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기저세포 모반 증후군 : 증례보고

Basal Cell Nevus Syndrome : A Case Report

  • 김지연 (한림대학교 성심병원 소아치과) ;
  • 오소희 (한림대학교 성심병원 소아치과)
  • Kim, Jeeyoun (Department of Pediatric Dentistry, Sacred Heart Hospital, Hallym University) ;
  • Oh, Sohee (Department of Pediatric Dentistry, Sacred Heart Hospital, Hallym University)
  • 투고 : 2013.11.05
  • 심사 : 2013.11.27
  • 발행 : 2014.05.30

초록

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.

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.

키워드

참고문헌

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