Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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GINGIVAL NEUROFIBROMAS OF NEUROFIBROMATOSIS TYPE 1: CASE REPORT

치은에 발생한 신경섬유종의 외과적 치험례

  • Park, Seung-Hyo (Department of Pediatric Dentistry, Collage of Dentistry, Chosun University) ;
  • Lee, Nan-Young (Department of Pediatric Dentistry, Collage of Dentistry, Chosun University) ;
  • Lee, Sang-Ho (Department of Pediatric Dentistry, Collage of Dentistry, Chosun University)
  • 박승효 (조선대학교 치의학전문대학원 소아치과학교실) ;
  • 이난영 (조선대학교 치의학전문대학원 소아치과학교실) ;
  • 이상호 (조선대학교 치의학전문대학원 소아치과학교실)
  • Received : 2009.11.06
  • Accepted : 2010.02.16
  • Published : 2010.05.31
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Abstract

Neurofibromatosis type 1, an autosomal dominant disorder with prevalence of 1 per 3000 people, has clinical features of cafe au lait spots in skin, multiple neurofibroma and dysplasia in skeletal, endocrinal, and blood vessel systems. Actual intraoral neurofibromas are known to occur in 25% of patients. A 9 year-old girl diagnosed with type 1 neurofibromatosis visited our hospital with chief complaint of gingival swelling. Gingival enlargement in lower anterior region existed and cafe au lait spots were confirmed in patient' skin. Enlarged gingival tissue were excised under local anesthesia. Neurofibroma was confirmed with biopsy. Clinical examination after months showed fine recovery without any evidence of recurrence. Due to its possibility of recurrence, periodic follow-up will be needed.

제1형 신경섬유종증은 약 3000명당 1명 꼴로 발생되며 상염색체 우성유전질환이다. 피부의 카페오레반점 및 다발성 신경 섬유종과 피부, 신경계, 골격계, 내분비계, 혈관계의 다양한 이형성이 특징이다. 실제적인 구강 내 신경섬유종은 25%의 환자에서 발생한다고 알려져 있다. 제1형 신경섬유종증으로 진단된 만9세의 여아가 치은의 부종을 주소로 본원에 내원하였다. 하악 전치부 설측에 치은비대가 존재하였고, 환자의 사지와 몸통에서 갈색반점인 카페오레 반점을 확인할 수 있었다. 비대된 치은조직을 국소마취 하에 제거하였고, 조직검사를 시행한 결과 신경섬유종으로 확진하였다. 7개월 후 검사 결과 재발의 증거없이 양호한 치유를 보였다. 재발의 가능성이 있으므로 정기적인 검사가 필요하다.

Keywords

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