistry, Sacred Heart Hospital, Hallym University) ;
  • Oh, Sohee (Department of Pediatric Dentistry, Sacred Heart Hospital, Hallym University)
  • 김지연 (한림대학교 성심병원 소아치과) ;
  • 오소희 (한림대학교 성심병원 소아치과)
  • Received : 2013.11.05
  • Accepted : 2013.11.27
  • Published : 2014.05.30
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Abstract

The basal cell nevus syndrome is also known as the Gorlin-Goltz syndrom. It is a dominant autosomal disorder which is characterized by keratocystic odontogenic tumors in the jaw, skeletal abnormalities, and multiple basal cell nevi carcinomas. This study reports an 11-year-old boy with multiple odontogenic keratocysts in the jaw, hypertelorism, and frontal bossing. When a young patient has cystic lesions with an impacted permanent teeth, it is important to preserve the teeth. For a growing patient with impacted permanent teeth, a more conservative method is suggested, which will enable the preservation the permanent teeth in Gorlin-Goltz syndrome.

기저세포 모반 증후군은 상염색체 우성 방식으로 유전되는 질환으로 다발성 각화낭성 치성종양, 대두증 및 두정골 융기, 골격계 기형 등을 보이는 질환이다. 다발성 각화낭성 치성 종양, 두정골 융기, 양안격리증을 가진 만 11세 환자의 하악과 상악에서 발생한 다양한 크기의 낭종에 대한 적출술과 이종골 이식술을 시행 후 교정적 견인을 시행하였다. 낭종에 의한 매복 영구치를 가진 성장중인 기저세포 모반 증후군 환자에서의 보존적인 치료를 하였기에 문헌고찰과 더불어 보고하고자 한다.

Keywords

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